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Albireo Pharma

albo · NASDAQ Healthcare
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FY2020 Annual Report · Albireo Pharma
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ANNUAL REPORT
2020

www.albireopharma.com

UNITED STATES 
SECURITIES AND EXCHANGE COMMISSION 
Washington, D.C. 20549 

Form 10-K 

(Mark One) 
☒ 

ANNUAL REPORT PURSUANT TO SECTION 13 OR 15(d) OF THE SECURITIES EXCHANGE ACT OF 1934 

For the fiscal year ended December 31, 2020 

or 

☐ 

TRANSITION REPORT PURSUANT TO SECTION 13 OR 15(d) OF THE SECURITIES EXCHANGE ACT OF 1934 

For the transition period from            to           . 

Commission File Number 001-33451 

Albireo Pharma, Inc. 
(Exact Name of Registrant as Specified in Its Charter) 

Delaware 
(State or Other Jurisdiction of 
Incorporation or Organization) 

10 Post Office Square, Suite 1000  
Boston, MA 
(Address of Principal Executive Offices) 

90-0136863 
(I.R.S. Employer 
Identification No.) 

02109 
(Zip Code) 

Registrant’s telephone number, including area code 
(857) 254-5555 

Securities registered pursuant to Section 12(b) of the Act: 

Title of Each Class 
Common Stock, par value $0.01 per share 

Trading Symbol(s) 
ALBO 

Name of Each Exchange on Which Registered 
The Nasdaq Capital Market 

Securities registered pursuant to Section 12(g) of the Act: 
None 

Indicate by check mark if the registrant is a well-known seasoned issuer, as defined in Rule 405 of the Securities Act.   Yes  ☐    No  ☒ 

Indicate by check mark if the registrant is not required to file reports pursuant to Section 13 or Section 15(d) of the Act.   Yes  ☐    No  ☒ 

Indicate by check mark whether the registrant (1) has filed all reports required to be filed by Section 13 or 15(d) of the Securities Exchange Act of 1934 during the preceding 12 months (or for 
such shorter period that the registrant was required to file such reports), and (2) has been subject to such filing requirements for the past 90 days.    Yes  ☒    No  ☐ 

Indicate by check mark whether the registrant has submitted electronically every Interactive Data File required to be submitted pursuant to Rule 405 of Regulation S-T (§232.405 of this 
chapter) during the preceding 12 months (or for such shorter period that the registrant was required to submit such files).   Yes  ☒    No  ☐ 

Indicate by check mark if disclosure of delinquent filers pursuant to Item 405 of Regulation S-K (§ 229.405 of this chapter) is not contained herein, and will not be contained, to the best of 
registrant’s knowledge, in definitive proxy or information statements incorporated by reference in Part III of this Form 10-K or any amendment to this Form 10-K. ☐ 

Large accelerated filer 
Non-accelerated filer 

☐ 
☒   

Accelerated filer  
Smaller reporting company 
Emerging growth company  

☐ 
☒ 
☐ 

If an emerging growth company, indicate by check mark if the registrant has elected not to use the extended transition period for complying with any new or revised financial accounting 
standards provided pursuant to Section 13(a) of the Exchange Act. ☐ 

Indicate by check mark whether the registrant has filed a report on and attestation to its management’s assessment of the effectiveness of its internal control over financial reporting under 
Section 404(b) of the Sarbanes-Oxley Act (15 U.S.C. 7262(b)) by the registered public accounting firm that prepared or issued its audit report.  ☐ 

Indicate by check mark whether the registrant is a shell company (as defined in Rule 12b-2 of the Exchange Act).    Yes  ☐    No  ☒ 

The aggregate market value of the common stock of the registrant held by non-affiliates of the registrant (without admitting that any person whose shares are not included in such calculation is 
an affiliate) was approximately $394.2 million based on the price at which the common stock was last sold on The Nasdaq Capital Market as of the last business day of the registrant’s most 
recently completed second fiscal quarter. 

The number of shares of the registrant’s common stock outstanding as of February 18, 2021, was 19,113,603. 

Documents Incorporated by Reference 

The following documents (or parts thereof) are incorporated by reference into the following parts of this Form 10-K: Certain information required in Part III of this Annual Report on 

Form 10-K is incorporated from the Registrant’s Proxy Statement for its 2021 Annual Meeting of Stockholders to be filed with the Securities and Exchange Commission. 

 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
INDEX TO ANNUAL REPORT ON FORM 10-K 

CAUTIONARY NOTE REGARDING FORWARD-LOOKING STATEMENTS  . . . . . . . . . . . . . . . . . . . . . . . . . .  
PART I 

Item 1. 
Business  . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  
Item 1A.  Risk Factors  . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  
Item 1B.  Unresolved Staff Comments . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  
Item 2. 
Properties  . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  
Legal Proceedings  . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  
Item 3. 
Item 4.  Mine Safety Disclosures  . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  

PART II 

Item 5.  Market for Registrant’s Common Equity, Related Stockholder Matters and Issuer Purchases of 

Equity Securities . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  
Item 6. 
Selected Financial Data . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  
Item 7.  Management’s Discussion and Analysis of Financial Condition and Results of Operations . . . . . . .  
Item 7A.  Quantitative and Qualitative Disclosures About Market Risk  . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  
Item 8. 
Financial Statements and Supplementary Data  . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  
Changes in and Disagreements with Accountants on Accounting and Financial Disclosure . . . . . . .  
Item 9. 
Item 9A.  Controls and Procedures  . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  
Item 9B.  Other Information . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  

PART III 

Item 10.  Directors, Executive Officers and Corporate Governance  . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  
Item 11.  Executive Compensation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  
Item 12.  Security Ownership of Certain Beneficial Owners and Management and Related Stockholder 

Matters  . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  
Item 13.  Certain Relationships and Related Transactions, and Director Independence . . . . . . . . . . . . . . . . . . .  
Item 14.  Principal Accounting Fees and Services . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  

PART IV 

Item 15.  Exhibits and, Financial Statement Schedules . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  
Item 16.  Form 10-K Summary . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  
SIGNATURES  . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  

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CAUTIONARY NOTE REGARDING FORWARD-LOOKING STATEMENTS 

This Annual Report on Form 10-K includes forward-looking statements within the meaning of Section 27A of the 
Securities Act of 1933, as amended, which we refer to as the Securities Act, and Section 21E of the Securities Exchange 
Act of 1934, as amended, which we refer to as the Exchange Act, that relate to future events or to our future operating or 
financial performance. Any forward-looking statement involves known and unknown risks, uncertainties and other 
factors that may cause our actual results, levels of activity, performance or achievements to differ materially from any 
future results, levels of activity, performance or achievements expressed or implied by such forward-looking statement. 
Forward-looking statements include statements, other than statements of historical fact, about, among other things: 

• 

• 

• 

• 

• 

• 

• 

the progress, number, scope, cost, duration or results of our development activities, nonclinical studies and 
clinical trials of elobixibat, A3907, A2342 or any of our other product candidates or programs, such as the 
target indication(s) for development or approval, the size, design, population, conduct, cost, objective or 
endpoints of any clinical trial, or the timing for initiation or completion of or availability of results from any 
clinical trial (including BOLD, our pivotal clinical trial of odevixibat in patients with biliary atresia or 
ASSERT, our pivotal trial of odevixibat in Alagille syndrome, or ALGS), for submission, review or 
approval of any regulatory filing, access to the Expanded Access Program (EAP) for odevixibat, or for 
meeting with regulatory authorities; 

the potential benefits that may be derived from any of our product candidates; 

the timing of and our ability to obtain and maintain regulatory approval of our existing product candidates, 
any product candidates that we may develop, and any related restrictions, limitations, or warnings in the 
label of any approved product candidates; 

any payment that EA Pharma Co., Ltd., or EA Pharma, may make to us or any other action or decision that 
EA Pharma may make concerning elobixibat or our business relationship; 

the potential impacts of the COVID-19 pandemic on our business operations or financial condition; 

our future operations, financial position, revenues, costs, expenses, uses of cash, capital requirements, our 
need for additional financing or the period for which our existing cash resources will be sufficient to meet 
our operating requirements; or 

our strategies, prospects, plans, expectations, forecasts or objectives. 

Words such as, but not limited to, “believe,” “expect,” “anticipate,” “estimate,” “forecast,” “intend,” “may,” 
“plan,” “potential,” “predict,” “project,” “targets,” “likely,” “will,” “would,” “could,” “should,” “continue,” “scheduled” 
and similar expressions or phrases, or the negative of those expressions or phrases, are intended to identify forward-
looking statements, although not all forward-looking statements contain these identifying words. Although we believe 
that we have a reasonable basis for each forward-looking statement contained in this report, we caution you that these 
statements are based on our estimates or projections of the future that are subject to known and unknown risks and 
uncertainties and other important factors that may cause our actual results, level of activity, performance or 
achievements to differ materially from those expressed or implied by any forward-looking statement. The description of 
our Business set forth in Item 1, the Risk Factors set forth in Item 1A and our Management’s Discussion and Analysis of 
Financial Condition and Results of Operations set forth in Item 7, as well as other sections in this report, discuss some of 
the factors that could contribute to these differences. 

1 

Actual results, level of activity, performance or achievements may differ materially from those expressed or 
implied by any forward-looking statement as a result of various important factors, including our critical accounting 
policies and risks and uncertainties relating, among other things, to: 

• 

• 

• 

• 

• 

• 

• 

• 

• 

• 

• 

the design, size, duration and endpoints for, and results from BOLD, our pivotal trial of odevixibat in biliary 
atresia, and ASSERT, our pivotal trial of odevixibat in ALGS, or any other trials that will be required to 
obtain marketing approval for odevixibat to treat patients with PFIC, biliary atresia, ALGS or any other 
pediatric cholestatic liver disease or for A3907 and A2342 as potential treatments for adult liver and viral 
diseases; 

whether favorable findings from clinical trials of odevixibat to date, including findings in our completed 
Phase 3 clinical trial in PFIC and findings in indications other than PFIC, will be predictive of results from 
future clinical trials, including our pivotal trial of odevixibat in biliary atresia and our pivotal trial of 
odevixibat in ALGS; whether either or both of the U.S. Food and Drug Administration, or FDA, and 
European Medicines Agency, or EMA, will determine that the primary endpoint and treatment duration of 
our completed Phase 3 trial in patients with PFIC are sufficient, even if such primary endpoint is met with 
statistical significance, to support approval of odevixibat in the United States or the European Union, to treat 
PFIC, a symptom of PFIC, a specific PFIC subtype(s) or otherwise; and whether either agency will complete 
their respective reviews within the target timelines, including the FDA’s Prescription Drug User Fee Act 
goal date of July 20, 2021, as a potential result of the impact of the COVID-19 pandemic or otherwise; 

the outcome and interpretation by regulatory authorities of an ongoing third-party study pooling and 
analyzing long-term PFIC patient data; 

the timing for initiation or completion of, or for availability of data from, our pivotal trial of odevixibat in 
biliary atresia and our pivotal trial of odevixibat in ALGS, and the outcomes of such trials; 

delays or other challenges in the recruitment of patients for the pivotal trial of odevixibat in biliary atresia 
and the pivotal trial of odevixibat in ALGS; 

 whether odevixibat will meet the criteria to receive a rare pediatric disease priority review voucher from the 
FDA when applicable, whether a rare pediatric disease priority review voucher that we may receive in the 
future for odevixibat, if any, will be valuable to us, and, if necessary, whether the rare pediatric disease 
priority review voucher program will be renewed beyond 2026; 

the COVID-19 pandemic, which may negatively impact the conduct of, and the timing of initiation, 
enrollment, completion and reporting with respect to, our clinical trials; negatively impact the supply of drug 
product for our clinical and preclinical programs; and/or result in other adverse impacts on our business; 

the competitive environment and commercial opportunity for a potential treatment for PFIC and other 
orphan pediatric cholestatic liver diseases; 

the conduct and results of clinical trials and nonclinical studies and assessments of odevixibat, A3907, 
A2342 or any of our other product candidates and programs, including the performance of third parties 
engaged to execute them and difficulties or delays in patient enrollment and data analysis; 

the medical benefit that may be derived from odevixibat, A3907, A2342 or any of our other product 
candidates; 

the extent to which our agreement with EA Pharma for elobixibat generates nondilutive income for us; 

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• 

• 

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• 

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• 

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• 

the timing and success of submission, acceptance and approval of regulatory filings and any related 
restrictions, limitations or warnings in the label of any approved product candidates; 

the significant control or influence that EA Pharma has over the commercialization of elobixibat in Japan 
and the development and commercialization of elobixibat in EA Pharma’s other licensed territories; 

whether we elect to seek and, if so, our ability to establish a license or other partnering transaction with a 
third party for elobixibat in the United States or Europe; 

the accuracy of our estimates regarding expenses, costs, future revenues, uses of cash and capital 
requirements; 

our ability to obtain additional financing on reasonable terms, or at all; 

our ability to establish additional licensing, collaboration or similar arrangements on favorable terms and our 
ability to attract collaborators with development, regulatory and commercialization expertise; 

the success of competing third-party products or product candidates; 

our ability to successfully commercialize any approved product candidates, including their rate and degree of 
market acceptance; 

whether we are able to maintain compliance with the terms and conditions of our loan and security 
agreement with Hercules Capital, Inc.; 

our ability to expand and protect our intellectual property estate; 

regulatory developments in the United States and other countries; 

the effectiveness of our internal control over financial reporting; 

the performance of our third-party suppliers, manufacturers and contract research organizations and our 
ability to obtain alternative sources of raw materials;  

our ability to attract and retain key personnel; and 

our ability to comply with regulatory requirements relating to our business, and the costs of compliance with 
those requirements, including those on data privacy and security. 

These and other risks and uncertainties are described in greater detail under the caption “Risk Factors” in Item 1A 

of Part I of this report and in other filings that we make with the Securities and Exchange Commission, or SEC. As a 
result of the risks and uncertainties, the results or events indicated by the forward-looking statements may not occur. We 
caution you not to place undue reliance on any forward-looking statement. 

In addition, any forward-looking statement in this Annual Report on Form 10-K represents our views only as of 
the filing date of this annual report and should not be relied upon as representing our views as of any subsequent date. 
We anticipate that subsequent events and developments may cause our views to change. Although we may elect to 
update these forward-looking statements publicly at some point in the future, we specifically disclaim any obligation to 
do so, except as required by applicable law. Our forward-looking statements do not reflect the potential impact of any 
future acquisitions, mergers, dispositions, joint ventures or investments we may make. 

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PART I 

All brand names or trademarks appearing in this report are the property of their respective holders. Use or 
display by us of other parties’ trademarks, trade dress, or products in this report is not intended to, and does not, imply 
a relationship with, or endorsements or sponsorship of, us by the trademark or trade dress owners. Unless the context 
requires otherwise, references in this report to “we,” “us,” and “our” refer to Albireo Pharma, Inc. and its direct and 
indirect subsidiaries. 

Item 1. 

BUSINESS 

Overview 

We are a biopharmaceutical company focused on the development and commercialization of novel bile acid 
modulators to treat orphan pediatric liver diseases and other liver or gastrointestinal diseases and disorders. We are 
pursuing the development of our lead product candidate, odevixibat (formerly known as A4250), for patients with 
progressive familial intrahepatic cholestasis, or PFIC, a rare, life-threatening genetic disorder affecting young children 
for which there is currently no approved drug treatment. In September 2020, we announced topline results from our 
Phase 3 trial in PFIC, and in December 2020, we announced that we submitted a new drug application, or NDA, to the 
U.S. Food and Drug Administration, or FDA, and a marketing authorization application, or MAA, to the European 
Medicines Agency, or EMA, seeking approval of odevixibat for the treatment of patients with PFIC, in anticipation of 
potential regulatory approval, issuance of a rare pediatric disease priority review voucher and commercial launch in the 
second half of 2021. In January 2021 we announced the FDA accepted our NDA for odevixibat for the treatment of 
patients with PFIC, which was submitted in November 2020, with priority review and a user fee goal date under the 
Prescription Drug User Fee Act (PDUFA) of July 20, 2021. We are also pursuing the development of odevixibat in 
biliary atresia and in Alagille syndrome, or ALGS, each of which is a rare, life threatening disease that affects the liver 
and for which there is no approved pharmacologic treatment option. We initiated a pivotal clinical trial of odevixibat in 
biliary atresia, the BOLD trial, in the first half of 2020, and continue to enroll patients in the trial. We expect topline 
results from the BOLD trial in 2024. We also initiated a pivotal trial of odevixibat in ALGS, the ASSERT trial, in the 
fourth quarter of 2020. We expect topline results from the ASSERT trial in 2022. Our most advanced product candidate 
in addition to odevixibat is elobixibat, which is approved in Japan for the treatment of chronic constipation. In 
August 2020, we announced topline results from our Phase 2 clinical trial as a treatment for nonalcoholic fatty liver 
disease, or NAFLD, and nonalcoholic steatohepatitis, or NASH, and based on the results of the trial, we decided not to 
pursue further development of elobixibat in NAFLD or NASH. We also have a preclinical program in adult liver and 
viral diseases. Our lead preclinical candidate for adult liver diseases, A3907, is a selective inhibitor of the apical sodium-
dependent bile acid transporter (ASBT) that has, based on animal studies, high predicted systemic bioavailability in man. 
As a result, A3907 has the potential to not only affect the bile acid pool by increased bile acid excretion in the stools but 
also through other pathways, including increased urinary bile acid excretion. This unique approach may yield greater 
dosing flexibility, greater efficacy and lower rates of adverse events associated with the category, such as diarrhea. We 
have completed IND enabling studies for A3907, plan to initiate a Phase 1 clinical trial in adult liver disease in the first 
half of 2021 and expect topline results in 2021. Our lead candidate for adult viral and liver diseases is A2342, an oral 
systemic sodium-taurocholate co-transporting peptide, or NTCP inhibitor, which is a potent small molecule that blocks 
entry of bile acids into the liver. We plan to complete IND enabling studies with A2342 this year. Preclinical efforts with 
other bile acid modulator approaches continue.  

Odevixibat —our lead product candidate for PFIC.     

In September 2020, we announced topline results from PEDFIC 1, our Phase 3 clinical trial for odevixibat, given 
once per day as an oral capsule or sprinkled over food, in children ages 6 months to 18 years with PFIC types 1 and 2, 
which was conducted at 45 global sites. PEDFIC 1 tested two doses of odevixibat, 40 µg/kg/day and 120 µg/kg/day, 
along with placebo, over a treatment period of 24 weeks. PEDFIC 1, met its two primary endpoints, demonstrating that 
odevixibat reduced serum bile acid responses, or sBAs, (p=0.003) and improved pruritus assessments (p=0.004) with a 
single digit diarrhea rate. In the primary analysis, PEDFIC 1 met the U.S regulatory primary endpoint with the 
proportion of positive pruritus assessments being 53.5% in the odevixibat arms compared to 28.7% in the placebo arm 
(p=0.004). As a secondary endpoint, 42.9% of patients in the odevixibat arms had a clinically meaningful improvement 

4 

 
in the pruritus score, defined as a drop from baseline of 1.0 point or more on the 0-4 point scale, at week 24 compared to 
10.5% in the placebo arm (p=0.018). PEDFIC 1 also met the E.U. regulatory primary endpoint with 33.3% of subjects in 
the odevixibat arms experiencing either a 70% reduction in sBAs or reaching a level of 70 µmol/L compared to no 
patients in the placebo arm (p=0.003). As an E.U. regulatory secondary endpoint, mean reduction of bile acids was 
114.3 µmol/L in the odevixibat arms compared to an increase of 13.1 µmol/L in the placebo arm (p=0.002). Both doses 
of odevixibat were statistically significant for each of the U.S. and E.U. primary endpoints. Odevixibat was well 
tolerated, with an overall adverse event incidence similar to placebo. There were no drug-related serious adverse events, 
or SAEs, reported during the study. Diarrhea/frequent bowel movements were the most common treatment-related 
gastrointestinal adverse events, which occurred in 9.5% of odevixibat treated patients vs. 5.0% of placebo patients. In 
December 2020, we announced that we submitted an NDA to the FDA and an MAA to the EMA seeking approval of 
odevixibat for the treatment of PFIC, which affirms our eligibility to apply for a rare pediatric disease priority review 
voucher. In January 2021 we announced the FDA accepted our NDA for odevixibat for the treatment of patients with 
PFIC, with priority review and a user fee goal date under the Prescription Drug User Fee Act (PDUFA) of July 20, 2021. 
In June 2018, the FDA granted a rare pediatric disease designation to odevixibat for the treatment of PFIC, and we 
applied for a rare pediatric disease priority review voucher in connection with the submission of our NDA in 
November 2020. In September 2018, the FDA granted fast track designation to odevixibat for the treatment of pruritus 
associated with PFIC. In July 2020, we initiated an Expanded Access Program (EAP) for odevixibat in the United States, 
Canada, Australia and Europe. 

PEDFIC 2, our long term, open label extension study, includes a cohort of patients who completed 24 weeks in 

PEDFIC 1 or moved into PEDFIC 2 after 12 weeks in PEDFIC 1, as well as an additional cohort of PFIC patients who 
were not eligible for PEDFIC 1. Patients in PEDFIC 2 receive odevixibat 120 µg/kg once per day over 72 weeks.  
Primary outcome measures in PEDFIC 2 are change in pruritus as indexed by caregiver reported observed scratching 
using our proprietary PRUCISION instrument, and change in sBAs, in each case from baseline over 72 weeks. In 
November 2020, we announced interim results from 69 patients through 24 weeks of treatment in PEDFIC 2 that show 
that the reductions in sBAs and/or pruritus observed in patients receiving odevixibat in PEDFIC 1 was maintained or 
increased during continued odevixibat treatment in PEDFIC 2. For patients who were treatment naïve to odevixibat 
(patients who received placebo in PEDFIC 1 or patients who enrolled directly into PEDFIC 2), reductions in sBAs and 
pruritus were similar to those observed during odevixibat treatment in PEDFIC 1.  Continued treatment with odevixibat 
in PEDFIC 2 resulted in increased growth rates and catch-up growth in children with PFIC.  Four patients with PFIC 
type 3 enrolled into PEDFIC 2 and had 12 weeks of data available at the time of the interim data cut; reductions in both 
sBAs and pruritus were also observed in these patients. No deaths or treatment related serious adverse events had been 
reported in PEDFIC 2 at the time of the interim data cut as of July 15, 2020. Odevixibat was generally well tolerated; 
diarrhea was reported in 10.1% of patients, all mild or moderate in severity.  

The precise prevalence of PFIC is unknown, and we are not aware of any patient registries or other method of 
establishing with precision the actual number of patients with PFIC in any geography. PFIC has been estimated to affect 
between one in every 75,000 children born worldwide. Based on the published incidence, published regional 
populations, and estimated median life expectancies, we estimate the prevalence of PFIC across the spectrum of the 
disease to be approximately 15,000 patients worldwide, not including China and India, but we are not able to estimate 
the prevalence of PFIC with precision.  We hold global rights to odevixibat unencumbered. Our current plan is to 
commercialize odevixibat ourselves in the United States and Europe, we have entered into license agreements with a 
partner in Israel and we are identifying potential partners for other regions. There are currently no drugs approved for the 
treatment of PFIC. First-line treatment for PFIC is typically off-label ursodeoxycholic acid, or UDCA, which is 
approved in France only for PFIC type 3, and in the United States and elsewhere for the treatment of primary biliary 
cholangitis, or PBC. However, many PFIC patients do not respond well to UDCA, undergo partial external bile 
diversion, or PEBD, surgery and often require liver transplantation. PEBD surgery is a life-altering and undesirable 
procedure in which bile is drained outside the body to a stoma bag that must be worn by the patient 24 hours a day. 

Other indications under development for odevixibat. 

We are also pursuing the development of odevixibat in patients with biliary atresia, another rare, life-threatening 

disease that affects the liver and for which there is no approved pharmacologic treatment option. In December 2018, the 
European Commission granted orphan designation to odevixibat for the treatment of biliary atresia, and in January 2019, 

5 

the FDA granted orphan drug designation to odevixibat for the treatment of biliary atresia. We initiated the BOLD 
clinical trial, a global pivotal trial and the largest prospective intervention trial ever conducted in biliary atresia, in the 
first half of 2020. The first patients have been enrolled in the trial, and we plan for full site activation in the first half of 
2021, subject to any potential impacts of COVID-19 on the ability of sites to complete required activities. We expect 
topline results from the BOLD trial in 2024. We believe biliary atresia is one of the most common rare pediatric liver 
diseases, and is the leading cause of liver transplants in children. Our double-blind, placebo controlled pivotal trial in 
biliary atresia is designed to enroll approximately 200 patients at 70 sites globally. Patients will receive either placebo or 
high-dose (120µg/kg) odevixibat once daily. The primary endpoint is survival with native liver after two years of 
treatment. 

Biliary atresia is a partial or total blocking or absence of large bile ducts that causes cholestasis and resulting 
accumulation of bile that damages the liver. The estimated worldwide incidence of biliary atresia is between 6 and 10 for 
every 100,000 live births. We estimate the prevalence of biliary atresia to be approximately 18,100 patients across the 
U.S. and Europe, and approximately 27,000 combined in other jurisdictions worldwide, but we are not able to estimate 
the prevalence of biliary atresia with precision. There are currently no drugs approved for the treatment of biliary atresia. 
The current standard of care is a surgery known as the Kasai procedure, or hepatoportoenterostomy, in which the 
obstructed bile ducts are removed and a section of the small intestine is connected to the liver directly. However, only an 
estimated 25% of those initially undergoing the Kasai procedure will survive to their twenties without need for liver 
transplantation. 

In addition, we initiated a pivotal trial of odevixibat in ALGS, the ASSERT trial, in the fourth quarter of 2020. The 

trial is expected to enroll approximately 45 patients aged 0 to 17 years of age with a genetically confirmed diagnosis of 
ALGS across 35 sites in North America, Europe, Middle East and Asia Pacific. We plan for full site activation in the 
first half of 2021, subject to any potential impacts of COVID-19 on the ability of sites to complete required activities. 
We expect topline data to be available in 2022, before the announcement of the topline results from the BOLD trial. 
ALGS is a genetic condition associated with liver, heart, eye, kidney and skeletal abnormalities. In particular, ALGS 
patients have fewer than normal bile ducts inside the liver, which leads to cholestasis and the accumulation of bile and 
causes scarring in the liver. ALGS is estimated to affect between one in every 50,000 children born worldwide. We 
estimate the prevalence of ALGS to be approximately 11,700 patients across the U.S. and Europe, and approximately 
13,000 combined in other jurisdictions worldwide, but we are not able to estimate the prevalence of ALGS with 
precision. There are currently no drugs approved for the treatment of ALGS. Current treatment for ALGS is generally in 
line with current treatments for PFIC as described above. In August 2012, the European Commission granted orphan 
designation to odevixibat for the treatment of ALGS. In October 2018, the FDA granted orphan drug designation to 
odevixibat for the treatment of ALGS. 

We continue to evaluate potential clinical development in other indications, including primary sclerosing 
cholangitis, which refers to swelling (inflammation), scarring, and destruction of bile ducts inside and outside of the 
liver. The first symptoms are typically fatigue, itching and jaundice, and many patients with sclerosing cholangitis also 
suffer from inflammatory bowel disease. The estimated incidence of primary sclerosing cholangitis is 9 cases per 
100,000 people. There are currently no drugs approved for the treatment of sclerosing cholangitis. First-line treatment is 
typically off-label UDCA, although UDCA has not been established to be safe and effective in patients with sclerosing 
cholangitis in well controlled clinical trials. 

Our Corporate History 

Prior to November 3, 2016, we were a specialty biopharmaceutical company known as Biodel Inc. that historically 
had been focused on the development and commercialization of innovative treatments for diabetes. Biodel was originally 
incorporated in the State of Delaware in December 2003 under the name “Global Positioning Group, Ltd.” and 
subsequently changed its name to “Biodel Inc.” Albireo Limited was formed in connection with a spinout transaction 
from AstraZeneca AB in 2008 in which AstraZeneca assigned to Albireo AB all of its rights in and to its portfolio of 
IBAT inhibitors, including elobixibat and odevixibat, as well as other programs that are currently at a preclinical stage. 

6 

On November 3, 2016, we completed a share exchange transaction with Biodel Inc, pursuant to an Amended and 

Restated Share Exchange Agreement dated July 13, 2016 that we entered into with Albireo Limited and the shareholders 
and noteholders of Albireo Limited. As a result, Albireo Limited became a wholly owned subsidiary of Biodel, Biodel’s 
corporate name was changed to Albireo Pharma, Inc. and the business of Albireo Limited became our business. 

The Role of Bile Acids and IBAT 

The liver is responsible for many vital body functions, including the regulation of bile acid synthesis and 

metabolism. The liver uses cholesterol to produce bile acids, which are then transported to, and stored in, the gall 
bladder. In response to food ingestion, the gall bladder contracts and releases bile acids into the small intestine where 
they promote digestion and absorption of dietary fats and fat soluble vitamins A, D, E and K. 

After completing digestion, bile acids bind to IBAT, which is sometimes referred to as the apical sodium bile acid 

transporter, or ASBT, at a location at the end of the small intestine known as the terminal ileum. As depicted below, 
IBAT then initiates the transport of bile acids across the intestinal wall through the portal vein back to the liver in the 
enterohepatic circulation process. 

In healthy persons, approximately 95% of bile acids recirculate back to the liver, with the remainder being 

excreted to the colon. The liver produces a small amount of new bile acids every day to make up for this loss. 

In addition to their role in digestion, bile acids are important signaling molecules that help regulate a network of 

metabolic pathways throughout the GI system. Bile acids bind to receptors in the colon that promote the release of 
intestinal hormones, such as glucagon-like peptide-1, or GLP-1, that can stimulate insulin release from the pancreas and, 
over time, decrease levels of plasma hemoglobin A1c, or HbA1c, a measure of glucose. In the liver, bile acids bind to 
other receptors that regulate bile acid production from cholesterol. Under normal conditions, bile acids bind to these 
receptors and inhibit the synthesis of new bile acids. As bile acid levels are lowered, the liver produces needed bile acids 
from cholesterol, which requires increased uptake of cholesterol and results in the decrease of cholesterol levels in the 
liver and otherwise in circulation in the body. 

Cholestatic liver disease results in the accumulation of elevated bile acids in the liver and in the serum. Elevated 

bile acid levels are linked with progressive liver disease. In addition, although a direct causative correlation has not been 
definitely established, there is substantial clinical support linking elevated serum bile acids to pruritus, a challenging 
symptom impacting patients with cholestatic liver disease. 

7 

 
Interruption of enterohepatic circulation in patients with PFIC or ALGS surgically via the partial external biliary 

diversion, or PEBD, procedure has been shown to lower serum bile acid levels, relieve pruritus, improve clinical 
outcomes and delay the progression of serious liver disease. Odevixibat is designed to treat PFIC and other cholestatic 
liver diseases pharmacologically by inhibiting IBAT to reduce bile acids in the liver and serum, while at the same time 
reducing pruritus. In addition to the beneficial effects that may be achievable through IBAT inhibition, odevixibat is 
minimally absorbed into the bloodstream, resulting in minimal systemic exposure of the drug to the body. 

Our Strategy 

Our goal is to be a leader in the development and commercialization of novel therapeutics for orphan pediatric 

cholestatic liver diseases and disorders where there is high unmet medical need, while also leveraging our expertise in 
bile acid modulation to treat other liver and GI diseases and disorders. To achieve our goal, we intend to pursue the 
following strategies. 

• 

Rapidly develop odevixibat to regulatory approval to treat patients with PFIC. We have announced results 
from our Phase 3 clinical trial in patients with PFIC, which we refer to as PEDFIC 1. It is our objective that 
PEDFIC 1, together with available data from PEDFIC 2, our long-term, open label extension study, forms 
the primary support for applications for marketing approval of odevixibat in both the United States and 
Europe, which were submitted in November 2020, as well as other key markets outside those jurisdictions. 
The EMA granted accelerated assessment to our MAA, and the FDA accepted our NDA with priority review 
and a user fee goal date under PDUFA of July 20, 2021. 

•  Maximize the benefit and commercial potential of odevixibat by expanding development to additional 
orphan pediatric cholestatic indications.  Although we have chosen PFIC as our lead indication for 
odevixibat, we also believe odevixibat can benefit children suffering from other cholestatic diseases and 
disorders. We initiated a pivotal clinical trial with odevixibat for the treatment of biliary atresia, the BOLD 
trial, in the first half of 2020 and continue to enroll patients in the trial. We estimate that biliary atresia 
impacts an aggregate of 18,100 patients in the United States and Europe, with another 27,000 patients in 
other jurisdictions worldwide, and is the cause of over 50% of liver transplants in children. We also initiated 
a pivotal trial for odevixibat as a treatment for ALGS, the ASSERT trial, in the fourth quarter of 2020.  

• 

• 

Develop the capability to commercialize odevixibat to treat orphan pediatric liver diseases, if approved, 
through a targeted sales force in the United States and Europe and collaborate selectively to 
commercialize odevixibat outside of these regions. If we receive marketing approval in the United States or 
Europe for odevixibat to treat patients with PFIC or any other pediatric cholestatic liver disease or disorder, 
we plan to build the capabilities to effectively commercialize odevixibat in the approved indication(s) in the 
applicable region. We believe that the required commercial organization would be modest in size and 
targeted to the relatively small number of specialists in the United States and Europe who treat children with 
cholestatic liver disease. If we receive marketing approval outside of the United States and Europe for 
odevixibat to treat patients with PFIC or any other pediatric cholestatic liver disease or disorder, we plan to 
selectively utilize collaboration, distribution and other marketing arrangements with third parties to 
commercialize odevixibat in the approved indication(s) in the regions or markets where we receive approval. 
We have entered into a Co-Promotion Agreement with Travere Therapeutics, Inc. for the co-promotion of 
odevixibat in the United States. The initial term of the arrangement is two years from launch of odevixibat, 
terminable at will by either party after one year following launch. 

Collaborate selectively to develop and commercialize product candidates targeting nonorphan indications, 
potentially including any future product candidate to treat adult liver diseases. We intend to selectively 
seek alliances and collaborations to assist us in furthering the development or commercialization of product 
candidates targeting large primary care markets that must be served by large sales and marketing 
organizations. These product candidates may include A3907, A2342 and any potential future product 
candidate that arises from our preclinical program in adult liver diseases. 

8 

Our Pipeline 

Odevixibat 

In September 2020, we announced topline results from our Phase 3 trial in PFIC, and in November 2020, we 
announced that we submitted an NDA to the FDA and an MAA to the EMA, seeking approval of odevixibat for the 
treatment of patients with PFIC, in anticipation of potential regulatory approval, issuance of a rare pediatric disease 
priority review voucher and commercial launch in the second half of 2021. In January 2021, we announced the FDA 
accepted our NDA, which was submitted in November 2020, and granted priority review with a user fee goal date under 
PDUFA of July 20, 2021. PEDFIC 2, our ongoing long term, open label extension study to evaluate long-term outcomes, 
includes a cohort of patients who completed 24 weeks in PEDFIC 1 or moved into PEDFIC 2 after 12 weeks in PEDFIC 
1, as well as an additional cohort of PFIC patients who were not eligible for PEDFIC 1.In addition to PFIC, we initiated 
a pivotal clinical trial in biliary atresia in the first half of 2020 and a pivotal trial for odevixibat as a treatment for ALGS 
in the fourth quarter of 2020.   

Odevixibat is a highly potent and selective inhibitor of IBAT that is designed to reduce bile acid reabsorption from 

the small intestine to the liver, and therefore reduce levels of bile acids in the serum and liver and increase excretion of 
bile acids via the colon. We believe that reducing liver and serum bile acid levels may reduce bile acid-related liver 
damage to improve liver function and alleviate symptoms of PFIC and other cholestatic liver diseases, including 
pruritus. Moreover, at therapeutic doses, odevixibat has minimal systemic exposure, acts locally in the gut and, based on 
preclinical testing, appears to be excreted substantially intact in the feces, which may reduce the risk of systemic side 
effects and undesirable drug-drug interactions compared with drugs that have broad distribution in the body. Odevixibat 
has been granted orphan drug designation for PFIC, PBC, biliary atresia and ALGS in the United States and the 
European Union.  

Lead Indication for Odevixibat 

PFIC. PFIC is our lead indication for odevixibat. PFIC is a rare genetic disorder that causes progressive, life-
threatening liver disease, which may start early after birth or at a young age and rapidly progress to end-stage liver 
disease. PFIC is commonly associated with elevated serum bile acids. Prominent symptoms of PFIC include pruritus, 
which is associated with severe sleep disturbance and diminished overall quality of life, and poor growth. First-line 
treatment in PFIC is typically off-label UDCA, which is approved in France only for PFIC type 3. UDCA is itself a type 
of bile acid that is thought to act by diluting the toxic effects in the liver and bile ducts of a different type of bile acids, 
known as hydrophobic bile acids, which are often elevated in cholestatic liver disease. Third-party retrospective analyses 
published in 2009 and 2010 indicate that, following treatment with UDCA, many PFIC patients require PEBD surgery 
and PFIC patients will often ultimately require liver transplantation. Although success rates vary, published third-party 

9 

 
studies have shown that PEBD surgery can slow and, in some cases, stop the progression of liver disease and lead to 
reduced pruritus and improved sleep. We believe these outcomes validate our approach of reducing liver and serum bile 
acids with an IBAT inhibitor such as odevixibat to treat PFIC. 

The precise prevalence of PFIC is unknown, and we are not aware of any patient registries or other method of 
establishing with precision the actual number of patients with PFIC in any geography. PFIC has been estimated to affect 
between one in every 75,000 children born worldwide. Based on the published incidence, published regional 
populations, and estimated median life expectancies, we estimate the prevalence of PFIC across the spectrum of the 
disease to be approximately 15,000 patients worldwide, not including China and India, but we are not able to estimate 
the prevalence of PFIC with precision. We hold global rights to odevixibat unencumbered. Our current plan is to 
commercialize odevixibat ourselves in the United States and Europe, we have entered into license agreements with a 
partner in Israel and are identifying potential partners for other regions. There are currently no drugs approved for the 
treatment of PFIC. First-line treatment for PFIC is typically off-label ursodeoxycholic acid, or UDCA, which is 
approved in France only for PFIC type 3, and in the United States and elsewhere for the treatment of primary biliary 
cholangitis, or PBC. However, many PFIC patients do not respond well to UDCA, undergo partial external bile 
diversion, or PEBD, surgery and often require liver transplantation. PEBD surgery is a life-altering and undesirable 
procedure in which bile is drained outside the body to a stoma bag that must be worn by the patient 24 hours a day. 
Three alternative gene defects have been identified that correlate to three separate PFIC subtypes, known as types 1, 2 
and 3. 

PFIC, type 1, which is sometimes referred to as “Byler disease” or “FIC1 deficiency,” is caused by impaired bile 
secretion due to mutations in the ATP8B1 gene that result in an imbalance of molecules known as phospholipids that is 
associated with cholestasis and elevated bile acids in the liver. Children affected by PFIC, type 1 usually develop 
cholestasis in the first months of life and, in the absence of surgical treatment, progress to cirrhosis and end-stage liver 
disease before the end of the first decade of life. PFIC, type 1 is especially common in the Old Order Amish population 
in the United States, as well as the Inuit population of Greenland. 

• 

• 

PFIC, type 2, which is sometimes referred to as “Byler syndrome” or “BSEP deficiency,” is caused by 
impaired bile salt secretion due to mutations in the ABCB11 gene that result in the buildup of bile salts in 
liver cells. Children with PFIC, type 2 often develop liver failure within the first few years of life and are at 
increased risk of developing hepatocellular carcinoma, the most common form of liver cancer. 

PFIC, type 3, which typically presents in the first years of childhood with progressive cholestasis, is caused 
by mutations in the ABCB4 gene. Mutations in the ABCB4 gene lead to a lack of phospholipids available to 
bind to bile acids, resulting in a buildup of bile acids that damages liver cells. 

The TJP2 gene, NR1H4 gene or Myo5b gene mutations have also been proposed to be causes of PFIC. In addition, 

some patients with PFIC do not have a mutation in any of the ATP8B1, ABCB11, ABCB4, TJP2, NR1H4 or Myo5b 
genes. In these cases, the cause of the condition is unknown. 

Biliary Atresia Indication for Odevixibat 

Biliary atresia. Biliary atresia is a partial or total blocking or absence of large bile ducts that irreversibly prevents 

bile flow from the liver to the small intestine, causing cholestasis and resulting accumulation of bile that damages the 
liver. The damage leads to scarring, loss of liver tissue and cirrhosis, which makes it difficult for the liver to remove 
toxins from the blood and deteriorates the liver. Biliary atresia is life threatening. 

There are currently no drugs approved for the treatment of biliary atresia. The current standard of care is the Kasai 

procedure. The chance of a successful Kasai procedure is highest if performed before a patient is two months of age. 
However, even with early intervention, scarring of the liver can continue, resulting in cirrhosis and eventually the need 
for transplantation. Only an estimated 25% of those initially undergoing the Kasai procedure will survive to their 
twenties without need for liver transplantation. 

10 

The estimated worldwide incidence of biliary atresia is between 6 and 10 for every 100,000 live births. Of all 

biliary atresia patients, we believe odevixibat will primarily benefit those who have undergone a Kasai procedure that 
has been sufficiently successful to obviate the need for liver transplant within the first year of life. We estimate the 
prevalence of biliary atresia to be approximately 18,100 patients across the U.S. and Europe, and 27,000 combined in 
other jurisdictions worldwide, but we are not able to estimate the prevalence of biliary atresia with precision.  

The exact cause of biliary atresia is unknown, but it is thought to result from an event in the womb around the time 

of birth. Possible triggers may include viral or bacterial infection, an immune system malfunction, a genetic mutation, a 
problem during liver or bile duct development or exposure to toxic substances. In January 2019, the FDA granted orphan 
designation to odevixibat for the treatment of biliary atresia. The European Commission granted orphan drug designation 
to odevixibat for the treatment of biliary atresia in December of 2018.   We initiated the BOLD clinical trial, a global 
pivotal trial in biliary atresia, in the first half of 2020. Our double-blind, placebo controlled pivotal trial is designed to 
enroll approximately 200 patients at 70 sites globally. Patients will receive either placebo or 120µg/kg odevixibat once 
daily. The primary endpoint is survival with native liver after two years of treatment. The first patients have been 
enrolled in the trial, and we plan for full site activation in the first half of 2021, subject to any potential impacts of 
COVID-19 on the ability of sites to complete required activities. We anticipate having topline results available from this 
trial in 2024. 

ALGS. ALGS is a genetic condition associated with liver, heart, eye, kidney and skeletal abnormalities. In 

particular, ALGS patients have fewer than normal bile ducts inside the liver, which leads to cholestasis and the 
accumulation of bile and causes scarring in the liver. Symptoms include jaundice, pruritus, poor growth and specific 
facial features and typically develop in the first two years of life. 

We estimate the prevalence of ALGS to be approximately 11,700 patients across the U.S. and Europe, and 

approximately 13,000 combined in other jurisdictions worldwide, but we are not able to estimate the prevalence of 
ALGS with precision. There are currently no drugs approved for the treatment of ALGS. Current treatments for ALGS 
are generally in line with current treatments for PFIC as described above, including off-label UDCA, PEBD surgery and, 
where liver disease is advanced, liver transplantation.  

The estimated worldwide incidence of ALGS is one in every 50,000 newborns born worldwide. ALGS is 
predominately caused by mutations in a gene called Jagged1. In a small number of cases, ALGS results from mutations 
in a gene called Notch2. In October 2018, the FDA granted orphan drug designation to odevixibat for the treatment of 
ALGS. In the fourth quarter of 2020, we initiated the ASSERT global pivotal trial for odevixibat as a treatment for 
ALGS. We plan for full site activation in the first half of 2021, subject to any potential impacts of COVID-19 on the 
ability of sites to complete required activities. We expect topline data to be available in 2022, before the announcement 
of the topline results from the BOLD clinical trial. 

Potential Additional Target Indications for Odevixibat 

Primary Sclerosing cholangitis. Primary sclerosing cholangitis refers to swelling (inflammation), scarring, and 
destruction of bile ducts inside and outside of the liver. The first symptoms are typically fatigue, itching and jaundice, 
and many patients with sclerosing cholangitis also suffer from inflammatory bowel disease. The estimated incidence of 
primary sclerosing cholangitis is 9 cases per 100,000 people. There are currently no drugs approved for the treatment of 
sclerosing cholangitis. First-line treatment is typically off-label UDCA, although UDCA has not been established to be 
safe and effective in patients with primary sclerosing cholangitis in well controlled clinical trials. 

Development of Odevixibat 

Phase 3 Clinical Program in Patients with PFIC 

In September 2020, we announced topline results from PEDFIC 1, our Phase 3 clinical trial for odevixibat, given 

once per day as an oral capsule or sprinkled over food, in patients ages 6 months to 18 years with PFIC types 1 and 2, 
which was conducted at 33 global sites. PEDFIC 1 tested two doses of odevixibat, 40 µg/kg/day and 120 µg/kg/day, 

11 

along with placebo, over a treatment period of 24 weeks. PEDFIC 1, met its two primary endpoints, demonstrating that 
odevixibat reduced serum bile acid responses, or sBAs, (p=0.003) and improved pruritus assessments (p=0.004) with a 
single digit diarrhea rate. In the primary analysis, PEDFIC 1 met the U.S. regulatory primary endpoint with the 
proportion of positive pruritus assessments being 53.5% in the odevixibat arms compared to 28.7% in the placebo arm 
(p=0.004). As a secondary endpoint, 42.9% of patients in the odevixibat arms had a clinically meaningful improvement 
in the pruritus score, defined as a drop from baseline of 1.0 point or more on the 0-4 point scale, at week 24 compared to 
10.5% in the placebo arm (p=0.018). PEDFIC 1 also met the E.U. regulatory primary endpoint with 33.3% of subjects in 
the odevixibat arms experiencing either a 70% reduction in sBAs or reaching a level of 70 µmol/L compared to no 
patients in the placebo arm (p=0.003). As an E.U. regulatory secondary endpoint, mean reduction of bile acids was 114.3 
µmol/L in the odevixibat arms compared to an increase of 13.1 µmol/L in the placebo arm (p=0.002). Both doses of 
odevixibat were statistically significant for each of the U.S. and E.U. primary endpoints. Odevixibat was well tolerated, 
with an overall adverse event incidence similar to placebo. There were no drug-related serious adverse events, or SAEs, 
reported during the study. Diarrhea/frequent bowel movements were the most common treatment-related gastrointestinal 
adverse events, which occurred in 9.5% of odevixibat treated patients vs. 5.0% of placebo patients. In November 2020, 
we submitted an NDA to the FDA and an MAA to the EMA seeking approval of odevixibat for the treatment of PFIC, 
which affirms our eligibility to apply for a rare pediatric disease priority review voucher. In June 2018, the FDA granted 
a rare pediatric disease designation to odevixibat for the treatment of PFIC, and in September 2018, the FDA granted fast 
track designation to odevixibat for the treatment of pruritus associated with PFIC. In July 2020, we initiated an 
Expanded Access Program (EAP) for odevixibat in the United States, Canada, Australia and Europe. In addition, we 
applied for a rare pediatric disease priority review voucher in connection with the submission of our NDA for odevixibat 
in November of 2020.  

PEDFIC 2, our long term, open label extension study, includes a cohort of patients who completed 24 weeks in 

PEDFIC 1 or moved into PEDFIC 2 after 12 weeks in PEDFIC 1, as well as an additional cohort of PFIC patients who 
were not eligible for PEDFIC 1. Patients in PEDFIC 2 receive odevixibat 120 µg/kg once per day over 72 weeks.  
Primary outcome measures in PEDFIC 2 are change in pruritus as indexed by caregiver reported observed scratching 
using our proprietary PRUCISION instrument, and change in sBAs, in each case from baseline over 72 weeks. Interim 
data from 69 patients through 24 weeks of treatment in PEDFIC 2 was presented at the November 2020 AASLD. The 
interim results show that the reductions in sBAs and/or pruritus observed in patients receiving odevixibat in PEDFIC 1 
was maintained or increased during continued odevixibat treatment in PEDFIC 2. For patients who were treatment naïve 
to odevixibat (patients who received placebo in PEDFIC 1 or patients who enrolled directly into PEDFIC 2), reductions 
in sBAs and pruritus were similar to those observed during odevixibat treatment in PEDFIC 1.  Continued treatment with 
odevixibat in PEDFIC 2 resulted in increased growth rates and catch-up growth in children with PFIC.  Four patients 
with PFIC type 3 enrolled into PEDFIC 2 and had 12 weeks of data available at the time of the interim data cut; 
reductions in both sBAs and pruritus were also observed in these patients. No deaths or treatment related serious adverse 
events had been reported in PEDFIC 2 at the time of the interim data cut as of July 15, 2020. Odevixibat was generally 
well tolerated; diarrhea was reported in 10.1% of patients, all mild or moderate in severity.  

Elobixibat 

Our product candidate elobixibat is licensed for the treatment of chronic constipation and other functional diseases 

in Japan and other select markets in Asia to EA Pharma. In January 2018, the Japanese Ministry of Health, Labour and 
Welfare, or MHLW, approved a new drug application filed by EA Pharma for elobixibat for the treatment of chronic 
constipation in Japan. EA Pharma co-markets elobixibat in Japan with another company, Mochida, and co-promotes 
elobixibat with Eisai in Japan under the trade name GOOFICE®. 

We have commercial rights to elobixibat in the United States, Europe and all other territories not licensed to EA 

Pharma. We do not have any current plan to seek a license or other partnering transaction with a third party for 
elobixibat for chronic constipation in the United States and Europe. Whether or not we elect to seek such a transaction, 
we do not anticipate that we will conduct future clinical trials of elobixibat as a treatment for chronic constipation 
independently. 

12 

Elobixibat is, like odevixibat, an IBAT inhibitor. In August 2020, we announced topline results from our Phase 2 
clinical trial as a treatment for NAFLD and NASH and based on the results of the trial, we decided not to pursue further 
development of elobixibat in NAFLD or NASH.  

Chronic (Idiopathic) Constipation 

Though occasional constipation is very common, some people experience chronic constipation that can interfere 

with their ability to go about their daily tasks. Pursuant to applicable Rome III diagnostic criteria, two or more of the 
following symptoms must be present: straining during at least 25% of defecations, lumpy or hard stools in at least 25% 
of defecations, sensation of incomplete evacuation for at least 25% of defecations, sensation of rectal obstruction or 
blockage for at least 25% of defecations, use of fingers or other manual maneuvers to facilitate at least 25% of 
defecations, and passing fewer than three stools per week. In order to support a diagnosis of constipation, the patient 
must rarely have loose stools without the use of laxatives. Further, in order for constipation to be considered chronic, 
these criteria must be present for at least three months, with symptom onset at least six months prior to diagnosis. The 
Rome III diagnostic criteria are established diagnostic measures for various GI disorders set forth by the Rome 
Foundation, a not-for-profit organization based in the United States. The term “idiopathic” indicates that the cause of the 
chronic constipation is unknown and not due to any underlying illness or medication. 

The current standard of care for constipation is over-the-counter, or OTC, laxatives, which may improve 

symptoms of constipation but often exacerbate pain and bloating. Marketed products in Japan that may be prescribed for 
chronic constipation include Amitiza (lubiprostone) and Linzess (linaclotide), although linaclotide is approved in Japan 
for the treatment of IBS-C. 

Preclinical and Early Clinical Development of Elobixibat 

Prior to Albireo Limited’s inception in 2008, elobixibat was evaluated by its predecessor owner, AstraZeneca, in 

various preclinical studies and Phase 1 single ascending dose and multiple ascending dose clinical trials. In Phase 1 
clinical development, elobixibat was generally well tolerated in healthy volunteers and showed minimal systemic 
exposure. Subsequently, we conducted a two-year nonclinical carcinogenicity toxicology study that did not result in any 
findings of concern and we or our former licensee conducted various additional Phase 1 and early Phase 2 clinical trials 
to assess the pharmacokinetics and various effects of elobixibat. Findings from these studies indicated, among other 
things, favorable effects of elobixibat on colonic transit and on low-density lipoprotein, or LDL or “bad” cholesterol. 

Completed Phase 2b Clinical Trial in the United States 

We completed a multicenter, double blind, placebo controlled Phase 2b clinical trial of elobixibat as a treatment 
for chronic idiopathic constipation, or CIC, in 2010. We conducted the trial at 45 sites in the United States. Enrollment 
criteria included a diagnosis of CIC and meeting specified thresholds for numbers of complete spontaneous bowel 
movements, or CSBMs, per week during the two weeks prior to randomization. A spontaneous bowel movement, or 
SBM, was defined in the trial as a bowel movement occurring without a laxative, enema or suppository usage in the past 
24 hours. A CSBM was defined in the trial as an SBM accompanied by a self-report of complete evacuation. 

After a screening period during which patients were taken off laxatives and other excluded medications, patients in 

the trial entered a two-week baseline period. Following the baseline period, 190 patients were randomized to receive a 
once daily oral tablet dose of one of three doses of elobixibat (5, 10 or 15 mg), or placebo, for eight weeks. Of the 
randomized patients, 161 patients completed the trial. 

The primary endpoint of the trial was change in number of weekly SBMs from baseline to the first treatment week 

for patients who received elobixibat compared with patients who received a placebo. The results demonstrated a dose 
response in favor of elobixibat among all three dose groups and were statistically significant in the 10 mg (p < 0.002) 
and 15 mg (p < 0.001) dose groups. A clinical trial result is statistically significant if it is unlikely to have occurred by 
chance. The statistical significance of a clinical trial result, such as an observed difference between two treatment groups 
or cohorts, is determined by a widely used statistical method that establishes the “p”-value of the result. A p-value of 

13 

0.05 (or less) indicates that there is a 1-in-20 (or less) statistical probability that the clinical trial result occurred by 
chance and typically represents statistical significance. If a p-value is above 0.05, the result is generally not considered 
statistically significant. The p-value of < 0.002 for the 10 mg elobixibat dose group indicates that there is a less 
than 1-in-500 statistical probability that the difference compared with placebo occurred by chance, and the p-value of 
< 0.001 for the 15 mg elobixibat dose group indicates that there is a less than 1-in-1,000 statistical probability that the 
difference compared with placebo occurred by chance. 

Secondary efficacy endpoints of the trial included evaluations of changes in mean weekly number of SBMs and 

CSBMs, time to first SBM or CSBM, overall constipation response and reduction in C4 and LDL cholesterol levels. The 
10 mg and 15 mg elobixibat doses met all of these secondary endpoints with statistical significance. 

All doses of elobixibat were generally well tolerated in the clinical trial. The most frequently reported adverse 
events in the trial were abdominal pain and diarrhea, which occurred most often in the highest elobixibat dose group 
(abdominal pain: 10.4%, 5 mg elobixibat; 10.6%, 10 mg elobixibat; and 27.1%, 15 mg elobixibat; versus 0% placebo; 
and diarrhea: 8.3%, 5 mg elobixibat; 6.4%, 10 mg elobixibat; and 12.5%, 15 mg elobixibat; versus 2.2% placebo). None 
of the three SAEs reported in the trial (bleeding colonic diverticulum two weeks after the end of treatment in the 5 mg 
elobixibat dose group, breast carcinoma in the 10 mg elobixibat dose group, and shoulder pain in the placebo group) was 
considered by the applicable investigator to be related to study drug. 

Completed Phase 2b Clinical Trial Conducted by EA Pharma in Japan 

Our licensee, EA Pharma, completed a multicenter, double blind, placebo controlled Phase 2b clinical trial of 
elobixibat as a treatment for chronic constipation in 2015. Patients in the trial entered the two-week baseline period 
during which they were taken off excluded medications. Following the baseline period, patients were randomized to 
receive a once daily oral dose of either a low, mid or high dose of elobixibat for two weeks. During the baseline period 
and the treatment period, patients reported daily bowel and abdominal symptoms. Of the randomized patients, 154 
patients completed the trial. 

The primary endpoint of the trial was change in number of weekly SBMs from baseline to the first treatment week 

for patients who received elobixibat compared with patients who received a placebo. In the trial, both the mid and high 
dose groups of elobixibat showed a highly statistically significant advantage on change from baseline in weekly SBM 
frequency compared with placebo (p < 0.001). The findings in favor of elobixibat were substantially the same on a 
secondary endpoint of the trial assessing change from baseline in weekly CSBM frequency. 

All doses of elobixibat were generally well tolerated in the trial, and no SAEs were reported. As in our completed 
Phase 2b clinical trial, the most frequently reported adverse events in the trial were abdominal pain and diarrhea, which 
were both assessed by EA Pharma to be typically mild. 

Completed Phase 3 Clinical Trial and Long-Term Safety Trial Conducted by EA Pharma in Japan 

In October 2016, we announced positive results from a Phase 3 clinical trial of elobixibat as a treatment for 
chronic constipation conducted by EA Pharma in Japan. The trial was a multicenter, double blind, placebo controlled 
trial in which patients with chronic constipation received a fixed dose of elobixibat or placebo once daily for two weeks. 
In the trial, elobixibat met the primary endpoint, which was change in the number of weekly SBMs from baseline to the 
first treatment week compared with placebo, with high statistical significance. Elobixibat also met all secondary efficacy 
endpoints in the trial assessed statistically, including assessments of change in frequency of CSBMs, time to first SBM, 
severity of constipation and stool consistency, with high statistical significance. 

There were no SAEs reported in the trial. Consistent with prior clinical trials of elobixibat, the most common 

adverse events were abdominal pain (18.8%) and diarrhea (13.0%), all of which were characterized as mild or, in one 
case, moderate in severity. 

14 

EA Pharma also completed a clinical trial designed to evaluate the long-term safety of elobixibat in Japanese 
patients with chronic constipation over 52 weeks. The long-term safety trial was a multicenter, open label trial in which 
patients with chronic constipation received once daily dosing of elobixibat. In the trial, elobixibat showed a safety and 
tolerability profile consistent overall with prior clinical trials of elobixibat in Japan. 

Phase 3 Clinical Trials Conducted by a Former Licensee 

Two Phase 3 clinical trials conducted by a former licensee of ours to evaluate the efficacy and safety of elobixibat 

as a treatment for CIC, known as Echo 1 and Echo 2, ended in 2014. Our former licensee stopped Echo 1 and Echo 2 
early citing an issue related to the distribution of study drug to study sites that was unrelated to the performance of 
elobixibat. Subsequent analysis by our former licensee determined the issue to have affected only Echo 2 and a small 
number of patients. As a result of the early termination of the trials, each of Echo 1 and Echo 2 enrolled substantially 
fewer than the number of patients contemplated by the trial’s statistical plan. A third Phase 3 clinical trial conducted by 
our former licensee to evaluate the long-term safety of elobixibat, known as Echo 3, ended in 2015. 

Echo 1. Echo 1 was a multicenter, double blind, placebo controlled Phase 3 clinical trial of elobixibat as a 
treatment for CIC conducted at 71 sites in the United States, Belgium, Canada, Czech Republic, Germany, Israel, the 
United Kingdom, Poland and South Africa. SBM and CSBM were defined in the trial in the same manner as our 
completed Phase 2b trial of elobixibat in CIC discussed above. The statistical plan for the trial contemplated that 
840 patients would be enrolled. 

After a screening period, patients in the trial entered a two-week baseline period. Following the baseline period, 
patients were randomized in a 1:1:1 ratio to receive a once daily oral dose of 5 mg of elobixibat, 10 mg of elobixibat or 
placebo, in tablet form, for 26 weeks. During the baseline period and the treatment period, patients reported daily bowel 
and abdominal symptoms. At the time the trial was stopped, 376 patients had been randomized into the trial, of which 
312 patients had completed 12 weeks of treatment and 146 patients had completed 26 weeks of treatment. 

The primary endpoint of the trial was the overall CSBM response. CSBM response refers to a patient having at 

least three CSBMs per week and an increase of at least one CSBM per week from baseline, in each case for at least nine 
of the first 12 weeks of the treatment period and at least three of the weeks from week 9 to week 12. The 5 mg elobixibat 
dose met the primary endpoint, and the result was statistically significant based on the study’s predefined statistical 
methodology (p = 0.029). There was a trend in favor of the 10 mg elobixibat dose on the primary endpoint, but the result 
did not achieve statistical significance using the same methodology. Subsequently, at a meeting with the FDA held in 
2014, the FDA advised us that, based on the unplanned stopping of the study, the FDA would apply a different statistical 
methodology than had been predefined and utilized for the study. Using the FDA’s chosen statistical methodology, 
neither the 5 mg nor the 10 mg dose of elobixibat achieved statistical significance on the primary endpoint in Echo 1. 

All doses of elobixibat were generally well tolerated in the trial. The rate of discontinuation due to adverse events 

was dose related (7%, 5 mg elobixibat and 9%, 10 mg elobixibat, versus 2% placebo). There were dose-related 
incidences of treatment-emergent abdominal pain and diarrhea considered a reasonable possibility to be treatment related 
(abdominal pain: 4%, 5 mg elobixibat and 12%, 10 mg elobixibat, versus 2% placebo; diarrhea: 6%, 5 mg elobixibat and 
6%, 10 mg elobixibat, versus 2% placebo). None of the three SAEs reported in the 5 mg elobixibat dose group, two 
SAEs reported in the 10 mg elobixibat dose group and one SAE reported in the placebo group were considered by the 
applicable investigator to be related to study drug. The reported SAEs were: in the 5 mg elobixibat dose group, 
inflammation of the gallbladder, developmental bone growth disease and carpal tunnel syndrome; in the 10 mg 
elobixibat dose group, glaucoma and back pain worsening; and in the placebo group, hemorrhoids. 

Echo 2. Echo 2 was a multicenter, double blind, placebo controlled Phase 3 clinical trial of elobixibat as a 

treatment for CIC. The trial was conducted at 79 sites in the United States, Canada, Czech Republic, Germany, Hungary, 
Poland, Slovakia, Sweden, South Africa and the United Kingdom. Enrollment criteria, primary and key secondary 
endpoints and trial design were substantially the same as for Echo 1, except that Echo 2 from the outset provided for a 
12-week treatment period and included a four-week post-treatment withdrawal period. The statistical plan for the trial 
contemplated that 840 patients would be enrolled. 

15 

Following the screening and baseline periods, patients were randomized in a 1:1:1 ratio to receive a once daily oral 

dose of 5 mg of elobixibat, 10 mg of elobixibat or placebo, in tablet form. During the baseline period and the treatment 
period, patients reported daily bowel and abdominal symptoms. At the time the trial was stopped, 314 patients had been 
randomized into the trial, of which 219 patients completed the trial including the withdrawal period. 

In the trial, there were trends in favor of both the 5 mg and 10 mg elobixibat doses compared with placebo on the 

primary endpoint, but the result did not reach statistical significance. There were no signs of rebound during the four-
week withdrawal period after the treatment period. 

All doses of elobixibat were generally well tolerated in the trial. The rate of discontinuation due to adverse events 
was the same in the 5 mg elobixibat and placebo dose groups (2%) and greater in the 10 mg elobixibat dose group (6%). 
There were dose-related incidences of abdominal pain and diarrhea considered a reasonable possibility to be treatment 
related (abdominal pain: 2%, 5 mg elobixibat and 8%, 10 mg elobixibat, versus 2% placebo; diarrhea (7%, 5 mg 
elobixibat and 9%, 10 mg elobixibat, versus <1% placebo). There was one SAE reported in the 5 mg elobixibat dose 
group compared with five SAEs in the placebo group. The reported SAE in the 5 mg elobixibat dose group (basal cell 
carcinoma on the lip) occurred during the withdrawal period and was not considered by the applicable investigator to be 
related to study drug. The SAEs reported in the placebo group were tonsillitis, abnormal uterine bleeding, noncancerous 
uterine tumor, hysterectomy and exacerbation of hypertension. 

Long-Term Safety. The long-term safety trial was a multicenter, open label, Phase 3 extension clinical trial of 
elobixibat as a treatment for CIC. The trial was conducted at 62 sites in the United States, Belgium, Canada, Czech 
Republic, Hungary, Poland, Slovakia, South Africa, Sweden and the United Kingdom. Enrollment criteria included 
completion of at least 12 weeks of double blind treatment in either Echo 1 or Echo 2. The trial enrolled 411 patients. 
Patients received a 10 mg dose of elobixibat in tablet form once daily, subject to reduction to 5 mg in the discretion of 
the applicable investigator, for up to 52 weeks. Of these patients, 282 patients completed 52 weeks of treatment with 
elobixibat and 316 patients completed at least 24 weeks of treatment with elobixibat. 

There were several co-primary endpoints in the trial, all related to safety. In the trial, elobixibat was generally well 

tolerated, with a safety profile similar to Echo 1 and Echo 2. In particular, there was only one treatment-emergent SAE 
reported (constipation) that was considered by the applicable investigator to be related to study drug. Treatment-
emergent adverse events leading to discontinuation occurred in 6.1% of patients, and the majority of treatment-emergent 
adverse events overall were classified as mild or moderate. Most adverse events were classified as GI. 

U.S.  Phase 2 Trial in NAFLD/NASH 

We initiated a double-blind, placebo controlled Phase 2 trial which enrolled 47 patients across 15 U.S. sites, and 

was designed to assess the safety and efficacy of elobixibat 5 mg in NASH/NAFLD patients over 16 weeks, as measured 
by liver steatosis and various biomarkers. The primary endpoint of the trial was an assessment of the change in LDL-
cholesterol, and the secondary endpoints were an assessment in the change in liver fat by imaging, AST and ALT. 
Exploratory endpoints included measures of glucose and insulin homeostasis, which are biomarkers for inflammation 
and fibrosis. In August 2020, we announced that the trial achieved the primary endpoint of reduction of low-density 
lipoprotein cholesterol (LDL-C) but did not achieve proof-of- concept for other key NASH measures. Elobixibat 
achieved the primary endpoint of a reduction in LDL-C with a clinically meaningful effect of -20.5 mg/dL in serum 
LDL-C compared to -11.1 mg/dL, elobixibat vs. placebo (p<0.022). These reductions were in addition to lipid lowering 
treatment where 56.5% of the elobixibat and 45.8% of the placebo patients were on stable lipid lowering therapy at 
baseline. Liver fat reduction in the elobixibat group was -2.6% as measured by MRI PDFF. The baseline alanine 
aminotransferase (ALT) levels were within the normal range and there was no meaningful change observed in the study. 
Overall elobixibat was well tolerated, with adverse event incidence similar to placebo and with no serious adverse events 
or discontinuations due to treatment. The study showed an acceptable gastrointestinal tolerability with 3 of 23 elobixibat 
patients reporting mild to moderate transient diarrhea attributable to drug treatment. Based on the results of the trial, we 
decided not to pursue further development of elobixibat in NAFLD or NASH. 

16 

Preclinical Program 

A3907 

We also have a preclinical program in adult liver and viral diseases. Our lead preclinical candidate for adult liver 
diseases, A3907, is a selective inhibitor of the apical sodium-dependent bile acid transporter (ASBT) that has, based on 
animal studies, high   predicted systemic bioavailability in man. As a result, A3907 has the potential to not only affect 
the bile acid pool by increased bile acid excretion in the stools but also through other pathways, including increased 
urinary bile acid excretion. This unique approach may yield greater dosing flexibility, greater efficacy and lower rates of 
adverse events associated with the category, such as diarrhea. We have completed IND enabling studies for A3907, plan 
to initiate a Phase 1 clinical trial in adult liver disease in the first half of 2021 and expect topline results in 2021. 

A2342 

Our lead preclinical candidate for adult viral and liver diseases, A2342, is an oral systemic sodium-taurocholate 

co-transporting peptide, or NTCP inhibitor, which is a potent small molecule that blocks entry of bile acids into the liver. 
We plan to complete IND enabling studies with A2342 this year.  Preclinical efforts with other bile acid modulator 
approaches continue. 

License and Royalty Monetization Agreements 

Agreement with EA Pharma 

We entered into a license agreement with EA Pharma (formerly known as Ajinomoto Pharmaceuticals Co., Ltd.) 

for the development and commercialization of elobixibat in specified countries in Asia in April 2012.  

Pursuant to the agreement, we granted EA Pharma an exclusive license under patents and other technology owned 
or licensed by us to develop and commercialize elobixibat in Japan and certain Asian Pacific markets for all prophylactic 
or therapeutic uses of a pharmaceutical product for specified GI diseases and disorders, symptoms of constipation of all 
causes, or postoperative ileus or for use in colonoscopy cleansing procedures. The agreement also provides that the 
scope of the license may be expanded to include specified liver diseases, if we or an affiliate or licensee takes specified 
development actions outside of EA Pharma’s licensed territory with elobixibat in that specified liver disease, or files an 
application for regulatory approval of elobixibat outside of EA Pharma’s licensed territory for that specified liver 
disease, or otherwise approves that EA Pharma conduct a clinical trial in that specified liver disease. 

Payment Terms. As of February 15, 2021, we have received $49.9 million in upfront and milestone payments from 

EA Pharma under the agreement. We are eligible to receive additional amounts of up to $5.3 million if a specified 
regulatory event is achieved for elobixibat. In addition, subject to the terms of the royalty interest acquisition agreement, 
or RIAA, with HealthCare Royalty Partners III, L.P., or HCR, described below, we may become eligible under the 
license agreement to receive up to $29.1 million if specified sales milestones are achieved for elobixibat and we also 
receive stepped royalties beginning in the high single digits on all future net sales of elobixibat.  

EA Pharma’s obligation under the license agreement to pay royalties for elobixibat expires on a country-by-

country basis on the later of expiration of the patent rights in a country that have a specified scope and that we either 
licensed to EA Pharma or, subject to a specified term limit, are developed by EA Pharma, alone or together with us, in 
the course of its activities under the agreement or expiration of regulatory exclusivity for elobixibat in that country. The 
Japanese patent rights with respect to elobixibat that we licensed to EA Pharma expire between 2026 and 2029. In 
addition, we have two pending patent applications on specific crystal polymorphs of elobixibat that, if issued in Japan, 
will expire in 2034 and 2035, respectively. Royalty rates are subject to reduction under the agreement in specified 
circumstances, including in any country if elobixibat is subject to generic competition that exceeds a specified level, if 
the bulk price for unformulated elobixibat purchased from us for use in Japan exceeds a specified threshold or if EA 
Pharma licenses patent rights from any third party under circumstances where it is legally required to do so to 
commercialize elobixibat in its licensed field in a particular country in its licensed territory. 

17 

 
 
 
Development and Commercialization. EA Pharma is responsible for funding and using commercially reasonable 

efforts to execute the development and commercialization of elobixibat in its licensed field and licensed territory 
pursuant to agreed territory development and commercialization plans that are updated from time to time. In Japan, EA 
Pharma co-markets elobixibat with Mochida pursuant to a sublicense agreement and co-promotes elobixibat with Eisai. 
A joint development committee and a joint commercialization committee, each comprising representatives of each 
company, oversees activities under the agreement. 

EA Pharma is responsible for commercial manufacture and supply of elobixibat in its licensed territory. 

Restrictions. EA Pharma is not permitted to conduct clinical development or commercialize elobixibat outside of 

its licensed field of use or licensed territory. We are not permitted to commercialize elobixibat for any field of use in EA 
Pharma’s licensed territory. In addition, if we determine to develop elobixibat in a liver disease outside of EA Pharma’s 
licensed territory, our development is subject to specified restrictions on clinical trial design. After the first commercial 
sale of elobixibat in any country in EA Pharma’s licensed field, neither we nor EA Pharma may commercialize a 
different product for the treatment of chronic constipation or IBS-C in that country, subject to specified exceptions. 

Term and Termination. Either we or EA Pharma can terminate the agreement in its entirety or on a country-by-

country basis if the other party materially breaches the agreement and the breach is not cured within a specified period. 
Also, either we or EA Pharma can terminate the agreement in its entirety if a specified bankruptcy-related event with 
regard to the other party occurs. EA Pharma also has the right to terminate the agreement in its entirety or on a country-
by-country basis (except for Japan) for any reason upon 180 days’ notice. The rights and obligations of the parties that 
survive termination of the agreement vary depending on the basis for the termination. 

Royalty Monetization Agreement with HCR 

In December 2017, the Company entered into a royalty interest acquisition agreement (RIAA) with HealthCare 
Royalty Partners III, L.P. (HCR) pursuant to which it sold to HCR the right to receive all royalties from sales in Japan 
and sales milestones achieved from any covered territory potentially payable to the Company under the Agreement, up 
to a specified maximum “cap” amount of $78.8 million, based on the funds the Company received from HCR. In 
January 2018, the Company received $44.5 million from HCR, net of certain transaction expenses, under the RIAA. On 
June 8, 2020, the parties entered into an amendment to the RIAA pursuant to which HCR agreed to pay the Company an 
additional $14.8 million, net of certain transactions expenses, in exchange for the elimination of the (i) $78.8 million cap 
amount on HCR’s rights to receive royalties on sales in Japan and sales milestones for elobixibat in certain other 
territories that may become payable by EA Pharma and (ii) the $15.0 million payable to the Company if a specified sales 
milestone is achieved for elobixibat in Japan. The Company is obligated to make royalty interest payments to HCR 
under the RIAA only to the extent it receives future Japanese royalties, sales milestones or other specified payments 
from EA Pharma. Although the Company sold its rights to receive royalties from the sales of elobixibat in Japan, as a 
result of its ongoing involvement in the cash flows related to these royalties, the Company will continue to account for 
these royalties as revenue. Upon receipt of the payments from HCR the Company recorded net cash totaling 
$59.3 million as a liability related to sale of future royalties (royalty obligation). The royalty obligation will be amortized 
using the effective interest rate method. 

The RIAA requires us to take certain actions with respect to the elobixibat royalties and sales milestones and with 

respect to our license agreement with EA Pharma and contains certain representations and warranties, covenants, 
indemnification obligations and other provisions that are customary for a royalty monetization transaction. In addition, 
for protective purposes only, we (specifically, Albireo Pharma, Inc., Albireo AB and Elobix AB) have agreed to grant 
HCR a precautionary security interest in specified assets related to elobixibat, but only in the event that, notwithstanding 
the parties’ intentions, the transfer contemplated by the RIAA is held by a court of competent jurisdiction not to be a true 
sale. 

We record estimated royalties due for the current period in accrued other expenses until the payment is received 

from EA Pharma at which time we then remit payment to HCR. In order to determine the accretion of the royalty 
obligation, we are required to estimate the total amount of future royalty payments to be received and submitted to HCR. 

18 

The sum of these amounts less the $59.3 million proceeds we received will be recorded as interest expense over the life 
of the royalty obligation. At December 31, 2020, our estimate of our total interest expense resulted in an annual effective 
interest rate of approximately 19.0%. 

Terminated License Agreement with Ferring International Center S.A. 

We entered into a license agreement with Ferring International Center S.A., or Ferring, for the development and 
commercialization of elobixibat outside of the territories licensed to EA Pharma in July 2012, following completion of 
our Phase 2b clinical trial of elobixibat to treat CIC. In March 2015, Ferring terminated the agreement, effective in 
September 2015. As a result of the termination of the agreement, all licenses that we granted to Ferring under the license 
agreement terminated and commercial rights to elobixibat in Ferring’s licensed territory reverted to us. In addition, 
Ferring was required, among other things, to assign to us all rights to all regulatory submissions and approvals controlled 
by Ferring pertaining to elobixibat in the licensed territory and to grant to us an exclusive right of reference to data, and 
specified licenses to data and technology, related to elobixibat for the development and commercialization of elobixibat 
in its licensed field. Notwithstanding the termination of the license agreement, Ferring may be entitled to low single-digit 
royalty payments on net sales of elobixibat on a country-by-country and product-by-product basis in specified 
circumstances.  

Patents and Proprietary Rights  

We actively seek to protect the proprietary technology that we consider important to our business, including 
compositions and forms and their methods of use in the United States, Europe and other jurisdictions internationally that 
we consider key pharmaceutical markets. We also rely upon trade secrets and contracts to protect our proprietary 
information. 

As of December 31, 2020, our patent estate included 28 issued patents, 3 allowed applications and 19 pending 

patent applications in the United States and approximately 50 counterpart patents and patent applications in other 
jurisdictions, including 8European regional issued patents and Patent Cooperation Treaty, or PCT, applications which 
allow us to seek corresponding patent protection worldwide. The actual protection afforded by a patent varies from 
country to country and depends upon many factors, including the type of patent, the scope of its coverage and the 
availability of legal remedies in a particular jurisdiction. 

19 

 
We consider the following United States, European (EP) and, in the case of elobixibat, Japanese (JP) patents to be 

particularly important to the protection of our clinical-stage product candidates.  

Product Candidate      

Summary Description 

      Expiration Date 

Odevixibat  . . .  

Composition of matter of odevixibat 

  September 2022 

Method of using certain IBAT inhibitor(s) to treat certain 
liver diseases 

  November 2031 

Method of using certain IBAT inhibitor(s) in combination 
with 
a bile acid binder to treat certain liver diseases 

November 2031 
(EP 
pending in US) 

Crystal modifications of odevixibat 

Formulations of odevixibat 

Elobixibat . . . .  

Composition of matter of elobixibat 

June 2039  
(allowed in US 
and pending in 
EP) 

June 2039 
(pending in US 
and EP) 

December 2021 
(EP, JP); 
August 2022 
(US) 

Method of using an IBAT inhibitor to treat Chronic 
Idiopathic Constipation or  
Irritable Bowel Syndrome with Constipation 

  April 2024 

Crystal modifications of elobixibat 

  April 2034  

Crystal modifications of elobixibat 

Formulations of elobixibat 

Process for manufacturing  

October 2035 
(US and JP, 
pending in EP) 

2035 (issued and 
pending in US) 

2039 (US,  
pending in EP 
and JP) 

  Method of using certain IBAT inhibitor(s) to treat NASH    November 2031 

A3907 . . . . . . . .  

Composition of matter of A3907 

February 2040 
(allowed in US, 
pending PCT) 

We also have issued patents and pending patent applications with equivalent or substantially comparable 

protection for our product candidates in jurisdictions internationally that we consider key pharmaceutical markets. 

The patent expiration dates referenced above do not reflect any potential patent term extension that we may 
receive for the United States under The United States Drug Price Competition and Patent Term Restoration Act of 1984, 
known as the Hatch-Waxman Act, or similar patent term extension legislation in Europe and Japan. The Hatch-Waxman 

20 

 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Act generally permits a patent extension term of up to five years as compensation for patent term lost during the FDA 
regulatory review process. Patent term extension cannot extend the remaining term of a patent beyond a total of 14 years 
from the date of FDA approval. The patent term restoration period is generally one-half of the time between the effective 
date of an investigational new drug application, or IND, and the submission date of a new drug application, or NDA, 
plus the time between the submission date and approval date of an NDA. Typically, only one patent applicable to an 
approved drug is eligible for an extension, and, with limited exceptions, the extension must be applied for prior to 
expiration of the patent. The United States Patent and Trademark Office, in consultation with the FDA, reviews and 
approves applications for patent term extension. 

In the European Economic Area (EEA; the European Union member countries plus Iceland, Liechtenstein and 
Norway), Regulation (EC) No 469/2009 generally permits an extension of the patent term for a drug as compensation for 
the patent term lost during the regulatory review process by the European Medicines Agency, or EMA, or the national 
drug agencies. The term of a supplementary protection certificate, or SPC, corresponds to the period between the filing 
date of the patent and the date of the first Marketing Authorization in the EEA, reduced by a period of 5 years. An SPC 
may extend the patent term for an approved drug up to five years, but the remaining patent term may not exceed 15 years 
from the date of the first Marketing Authorization. Only one patent that covers the approved drug is eligible for an SPC. 
Applications for an SPC are reviewed and approved by the national patent offices of the EEA countries, and must be 
lodged within six months of the authorization date for the drug in each EEA country (or within six months of the grant of 
the patent, in case the Marketing Authorization is granted first). 

In Japan, the Japanese Pharmaceutical Affairs Law generally permits the extension of the patent term for a drug as 

compensation for patent term lost during the regulatory review process by the Japanese Pharmaceuticals and Medical 
Devices Agency (PMDA). The patent term extension corresponds to the period from the date of the start of clinical trials 
or the date of patent registration, whichever is later, until one day prior to the date of approval for the drug. The term of a 
patent can be extended for up to five years, irrespective of the remaining natural term of the patent as of the date of 
approval. Each patent that covers the active ingredient in the approved drug, or a method of using the approved drug in 
the approved indication, is eligible for the extension, which means that, for any particular drug, multiple patents may be 
extended. The extension must be applied for prior to expiration of the patent and within three months from the date of 
approval. The Japanese Patent Office reviews and approves applications for patent term extension. 

Sales and Marketing  

We currently are building the commercial leadership infrastructure, and expanding the sales and distribution 
organization that we believe will be necessary to effectively support the commercialization of odevixibat in the United 
States and Europe, if odevixibat is approved for PFIC or any other pediatric cholestatic liver disease or disorder. In 
addition to our Chief Commercial Officer, we have hired a President of Americas and a President of International, and 
continue to build sales and account teams in both jurisdictions. We have entered into a Co-Promotion Agreement with 
Travere Therapeutics, Inc. for the co-promotion of odevixibat in the United States. The initial term of the arrangement is 
two years from launch of odevixibat, terminable at will by either party after one year following launch. We believe that 
our commercial organization can be modest in size and targeted to the relatively small number of specialists in the 
United States and Europe who treat children with orphan cholestatic liver disease. 

The commercial infrastructure for orphan products typically consists of a targeted, specialty sales force that calls 

on a limited and focused group of physicians supported by sales management, internal sales support, an internal 
marketing group and distribution support. Additional capabilities important to the marketplace include the management 
of key accounts such as managed care organizations, group purchasing organizations, specialty pharmacies, government 
accounts and reimbursement support. Based on the number of physicians that treat orphan pediatric cholestatic liver 
diseases and disorders, we believe that we can effectively target the physician audience for odevixibat in the United 
States and Europe by establishing a sales force either internally or by contract. To develop the appropriate commercial 
infrastructure, we will have to invest significant amounts of financial and management resources, some of which may be 
committed prior to any confirmation that odevixibat will be approved. 

21 

Outside of the United States and Europe, we plan to selectively utilize collaborations, distribution or other 

marketing arrangements with third parties to commercialize odevixibat in any approved indication(s). Likewise, we 
intend to selectively seek alliances and collaborations to assist us in furthering the development or commercialization of 
product candidates, such as potentially, elobixibat, targeting large primary care markets that must be served by large 
sales and marketing organizations. 

Manufacturing 

We do not own or operate, and currently have no plans to establish, manufacturing facilities for the production of 
clinical or commercial quantities of odevixibat, elobixibat, A3907 or any of our other product candidates. We currently 
rely, and expect to continue to rely, on third parties for the manufacture of our product candidates and any products that 
we may develop. 

We currently engage a single third-party manufacturer to provide the active pharmaceutical ingredient, or API, for 

odevixibat and elobixibat. We also currently engage single third-party manufacturers to provide fill and finish services 
for the final drug product formulation of each of odevixibat, elobixibat and A3907 for use in our clinical trials. 

We obtain the supplies of our API and drug products from these manufacturers pursuant to agreements that include 

specific supply timelines, quality and volume expectations. We obtain the supplies of our product candidates from these 
manufacturers under master services contracts and specific work orders. However, we do not have long-term supply 
arrangements in place. We do not currently have arrangements in place for redundant supply or a second source for API 
for any of odevixibat, elobixibat or A3907. If any of our current manufacturers becomes unavailable to us for any 
reason, we believe that there are a number of potential replacements, although we would likely incur significant delay in 
identifying and qualifying such replacements.  

Odevixibat, elobixibat and A3907 are organic compounds of low molecular weight, and are referred to as “small 

molecules.” We have selected these compounds based on their potential efficacy and safety, although they are also 
associated with reasonable cost of goods. We expect to continue to develop product candidates that can be produced 
cost-effectively at contract manufacturing facilities. 

Competition 

The biotechnology and pharmaceutical industries are characterized by rapidly advancing technologies, intense 

competition and a strong emphasis on proprietary products. We face potential competition from many different sources, 
including major pharmaceutical, specialty pharmaceutical and biotechnology companies, academic institutions, 
government agencies and private and public research institutions. Any product candidates that we successfully develop 
and commercialize will compete with existing therapies and new therapies that may become available in the future. 

Many of our competitors may have significantly greater financial resources and expertise in research and 

development, manufacturing, preclinical testing, conducting clinical trials, obtaining regulatory approvals and marketing 
approved products than we do. These competitors also compete with us in recruiting and retaining qualified scientific 
and management personnel and establishing clinical trial sites and patient enrollment for clinical trials, as well as in 
acquiring technologies complementary to, or necessary for, our programs. Smaller or early stage companies may also 
prove to be significant competitors, particularly through collaborative arrangements with larger or more established 
companies. 

Our commercial opportunity could be reduced or eliminated if our competitors develop and commercialize 
products that are safer, more effective, have fewer or less severe side effects, are more convenient or are less expensive 
than any products that we may develop. Our competitors also may obtain marketing approvals for their products more 
rapidly than we obtain approval for our products. In addition, our ability to compete may be affected because in some 
cases insurers or other third-party payors seek to encourage the use of generic products. This may have the effect of 
making branded products less attractive, from a cost perspective, to buyers. 

22 

The key competitive factors affecting the success of our product candidates are likely to be their efficacy, safety, 

tolerability, convenience, price and the availability of coverage and reimbursement from government and other third-
party payors. 

We are aware of other companies that are developing product candidates that, like odevixibat and elobixibat, act 

via IBAT inhibition. Mirum Pharmaceuticals, Inc. is conducting a Phase 3 clinical trial of maralixibat in PFIC, for which 
it has reported that it expects to complete enrollment in the second quarter of 2021 and announce topline data by year-
end 2021. As of February 2021, Mirum announced the completion of a rolling NDA submission for maralixibat in 
ALGS, and is planning for a potential launch in ALGS in the second half of 2021. The FDA granted breakthrough 
therapy designation to maralixibat for the treatment of pruritus associated with ALGS in October 2019. We understand 
that Mirum’s MAA for maralixibat for the treatment of patients with PFIC type 2 has been accepted for review by the 
EMA. GlaxoSmithKline’s linerixibat has completed Phase 2 clinical development as a treatment for pruritus in patients 
with PBC, with plans underway to progress linerixibat to Phase 3 in 2021. The FDA granted orphan drug designation to 
linerixibat for the treatment of PBC in September 2019. 

The competition in our target indications includes the following. 

PFIC and other pediatric cholestatic liver diseases and disorders. For many cholestatic liver diseases and 
disorders, including in particular PFIC, there are no approved therapies. With regard to the pruritus that is characteristic 
of these diseases, symptomatic off-label treatment with bile acid sequestrants, such as cholestyramine (marketed as 
Colestyr, Efensol, Ipocol, Kolestran, Lipocol, Olestyr, Prevalite or Quantalan in various countries), typically provides 
only modest relief. Bristol Myers Squibb has discontinued manufacture of Questran, but generic versions of the drug are 
marketed by Upsher-Smith Laboratories, Inc., Par Pharmaceutical Companies, Inc. and Sandoz, the generic 
pharmaceuticals division of Novartis AG. 

A number of other drugs, including UDCA, a bile acid which is approved in France only for PFIC type 3, 
rifampin, an antibiotic derivative, and naltrexone, an opioid antagonist, are used off-label for patients suffering from 
cholestatic liver disease. Additionally, surgical interventions, such as PEBD surgery, and external liver filtering 
procedures are also employed in an attempt to lower bile acid levels, manage pruritus and improve measures of liver 
function. 

As noted above, Mirum is conducting a Phase 3 clinical trial of maralixibat in PFIC, for which it has reported that 
it expects to complete enrollment in the second quarter of 2021 and announce topline data by year-end 2021. Mirum has 
also announced that it initiated a rolling submission of an NDA for maralixibat for the treatment of cholestatic pruritus 
associated with ALGS in the third quarter of 2020, expects to complete the rolling submission of the NDA in the first 
quarter of 2021, and is planning for a potential launch in ALGS in the second half of 2021. We understand that Mirum’s 
MAA for maralixibat for the treatment of patients with PFIC type 2 has been accepted for review by the EMA. In 
addition, Intercept Pharmaceuticals’ obeticholic acid, or OCA, is approved in the U.S. in combination with UDCA, or as 
a monotherapy for patients unable to tolerate UDCA, to treat PBC. OCA is also in Phase 2 development at Intercept as a 
treatment for biliary atresia. In July 2019, Genfit SA received orphan designations from the FDA and EMA for its PPAR 
alpha/delta agonist, (elafibranor), for the treatment of PBC. Genfit has announced plans to advance elafibranor into a 
Phase 3 program for PBC in 2020. Gilead Sciences, Inc.’s FXR agonist, cilofexor, is in Phase 2 development for PBC, 
and Phase 3 development for PSC. 

Chronic Constipation. Linaclotide, marketed by Ironwood Pharmaceuticals, Inc. and Allergan as Linzess in the 
United States and as Constella in Europe, is approved in the United States for the treatment of CIC and IBS-C and in 
Europe for the treatment of IBS-C. Linaclotide is also approved for the treatment of IBS-C in Japan, where it is marketed 
by Astellas. It is also marketed by AstraZeneca’s partner Ironwood Pharmaceuticals, Inc. in China, where it was 
approved for use in 2019. Linaclotide targets guanylate cyclase C in the intestines and, by doing so, induces intestinal 
chloride secretion, which results in the outpouring of water into the intestine. The primary side effect of linaclotide is 
diarrhea. In addition, lubiprostone, which is marketed in the United States as Amitiza by Takeda Pharmaceuticals USA, 
Inc., is approved in the United States for the treatment of CIC, IBS-C and opioid-induced constipation. Amitiza is also 
approved for the treatment of CIC in the United Kingdom and Switzerland, and for the treatment of chronic constipation 

23 

in Japan, where it is marketed by Mylan, N.V. Amitiza binds selectively to and activates the type-2 chloride channel in 
the intestine releasing chloride and water into the intestine. The primary side effect of Amitiza is nausea. Prucalopride, 
marketed by Shire in the United States as Motegrity, in the European Union as Resolor, is a motility agent approved for 
the treatment of CIC. Motegrity and Resolor are associated with a high rate of headaches, and belong to a class of drugs 
known as 5-HT receptor drugs that has been linked to cardiovascular safety issues. 

Numerous OTC products are available for constipation. These include psyllium husk (such as Metamucil), 

methylcellulose (such as Citrucel), calcium polycarbophil (such as FiberCon), lactulose (such as Cephulac), 
polyethylene glycol (such as MiraLax), sennosides (such as Exlax), bisacodyl (such as Ducolax), docusate sodium (such 
as Colace), magnesium hydroxide (such as Milk of Magnesia), saline enemas (such as Fleet) and sorbitol. Given the low 
barriers to access, many CIC sufferers first try OTC fiber and laxatives, but these options are not sufficiently effective 
for many people. 

Plecanatide, marketed as Trulance by Bausch Health Companies, Inc., is approved in the United States to treat CIC 

and IBS-C and is, like linaclotide, a guanylate cyclase-C agonist. On September 12, 2019, the FDA approved 
Ardelyx, Inc.’s product, IBSRELA (tenapanor), for the treatment of IBS-C. IBSRELA inhibits the sodium transporter 
NHE3 and reduces sodium uptake from the gut to increase the secretion of water in the intestines. 

Government Regulation 

Government authorities in the United States, at the federal, state and local level, and in other countries and 
jurisdictions, including the European Union, extensively regulate, among other things, the research, development, 
testing, manufacture, quality control, approval, packaging, storage, recordkeeping, labeling, advertising, promotion, 
distribution, marketing, post-approval monitoring and reporting, and import and export of pharmaceutical products. The 
processes for obtaining regulatory approvals in the United States and in foreign countries and jurisdictions, along with 
subsequent compliance with applicable statutes and regulations and other regulatory requirements, require the 
expenditure of substantial time and financial resources. 

Review and Approval of Drugs in the United States 

In the United States, the FDA regulates drugs under the Federal Food, Drug, and Cosmetic Act, or FDCA, and its 

implementing regulations. Failure to comply with the applicable U.S. requirements at any time during the product 
development process,  the approval process or after  approval may subject an applicant or sponsor to a variety of 
administrative or judicial sanctions, including refusal by the FDA to approve pending applications, withdrawal of an 
approval, imposition of a clinical hold, issuance of warning letters and other types of  letters, product recalls, product 
seizures, total or partial suspension of production or distribution, injunctions, fines, refusals of government contracts, 
restitution, disgorgement of profits, or civil or criminal investigations and penalties brought by the FDA and the 
Department of Justice, or DOJ, or other governmental entities. 

An applicant seeking approval to market and distribute a new drug product in the United States must typically 

undertake the following: 

• 

• 

• 

• 

completion of preclinical laboratory tests, animal studies and formulation studies in compliance with the 
FDA’s good laboratory practice, or GLP, regulations; 

submission to the FDA of an IND, which must take effect before human clinical trials may begin; 

approval by an independent institutional review board, or IRB, representing each clinical site before each 
clinical trial may be initiated; 

performance of adequate and well-controlled human clinical trials in accordance with good clinical 
practices, or GCP, to establish the safety and efficacy of the proposed drug product for each proposed 
indication; 

24 

• 

• 

• 

• 

• 

• 

satisfactory completion of FDA audits of clinical trial sites to assure compliance with GCPs and the integrity 
of the clinical data; 

preparation and submission to the FDA of an NDA; 

review of the product by an FDA advisory committee, where appropriate or if applicable; 

satisfactory completion of one or more FDA inspections of the manufacturing facility or facilities at which 
the product, or components thereof, are produced to assess compliance with current Good Manufacturing 
Practices, or cGMP, requirements and to assure that the facilities, methods and controls are adequate to 
preserve the product’s identity, strength, quality and purity; 

payment of user fees and securing FDA approval of the NDA; and 

compliance with any post-approval requirements, including Risk Evaluation and Mitigation Strategies, or 
REMS, and post-approval studies required by the FDA, if applicable. 

Preclinical Studies 

Preclinical studies include laboratory evaluation of the purity and stability of the manufactured drug substance or 

API and the formulated drug or drug product, as well as in vitro and animal studies to assess the safety and activity of 
the drug for initial testing in humans and to establish a rationale for therapeutic use. The conduct of preclinical studies is 
subject to federal regulations and requirements, including GLP regulations. The results of the preclinical tests, together 
with manufacturing information, analytical data, any available clinical data or literature and plans for clinical trials, 
among other things, are submitted to the FDA as part of an IND. Some long-term preclinical testing, such as animal tests 
of reproductive adverse events and carcinogenicity, may continue after the IND is submitted. 

Human Clinical Trials in Support of an NDA 

Clinical trials involve the administration of the investigational product to human subjects under the supervision of 

qualified investigators in accordance with GCP requirements, which include, among other things, the requirement that 
all research subjects provide their informed consent in writing before their participation in any clinical trial. Clinical 
trials are conducted under written protocols detailing, among other things, the inclusion and exclusion criteria, the 
objectives of the trial, the parameters to be used in monitoring safety, and the effectiveness criteria to be evaluated. A 
protocol for each clinical trial and any subsequent protocol amendments must be submitted to the FDA as part of the 
IND. An IND automatically becomes effective 30 days after receipt by the FDA, unless the FDA raises concerns or 
questions related to a proposed clinical trial and places the trial on clinical hold. In such a case, the IND sponsor and the 
FDA must resolve any outstanding concerns before the clinical trial can be initiated or restarted (in cases when the trial 
is placed on clinical hold after it has already begun). 

In addition, an IRB representing each institution that is participating in the clinical trial must review and approve 
the plan for any clinical trial before it commences at that institution, and the IRB must conduct a continuing review and 
reapprove the trial at least annually. The IRB must review and approve, among other things, the trial protocol and 
informed consent information to be provided to clinical trial subjects. An IRB must operate in compliance with FDA 
regulations. Information about certain clinical trials, including details of the protocol and eventually study results, also 
must be submitted within specific timeframes to the National Institutes of Health for public dissemination on the 
ClinicalTrials.gov data registry. 

Human clinical trials are typically conducted in three sequential phases, which may overlap or be combined: 

• 

Phase 1. The drug is initially introduced into healthy human subjects or, in certain indications such as 
cancer, patients with the target disease or condition, in order to be tested for safety, dosage tolerance, 

25 

absorption, metabolism, distribution, excretion and, if possible, to gain an early indication of its 
effectiveness and to determine optimal dosage. 

• 

• 

Phase 2. The drug is administered to a limited patient population to identify possible adverse effects and 
safety risks, in order to preliminarily evaluate the efficacy of the product for specific targeted diseases and to 
determine dosage tolerance and optimal dosage. 

Phase 3. The drug is administered to an expanded patient population, generally at geographically dispersed 
clinical trial sites, in one or more well-controlled clinical trials in order to generate enough data to 
statistically evaluate the efficacy and safety of the product for approval, to establish the overall risk-benefit 
profile of the drug, and to provide adequate information for the labeling of the drug. 

Progress reports detailing the results of the clinical trials must be submitted at least annually to the FDA and more 
frequently if SAEs occur. In addition, IND safety reports must be submitted to the FDA for any of the following: serious 
and unexpected suspected adverse reactions; findings from other clinical trials or animal or in vitro testing that suggest a 
significant risk in humans exposed to the drug; and any clinically important increase in the case of a serious suspected 
adverse reaction over that listed in the protocol or investigator brochure. Phase 1, Phase 2 and Phase 3 clinical trials may 
not be completed successfully within any specified period, or at all. Furthermore, the FDA or the sponsor may suspend 
or terminate a clinical trial at any time on various grounds, including a finding that the research subjects are being 
exposed to an unacceptable health risk. Similarly, an IRB can suspend or terminate approval of a clinical trial at its 
institution, = if the clinical trial is not being conducted in accordance with the clinical protocol or GCP or if the drug has 
been associated with unexpected serious harm to patients. The FDA will typically inspect one or more clinical sites to 
assure compliance with GCP and the integrity of the clinical data submitted. 

Submission of an NDA to the FDA 

Assuming successful completion of required clinical testing and other requirements, the results of the preclinical 
studies and clinical trials, together with detailed information relating to the product’s chemistry, manufacture, controls 
and proposed labeling, among other things, are submitted to the FDA as part of an NDA, which requests approval to 
market the drug product for one or more indications. Under federal law, the fee for the submission of an NDA for which 
clinical data is required exceeds $2.8 million for FY2021, and the sponsor of an approved NDA is also subject to an 
annual program fee, currently exceeding $330,000 per program. These fees are typically increased annually, but 
exemptions and waivers may be available under certain circumstances (such as a waiver for the first human drug 
application submitted by a qualifying small business and exemptions for orphan products). 

The FDA conducts a preliminary review of an NDA within 60 days of its receipt and informs the sponsor by the 

74th day after the FDA’s receipt of the submission whether the application is sufficiently complete to permit substantive 
review. The FDA may request additional information rather than accept an NDA for filing. In this event, the application 
must be resubmitted with the additional information. The resubmitted application is also subject to review before the 
FDA accepts it for filing. After the submission is accepted for filing, the FDA begins an in-depth substantive review. 
The FDA has agreed to specified performance goals in the review process of NDAs. Most such applications are meant to 
be reviewed within ten months from the date the NDA is accepted for filing, and most applications for “priority review” 
products are meant to be reviewed within six months from the date the NDA is accepted for filing. The review process 
may be extended by the FDA for three additional months to consider new information or in the case of a clarification 
provided by the applicant to address an outstanding deficiency identified by the FDA following the original submission. 

Before approving an NDA, the FDA typically will inspect the facility or facilities where the product is or will be 
manufactured. These pre-approval inspections cover all facilities associated with an NDA submission, including drug 
component manufacturing (such as active pharmaceutical ingredients), finished drug product manufacturing, and control 
testing laboratories. The FDA will not approve an application unless it determines that the manufacturing processes and 
facilities are in compliance with cGMP requirements and are adequate to assure consistent production of the drug 
product within required specifications. Additionally, before approving an NDA, the FDA will typically inspect one or 
more clinical sites to assure compliance with GCP. 

26 

In addition, as a condition of approval, the FDA may require an applicant to develop a REMS.  REMS use risk 
minimization strategies beyond the professional labeling to ensure that the benefits of the product outweigh the potential 
risks. To determine whether a REMS is needed, the FDA will consider the size of the population likely to use the 
product, seriousness of the disease, expected benefit of the product, expected duration of treatment, seriousness of 
known or potential adverse events, and whether the product is a new molecular entity. REMS are tailored to the specific 
risk/benefit profile of a drug and can include requirements such as medication guides for patients, detailed 
communication plans for healthcare professionals, and elements to assure safe use, or ETASU. ETASU may include, but 
are not limited to, special training or certification for prescribing or dispensing, dispensing only under certain 
circumstances, special monitoring, restricted distribution, and the use of patient registries. The FDA may require a 
REMS as a condition of approval or post-approval if it becomes aware of a serious risk associated with use of the 
product. The requirement for a REMS and the specific components that are involved can materially affect the potential 
market and profitability of a product. 

The FDA often refers an application for a new drug to an advisory committee. Typically, an advisory committee is 

a panel of independent experts, including clinicians and other scientific experts, that reviews, evaluates and provides a 
recommendation as to whether the application should be approved and under what conditions. The FDA is not bound by 
the recommendations of an advisory committee, but it considers such recommendations carefully when making final 
approval decisions about a particular NDA. 

Fast Track, Breakthrough Therapy and Priority Review Designations 

The FDA is authorized to designate certain products for expedited development or review if they are intended to 

address an unmet medical need in the treatment of a serious or life-threatening disease or condition. These programs 
include fast track designation, breakthrough therapy designation and priority review designation. 

Specifically, the FDA may grant a product the fast track designation if it is intended, whether alone or in 
combination with one or more other drugs, for the treatment of a serious or life-threatening disease or condition, and it 
demonstrates the potential to address unmet medical needs for such a disease or condition. For fast track products, 
sponsors may have greater interactions with the FDA and the FDA may initiate review of sections of a fast track 
product’s NDA before the application is complete. This rolling review may be available if the FDA determines, after 
preliminary evaluation of clinical data submitted by the sponsor, that a fast track product may be effective. The sponsor 
must also provide, and the FDA must approve, a schedule for the submission of the remaining information and the 
sponsor must pay applicable user fees. However, the FDA’s time period goal for reviewing a fast track product 
application does not begin until the last section of the NDA is submitted. In addition, the fast track designation may be 
withdrawn by the sponsor or rescinded by the FDA if the designation is no longer supported by data emerging in the 
clinical trial process. In September of 2018, the FDA granted fast track designation to odevixibat for the treatment of 
pruritus associated with PFIC. 

In 2012, Congress enacted the Food and Drug Administration Safety and Innovation Act, or FDASIA. This law 

established a new regulatory program for products designated as “breakthrough therapies.” A product may be designated 
as a breakthrough therapy if it is intended, either alone or in combination with one or more other drugs, to treat a serious 
or life-threatening disease or condition and preliminary clinical evidence indicates that the product may demonstrate 
substantial improvement over existing therapies on one or more clinically significant endpoints, such as substantial 
treatment effects observed early in clinical development. The FDA may take certain actions with respect to designated 
breakthrough therapies, including: holding meetings with the sponsor throughout the development process; providing 
timely advice to the product sponsor regarding development and approval; involving more senior staff in the review 
process; assigning a cross-disciplinary project lead for the review team; and taking other steps to design the clinical trials 
in an efficient manner.  

Finally, the FDA may designate a product for priority review if it is a drug that treats a serious condition and, if 

approved, would provide a significant improvement in safety or effectiveness. The FDA determines at the time that the 
marketing application is submitted, on a case- by-case basis, whether the proposed drug represents a significant 
improvement when compared with other available therapies. Significant improvement may be illustrated by evidence of 

27 

increased effectiveness in the treatment of a condition, elimination or substantial reduction of a treatment-limiting drug 
reaction, documented enhancement of patient compliance that may lead to improvement in serious outcomes, or 
evidence of safety and effectiveness in a new subpopulation. A priority review designation is intended to direct overall 
attention and resources to the evaluation of such applications, and to shorten the FDA’s goal for taking action on a 
marketing application from ten months to six months. 

Accelerated Approval Pathway 

The FDA may grant accelerated approval to a drug for a serious or life-threatening condition that generally 
provides a meaningful therapeutic advantage to patients over existing treatments and based upon a demonstration that 
the drug has an effect on a surrogate endpoint that is reasonably likely to predict clinical benefit. The FDA may also 
grant accelerated approval for such a drug when the product has an effect on an intermediate clinical endpoint that can 
be measured earlier than an effect on irreversible morbidity or mortality, or IMM, and that is reasonably likely to predict 
an effect on IMM or other clinical benefit, taking into account the severity, rarity, or prevalence of the condition and the 
availability or lack of alternative treatments. Drugs granted accelerated approval must meet the same statutory standards 
for safety and effectiveness as those granted traditional approval.  

For the purposes of accelerated approval, a surrogate endpoint is a marker, such as a laboratory measurement, 

radiographic image, physical sign, or other measure that is thought to predict clinical benefit, but is not itself a measure 
of clinical benefit. Surrogate endpoints can often be measured more easily or more rapidly than clinical endpoints. An 
intermediate clinical endpoint is a measurement of a therapeutic effect that is considered reasonably likely to predict the 
clinical benefit of a drug, such as an effect on IMM. The FDA has limited experience with accelerated approvals based 
on intermediate clinical endpoints, but has indicated that such endpoints generally may support accelerated approval 
when the therapeutic effect measured by the endpoint is not itself a clinical benefit and basis for traditional approval, if 
there is a basis for concluding that the therapeutic effect is reasonably likely to predict the ultimate long-term clinical 
benefit of a drug. 

The accelerated approval pathway is most often used in settings in which the course of a disease is long and an 

extended period of time is required to measure the intended clinical benefit of a drug, even if the effect on the surrogate 
or intermediate clinical endpoint occurs rapidly. For example, accelerated approval has been used extensively in the 
development and approval of drugs for treatment of a variety of cancers in which the goal of therapy is generally to 
improve survival or decrease morbidity and the duration of the typical disease course requires lengthy and sometimes 
large clinical trials to demonstrate a clinical or survival benefit. 

The accelerated approval pathway is usually contingent on a sponsor’s agreement to conduct, in a diligent manner, 

additional post-approval confirmatory studies to verify and describe the drug’s clinical benefit. As a result, a product 
candidate approved on this basis is subject to rigorous post-marketing compliance requirements, including the 
completion of Phase 4 or post-approval clinical trials to confirm the effect on the clinical endpoint. Failure to conduct 
required post-approval studies, or to confirm the predicted clinical benefit of the product during post-marketing studies, 
would allow the FDA to withdraw approval of the drug. All promotional materials for product candidates being 
considered and approved under the accelerated approval program are subject to prior review by the FDA. 

Rare Pediatric Disease Priority Review Voucher 

The FDA may grant rare pediatric disease designation for indications in the treatment or prevention of a rare 

disease or condition that affects fewer than 200,000 individuals in the United States and that is a serious or life-
threatening disease that primarily affects individuals aged from birth to 18 years, including age groups often called 
neonates, infants, children and adolescents. In June 2018, the FDA granted a rare pediatric disease designation to 
odevixibat for the treatment of PFIC. Under the FDCA, a sponsor who receives approval of an NDA for a product that is 
for the prevention or treatment of a rare pediatric disease and meets certain additional criteria, may qualify for a rare 
pediatric disease priority review voucher, or PRV. A PRV can be redeemed to receive priority review under an expedited 
timeframe for a subsequent marketing application for a different product. A PRV may also be sold or transferred from 
the initial sponsor to another sponsor and may be further transferred any number of times before it is used. The United 

28 

States Congress recently extended the FDA’s authority to award rare pediatric disease PRVs  until 2024 and until 2026 
for products that receive rare pediatric disease designation by 2024. 

The FDA’s Decision on an NDA 

On the basis of the FDA’s evaluation of the NDA and accompanying information, including the results of the 
inspection of the manufacturing facilities, the FDA may issue either an approval letter or a complete response letter 
(CRL). An approval letter authorizes commercial marketing of the product with specific prescribing information and for 
specific indications. A CRL generally outlines the deficiencies in the submission and may require substantial additional 
testing or information in order for the FDA to reconsider the application. If and when those deficiencies have been 
addressed to the FDA’s satisfaction in a resubmission of the NDA, the FDA will issue an approval letter. The FDA has 
committed to reviewing such resubmissions in response to the CRL in either two or six months depending on the type of 
information included. Even with the submission of this additional information, however, the FDA ultimately may decide 
that the application does not satisfy the regulatory criteria for approval. 

If the FDA approves a product, it may limit the approved indications for use for the product; require that 

contraindications, warnings or precautions be included in the product labeling; require that post-approval studies, 
including Phase 4 clinical trials, be conducted to further assess the drug’s safety after approval; require testing and 
surveillance programs to monitor the product after commercialization; or impose other conditions, including distribution 
restrictions or other risk management mechanisms, including REMS, which can materially affect the potential market 
and profitability of the product. The FDA may prevent or limit further marketing of a product based on the results of 
post-market studies or surveillance programs. After approval, many types of changes to the approved product, such as 
adding new indications, manufacturing changes and additional labeling claims, are subject to further testing 
requirements and FDA review and approval. 

Post-Approval Requirements 

Drugs manufactured, marketed or distributed pursuant to FDA approvals are subject to pervasive and continuing 

regulation by the FDA, including, among other things, requirements relating to recordkeeping, periodic reporting, 
product sampling and distribution, advertising and promotion and reporting of adverse experiences with the product. 
After approval, most changes to the approved product, such as adding new indications or other labeling claims, are 
subject to prior FDA review and approval. There also are continuing, annual user fee requirements for any marketed 
products and related establishments, as well as new application fees for supplemental applications. 

In addition, drug manufacturers and other entities involved in the manufacture and distribution of approved drugs 

are required to register their establishments with the FDA and state agencies, and are subject to periodic unannounced 
inspections by the FDA for compliance with cGMP requirements. Changes to the manufacturing process are strictly 
regulated and often require prior FDA approval before being implemented. FDA regulations also require investigation 
and correction of any deviations from cGMP and impose reporting and documentation requirements upon the sponsor 
and others involved in the manufacturing process. Accordingly, manufacturers must continue to expend time, money and 
effort in the area of production and quality control to maintain cGMP compliance. 

Once an approval is granted, the FDA may withdraw the approval if compliance with regulatory requirements and 

standards is not maintained or if problems occur after the product reaches the market. Later discovery of previously 
unknown problems with a product, including adverse events of unanticipated severity or frequency, or with 
manufacturing processes, or failure to comply with regulatory requirements, may result in revisions to the approved 
labeling to add new safety information; imposition of post-market studies or clinical trials to assess new safety risks; or 
imposition of distribution or other restrictions under a REMS program. Other potential consequences include, among 
other things: 

• 

restrictions on the marketing or manufacturing of the product, complete withdrawal of the product from the 
market or product recalls; 

29 

• 

• 

• 

• 

fines, warning letters or holds on post-approval clinical trials; 

refusal of the FDA to approve pending NDAs or supplements to approved NDAs, or suspension or 
revocation of product license approvals; 

product seizure or detention, or refusal to permit the import or export of products; or 

injunctions or the imposition of civil or criminal penalties. 

The FDA strictly regulates marketing, labeling, advertising and promotion of products that are placed on the 
market. Drugs may be promoted only for the approved indications and in accordance with the provisions of the approved 
label. The FDA and other agencies actively enforce the laws and regulations prohibiting the promotion of off-label uses, 
and a company that is found to have improperly promoted off-label uses may be subject to significant penalties. 

In addition, the distribution of prescription pharmaceutical products is subject to the Prescription Drug Marketing 

Act, or PDMA, which regulates the distribution of drugs and drug samples at the federal level, and sets minimum 
standards for the registration and regulation of drug distributors by the states. Both the PDMA and state laws limit the 
distribution of prescription pharmaceutical product samples and impose requirements to ensure accountability in 
distribution. Most recently, the Drug Supply Chain Security Act, or DSCSA, was enacted with the aim of building an 
electronic system to identify and trace certain prescription drugs distributed in the United States. The DSCSA mandates 
phased-in and resource-intensive obligations for pharmaceutical manufacturers, wholesale distributors, and dispensers 
over a 10-year period that is expected to culminate in November 2023. 

Abbreviated New Drug Applications for Generic Drugs  

In 1984, with passage of the Hatch-Waxman Amendments to the FDCA, Congress authorized the FDA to approve 

generic drugs that are the same as drugs previously approved by the FDA under the NDA provisions of the statute. To 
obtain approval of a generic drug, an applicant must submit an abbreviated new drug application, or ANDA, to the 
agency. In support of such applications, a generic manufacturer may rely on the preclinical and clinical testing conducted 
for a drug product previously approved under an NDA, known as the reference listed drug, or RLD. Specifically, in 
order for an ANDA to be approved, the FDA must find that the generic version is identical to the RLD with respect to 
the active ingredients, the route of administration, the dosage form, and the strength of the drug. At the same time, the 
FDA must also determine that the generic drug is “bioequivalent” to the innovator drug. Under the statute, a generic drug 
is bioequivalent to a RLD if “the rate and extent of absorption of the drug do not show a significant difference from the 
rate and extent of absorption of the listed drug . . .” 

Upon approval of an ANDA, the FDA indicates whether the generic product is “therapeutically equivalent” to the 

RLD in its publication “Approved Drug Products with Therapeutic Equivalence Evaluations,” also referred to as the 
“Orange Book.” In addition, by operation of certain state laws and numerous health insurance programs, the FDA’s 
designation of therapeutic equivalence often results in substitution of the generic drug without the knowledge or consent 
of either the prescribing physician or patient. 

Under the Hatch-Waxman Amendments, the FDA may not approve an ANDA until any applicable period of 
nonpatent exclusivity for the RLD has expired. The FDCA provides a period of five years of nonpatent data exclusivity 
for a new drug containing a new chemical entity. In cases where such exclusivity has been granted, an ANDA may not 
be submitted to the FDA until the expiration of five years unless the submission is accompanied by a Paragraph IV 
certification, in which case the applicant may submit its application four years after the original product approval. The 
FDCA also provides for a period of three years of exclusivity if the NDA includes reports of one or more new clinical 
investigations, other than bioavailability or bioequivalence studies, that were conducted by or for the applicant and are 
essential to the approval of the application. This three-year exclusivity period often protects changes to a previously 
approved drug product, such as a new dosage form, route of administration, combination or indication. 

30 

Hatch-Waxman Patent Certification and the 30-Month Stay 

Upon approval of an NDA or a supplement thereto, NDA sponsors are required to list with the FDA each patent 

with claims that cover the applicant’s product or an approved method of using the product. Each of the patents listed by 
the NDA sponsor is published in the Orange Book. When an ANDA applicant submits its application to the FDA, the 
applicant is required to certify to the FDA concerning any patents listed for the RLD in the Orange Book, except for 
patents covering methods of use for which the ANDA applicant is not seeking approval. To the extent that the 
Section 505(b)(2) NDA applicant is relying on studies conducted for an already approved product, the applicant also is 
required to certify to the FDA concerning any patents listed for the approved product in the Orange Book to the same 
extent that an ANDA applicant would. Specifically, the applicant for a follow-on drug product must certify with respect 
to each patent that: 

• 

• 

• 

• 

the required patent information has not been filed; 

the listed patent has expired; 

the listed patent has not expired, but will expire on a particular date and approval is sought after patent 
expiration; or 

the listed patent is invalid, unenforceable or will not be infringed by the new product. 

A certification that the new product will not infringe the already approved product’s listed patents or that such 

patents are invalid or unenforceable is called a Paragraph IV certification. If the applicant does not challenge the listed 
patents or indicates that it is not seeking approval of a patented method of use, the ANDA application will not be 
approved until all the listed patents claiming the referenced product have expired (other than method of use patents 
involving indications for which the ANDA applicant is not seeking approval). 

If the ANDA applicant has provided a Paragraph IV certification to the FDA, the applicant must also send notice 

of the Paragraph IV certification to the NDA owner and patent holders once the ANDA in question has been accepted 
for filing by the FDA. The NDA owner and patent holders may then initiate a patent infringement lawsuit in response to 
the notice of the Paragraph IV certification. The filing of a patent infringement lawsuit within 45 days after the receipt of 
a Paragraph IV certification automatically prevents the FDA from approving the ANDA until the earlier of 30 months 
after the receipt of the Paragraph IV notice, expiration of the patent or a decision in the infringement case that is 
favorable to the ANDA applicant. 

Pediatric Clinical Trials and Exclusivity 

Under the Pediatric Research Equity Act of 2003 an NDA or supplement thereto must contain data that are 
adequate to assess the safety and effectiveness of the drug product for the claimed indications in all relevant pediatric 
subpopulations, and to support dosing and administration for each pediatric subpopulation for which the product is safe 
and effective. With enactment of the FDASIA in 2012, sponsors must also submit pediatric trial plans prior to the 
assessment data. Those plans must contain an outline of the proposed pediatric trial or trials the applicant plans to 
conduct, including trial objectives and design, any deferral or waiver requests and other information required by 
regulation. The applicant, the FDA, and the FDA’s internal review committee must then review the information 
submitted, consult with each other, and agree upon a final plan. The FDA or the applicant may request an amendment to 
the plan at any time. 

The FDA may, on its own initiative or at the request of the applicant, grant deferrals for submission of some or all 

pediatric data until after approval of the product for use in adults, or full or partial waivers from the pediatric data 
requirements. Additional requirements and procedures relating to deferral requests and requests for extension of 
deferrals are contained in FDASIA. Unless otherwise required by regulation, the pediatric data requirements do not 
apply to products with orphan designation. 

31 

Pediatric exclusivity is another type of nonpatent marketing exclusivity in the United States and, if granted, 
provides for the attachment of an additional six months of marketing protection to the term of any existing regulatory 
exclusivity, including the nonpatent and orphan exclusivity. This six-month exclusivity may be granted if an NDA 
sponsor submits pediatric data that fairly respond to a written request from the FDA for such data. The data do not need 
to show the product to be effective in the pediatric population studied; rather, if the clinical trial is deemed to fairly 
respond to the FDA’s request, the additional protection is granted. If reports of requested pediatric studies are submitted 
to and accepted by the FDA within the statutory time limits, whatever statutory or regulatory periods of exclusivity or 
patent protection cover the product are extended by six months. This is not a patent term extension, but it effectively 
extends the regulatory period during which the FDA cannot approve another application. 

Orphan Drug Designation and Exclusivity 

The FDA has granted orphan drug designation to odevixibat for the treatment of PFIC, ALGS, biliary atresia and 
PBC. Under the Orphan Drug Act, the FDA may designate a drug product as an “orphan drug” if it is intended to treat a 
rare disease or condition (generally meaning that it affects fewer than 200,000 individuals in the United States, or more 
in cases in which there is no reasonable expectation that the cost of developing and making a drug product available in 
the United States for treatment of the disease or condition will be recovered from sales of the product). A company must 
request orphan product designation before submitting an NDA. If the designation request is granted, the FDA will 
disclose the identity of the therapeutic agent and its potential use. Orphan product designation does not convey any 
advantage in or shorten the duration of the regulatory review and approval process. 

If a product with orphan status receives the first FDA approval for the disease or condition for which it has such 
designation, the product generally will receive orphan product exclusivity. Orphan product exclusivity means that the 
FDA may not approve any other applications for the same product for the same indication for seven years, except in 
certain limited circumstances. Competitors may receive approval of different products for the indication for which the 
orphan product has exclusivity and may obtain approval for the same product but for a different indication. If a drug or 
drug product designated as an orphan product ultimately receives marketing approval for an indication broader than what 
was designated in its orphan product application, it may not be entitled to exclusivity. 

Patent Term Restoration and Extension 

The term of a U.S. patent that covers a drug, biological product or approved medical device may also be eligible 

for patent term extension when FDA approval is granted, provided that certain statutory and regulatory requirements are 
met. The length of the patent term extension is related to the length of time the drug is under regulatory review while the 
patent is in force. For drugs, the Hatch-Waxman Act permits a patent term extension of up to five years beyond the 
expiration date set for the patent. Patent extension cannot extend the remaining term of a patent beyond a total of 
14 years from the date of product approval, only one patent applicable to each regulatory review period may be granted 
an extension, and only those claims reading on the approved drug may be extended. Similar provisions are available in 
Europe and certain other foreign jurisdictions to extend the term of a patent that covers an approved drug, provided that 
statutory and regulatory requirements are met. The U.S. Patent and Trademark Office reviews and approves the 
application for any patent term extension or restoration in consultation with the FDA. 

Regulation Outside the United States 

In order to market any product candidate outside of the United States, a company must also comply with numerous 

and varying regulatory requirements of other countries and jurisdictions regarding quality, safety and efficacy and 
governing, among other things, clinical trials, marketing authorization, commercial sales and distribution of drug 
products. Whether or not it obtains FDA approval for a product candidate, the company would need to obtain the 
necessary approvals by the comparable foreign regulatory authorities before it can commence clinical trials or marketing 
of a product in those countries or jurisdictions. The approval process ultimately varies between countries and 
jurisdictions and can involve additional testing and additional administrative review periods. The time required to obtain 
approval in other countries and jurisdictions might differ from and be longer than that required to obtain FDA approval. 

32 

Regulatory approval in one country or jurisdiction does not ensure regulatory approval in another, but a failure or delay 
in obtaining regulatory approval in one country or jurisdiction may negatively impact the regulatory process in others. 

Regulation and Marketing Authorization in the European Union 

Clinical Trial Approval 

Requirements for the conduct of clinical trials in the European Union including Good Clinical Practice, or GCP, 
are implemented in the Clinical Trials Directive 2001/20/EC and the GCP Directive 2005/28/EC. Pursuant to Directive 
2001/20/EC and Directive 2005/28/EC, as amended, a system for the approval of clinical trials in the European Union 
has been implemented through national legislation of the member states. Under this system, approval must be obtained 
from the competent national authority of an E.U. member state in which a trial is planned to be conducted, or in multiple 
member states if the clinical trial is to be conducted in a number of member states. To this end, a CTA is submitted, 
which must be supported by an investigational medicinal product dossier, or IMPD, and further supporting information 
prescribed by Directive 2001/20/EC and Directive 2005/28/EC and other applicable guidance documents. Furthermore, a 
clinical trial may only be started after a competent ethics committee has issued a favorable opinion on the clinical trial 
application in that country. 

In April 2014, the European Commission passed the new Clinical Trials Regulation, (EU) No 536/2014, which 

will replace the current Clinical Trials Directive 2001/20/EC. To ensure that the rules for clinical trials are identical 
throughout the European Union, the new E.U. clinical trials legislation was enacted as a regulation that is directly 
applicable in all E.U. member states. All clinical trials performed in the European Union are required to be conducted in 
accordance with the Clinical Trials Directive 2001/20/EC until the new Clinical Trials Regulation (EU) No 536/2014 
becomes applicable. 

The new Regulation (EU) No 536/2014 aims to simplify and streamline the approval of clinical trial in the 

European Union. The main characteristics of the regulation include: 

• 

• 

• 

• 

• 

a streamlined application procedure via a single entry point, the E.U. portal; 

a single set of documents to be prepared and submitted for the application as well as simplified reporting 
procedures that will spare sponsors from submitting broadly identical information separately to various 
bodies and different member states; 

a harmonized procedure for the assessment of applications for clinical trials, which is divided in two parts 
(Part I is assessed jointly by all member states concerned and Part II is assessed separately by each member 
state concerned); 

strictly defined deadlines for the assessment of clinical trial application; and 

the involvement of the ethics committees in the assessment procedure in accordance with the national law of 
the member state concerned but within the overall timelines defined by the Regulation (EU) No 536/2014. 

PRIME Designation 

The European Medicines Agency, or EMA, grants access to the Priority Medicines, or PRIME, program to 
investigational medicines for which it determines there to be preliminary data available showing the potential to address 
an unmet medical need and bring a major therapeutic advantage to patients. As part of the program, EMA provides early 
and enhanced dialogue and support to optimize the development of eligible medicines and speed up their evaluation, 
aiming to bring promising treatments to patients sooner. The EMA has granted access to the PRIME program at the 
“proof of concept” stage for odevixibat to treat PFIC. 

33 

Marketing Authorization 

Authorization to market a product in the member states of the European Union proceeds under one of four 
procedures: a centralized authorization procedure, a mutual recognition procedure, a decentralized procedure or a 
national procedure. 

Centralized Authorization Procedure 

The centralized procedure enables applicants to obtain a marketing authorization that is valid in all E.U. member 

states based on a single application. Certain medicinal products, including products developed by means of 
biotechnological processes, must undergo the centralized authorization procedure for marketing authorization, which, if 
granted by the European Commission, is automatically valid in all 28 E.U. member states. The EMA and the European 
Commission administer this centralized authorization procedure pursuant to Regulation (EC) No 726/2004. 

Pursuant to Regulation (EC) No 726/2004, this procedure is mandatory for various types of products, including, 

among others, products that are designated as orphan medicinal products pursuant to Regulation (EC) No 141/2000. 

The centralized authorization procedure is optional for other medicinal products if they contain a new active 
substance or if the applicant shows that the medicinal product concerned constitutes a significant therapeutic, scientific 
or technical innovation or that the granting of authorization is in the interest of patients in the European Union. 

Administrative Procedure 

Under the centralized authorization procedure, the EMA’s Committee for Human Medicinal Products, or CHMP, 
serves as the scientific committee that renders opinions about the safety, efficacy and quality of medicinal products for 
human use on behalf of the EMA. The CHMP is composed of experts nominated by each member state’s national 
authority for medicinal products, with an expert appointed to act as Rapporteur for the co-ordination of the evaluation 
with the possible assistance of a further member of the CHMP acting as a Co-Rapporteur. After approval, the 
Rapporteur(s) continue to monitor the product throughout its life cycle. The CHMP has 210 days to adopt an opinion as 
to whether a marketing authorization should be granted. The process usually takes longer in case additional information 
is requested, which triggers clock-stops in the procedural timelines. The process is complex and involves extensive 
consultation with the regulatory authorities of member states and a number of experts. When an application is submitted 
for a marketing authorization in respect of a drug that is of major interest from the point of view of public health and in 
particular from the viewpoint of therapeutic innovation, the applicant may pursuant to Article 14(9) Regulation 
(EC) No 726/2004 request an accelerated assessment procedure. If the CHMP accepts such request, the time-limit of 
210 days will be reduced to 150 days but it is possible that the CHMP can revert to the standard time-limit for the 
centralized procedure if it considers that it is no longer appropriate to conduct an accelerated assessment. Once the 
procedure is completed, a European Public Assessment Report, or EPAR, is produced. If the opinion is negative, 
information is given as to the grounds on which this conclusion was reached. After the adoption of the CHMP opinion, a 
decision on the MAA must be adopted by the European Commission, after consulting the E.U. member states, which in 
total can take more than 60 days. 

Conditional Approval 

In specific circumstances, E.U. legislation (Article 14(7) Regulation (EC) No 726/2004 and Regulation (EC) No 

507/2006 on Conditional Marketing Authorizations for Medicinal Products for Human Use) enables applicants to obtain 
a conditional marketing authorization prior to obtaining the comprehensive clinical data required for an application for a 
full marketing authorization. Such conditional approvals may be granted for product candidates (including medicines 
designated as orphan medicinal products) if (1) the risk-benefit balance of the product candidate is positive, (2) it is 
likely that the applicant will be in a position to provide the required comprehensive clinical trial data, (3) the product 
fulfills unmet medical needs and (4) the benefit to public health of the immediate availability on the market of the 
medicinal product concerned outweighs the risk inherent in the fact that additional data are still required. A conditional 
marketing authorization may contain specific obligations to be fulfilled by the marketing authorization holder, including 

34 

obligations with respect to the completion of ongoing or new studies, and with respect to the collection of 
pharmacovigilance data. Conditional marketing authorizations are valid for one year, and may be renewed annually, if 
the risk-benefit balance remains positive, and after an assessment of the need for additional or modified conditions or 
specific obligations. The timelines for the centralized procedure described above also apply with respect to the review by 
the CHMP of applications for a conditional marketing authorization. 

Marketing Authorization under Exceptional Circumstances 

Under Article 14(8) Regulation (EC) No 726/2004, products for which the applicant can demonstrate that 
comprehensive data (in line with the requirements laid down in Annex I of Directive 2001/83/EC, as amended) cannot 
be provided (due to specific reasons foreseen in the legislation) might be eligible for marketing authorization under 
exceptional circumstances. This type of authorization is reviewed annually to reassess the risk-benefit balance. The 
fulfillment of any specific procedures/obligations imposed as part of the marketing authorization under exceptional 
circumstances is aimed at the provision of information on the safe and effective use of the product and will normally not 
lead to the completion of a full dossier/approval. 

Market Authorizations Granted by Authorities of E.U. Member States 

In general, if the centralized procedure is not followed, there are three alternative procedures as prescribed in 

Directive 2001/83/EC: 

• 

• 

• 

The decentralized procedure allows applicants to file identical applications to several E.U. member states 
and receive simultaneous national approvals based on the recognition by E.U. member states of an 
assessment by a reference member state. 

The national procedure is only available for products intended to be authorized in a single E.U. member 
state. 

A mutual recognition procedure similar to the decentralized procedure is available when a marketing 
authorization has already been obtained in at least one E.U. member state. 

A marketing authorization may be granted only to an applicant established in the European Union. 

Pediatric Studies 

Prior to obtaining a marketing authorization in the European Union, applicants have to demonstrate compliance 

with all measures included in an EMA-approved Pediatric Investigation Plan, or PIP, covering all subsets of the pediatric 
population, unless the EMA has granted a product-specific waiver, a class waiver, or a deferral for one or more of the 
measures included in the PIP. The respective requirements for all marketing authorization procedures are set forth in 
Regulation (EC) No 1901/2006, which is referred to as the Pediatric Regulation. This requirement also applies when a 
company wants to add a new indication, pharmaceutical form or route of administration for a medicine that is already 
authorized. The Pediatric Committee of the EMA, or PDCO, may grant deferrals for some medicines, allowing a 
company to delay development of the medicine in children until there is enough information to demonstrate its 
effectiveness and safety in adults. The PDCO may also grant waivers when development of a medicine in children is not 
needed or is not appropriate, such as for diseases that only affect the elderly population. 

Before a marketing authorization application can be filed, or an existing marketing authorization can be amended, 

the EMA determines that companies actually comply with the agreed studies and measures listed in each relevant PIP. 

In 2017, the PDCO agreed to our PIP for odevixibat as a treatment for PFIC. 

35 

Periods of Authorization and Renewals 

A marketing authorization is valid for five years in principle and the marketing authorization may be renewed after 

five years on the basis of a re-evaluation of the risk-benefit balance by the EMA or by the competent authority of the 
authorizing member state. To this end, the marketing authorization holder must provide the EMA or the competent 
authority with a consolidated version of the file in respect of quality, safety and efficacy, including all variations 
introduced since the marketing authorization was granted, at least six months before the marketing authorization ceases 
to be valid. Once renewed, the marketing authorization is valid for an unlimited period, unless the European 
Commission or the competent authority decides, on justified grounds relating to pharmacovigilance, to proceed with one 
additional five-year renewal. Any authorization which is not followed by the actual placing of the drug on the E.U. 
market (in case of centralized procedure) or on the market of the authorizing member state within three years after 
authorization ceases to be valid (the so-called sunset clause). 

Orphan Drug Designation and Exclusivity 

The European Commission, following an evaluation by the EMA’s Committee for Orphan Medicinal Products, 

has designated odevixibat as an orphan medicinal product for the treatment of PFIC, as well as for the treatment of PBC 
and ALGS. Pursuant to Regulation (EC) No 141/2000 and Regulation (EC) No. 847/2000, the European Commission 
can grant such orphan medicinal product designation to products for which the sponsor can establish that it is intended 
for the diagnosis, prevention or treatment of a life-threatening or chronically debilitating condition affecting not more 
than five in 10,000 people in the European Union, or a life threatening, seriously debilitating or serious and chronic 
condition in the European Union and that without incentives it is unlikely that sales of the drug in the European Union 
would generate a sufficient return to justify the necessary investment. In addition, the sponsor must establish that there is 
no other satisfactory method approved in the European Union of diagnosing, preventing or treating the condition, or if 
such a method exists, the proposed orphan drug will be of significant benefit to patients. 

Orphan drug designation is not a marketing authorization. It is a designation that provides a number of benefits, 

including fee reductions, regulatory assistance, and the possibility to apply for a centralized E.U. marketing 
authorization, as well as ten years of market exclusivity following marketing authorization of the designated orphan 
drug. During this market exclusivity period, neither the EMA, the European Commission nor the member states can 
accept an application or grant a marketing authorization for a similar medicinal product. A “similar medicinal product” 
is defined as a medicinal product containing a similar active substance or substances as those contained in an authorized 
orphan medicinal product and that is intended for the same therapeutic indication. A “similar active substance” is 
defined as an active substance that is identical or has the same principal molecular structural features (but not necessarily 
all of the same molecular features) and acts via the same mechanism as the authorized orphan medicinal product. The 
market exclusivity period for the authorized therapeutic indication may be reduced to six years if, at the end of the 
fifth year, it is established that the orphan designation criteria are no longer met, including where it is shown that the 
product is sufficiently profitable not to justify maintenance of market exclusivity. In addition, a competing similar 
medicinal product may in limited circumstances be authorized prior to the expiration of the market exclusivity period, 
including if it is shown to be safer, more effective or otherwise clinically superior to the already approved orphan drug. 
Furthermore, a product can lose orphan designation and the related benefits, prior to us having obtained a marketing 
authorization, if it is demonstrated that the orphan designation criteria are no longer met. 

Regulatory Data Protection 

E.U. legislation also provides for a system of regulatory data and market exclusivity. According to Article 14(11) 
of Regulation (EC) No 726/2004, as amended, and Article 10(1) of Directive 2001/83/EC, as amended, upon receiving 
marketing authorization, new chemical entities approved on the basis of complete independent data package benefit from 
eight years of data exclusivity and an additional two years of market exclusivity. Data exclusivity prevents regulatory 
authorities in the European Union from referencing the innovator’s data to assess a generic (abbreviated) application. 
During the additional two-year period of market exclusivity, a generic marketing authorization can be submitted, and the 
innovator’s data may be referenced, but no generic medicinal product can be marketed until the expiration of the market 
exclusivity. The overall ten-year period will be extended to a maximum of 11 years if, during the first eight years of 

36 

those ten years, the marketing authorization holder, or MAH, obtains an authorization for one or more new therapeutic 
indications which, during the scientific evaluation prior to their authorization, are held to bring a significant clinical 
benefit in comparison with existing therapies. Even if a compound is considered to be a new chemical entity and the 
innovator is able to gain the period of data exclusivity, another company nevertheless could also market another version 
of the drug if such company obtained marketing authorization based on an MAA with a complete independent data 
package of pharmaceutical test, preclinical tests and clinical trials. However, products designated as orphan medicinal 
products enjoy, upon receiving marketing authorization, a period of ten years of orphan market exclusivity—see also 
“Orphan Drug Designation and Exclusivity.” Depending upon the timing and duration of the E.U. marketing 
authorization process, products may be eligible for up to five years’ supplementary protection certificates, or SPCs, 
pursuant to Regulation (EC) No 469/2009. Such SPCs extend the rights under the basic patent for the drug. 

Regulatory Requirements After a Marketing Authorization has been Obtained 

If we obtain authorization for a medicinal product in the European Union, we will be required to comply with a 

range of requirements applicable to the manufacturing, marketing, promotion and sale of medicinal products: 

Pharmacovigilance and Other Requirements 

We will, for example, have to comply with the E.U.’s stringent pharmacovigilance or safety reporting rules, 
pursuant to which post-authorization studies and additional monitoring obligations can be imposed. Other requirements 
relate, for example, to the manufacturing of products and APIs in accordance with good manufacturing practice 
standards. E.U. regulators may conduct inspections to verify our compliance with applicable requirements, and we will 
have to continue to expend time, money and effort to remain compliant. Noncompliance with E.U. requirements 
regarding safety monitoring or pharmacovigilance, or requirements related to the development of products for the 
pediatric population, can also result in significant financial penalties in the European Union. Similarly, failure to comply 
with the E.U.’s requirements regarding the protection of individual personal data can also lead to significant penalties 
and sanctions. Individual E.U. member states may also impose various sanctions and penalties in case we do not comply 
with locally applicable requirements. 

Manufacturing 

The manufacturing of authorized drugs, for which a separate manufacturer’s license is mandatory, must be 
conducted in strict compliance with the EMA’s cGMP requirements and comparable requirements of other regulatory 
bodies in the European Union, which mandate the methods, facilities and controls used in manufacturing, processing and 
packing of drugs to assure their safety and identity. The EMA enforces its cGMP requirements through mandatory 
registration of facilities and inspections of those facilities. The EMA may have a coordinating role for these inspections 
although the responsibility for carrying them out rests with the member states’ competent authority under whose 
responsibility the manufacturer falls. Failure to comply with these requirements could interrupt supply and result in 
delays, unanticipated costs and lost revenues, and could subject the applicant to potential legal or regulatory action, 
including but not limited to warning letters, suspension of manufacturing, seizure of product, injunctive action or 
possible civil and criminal penalties. 

Marketing and Promotion 

The marketing and promotion of authorized drugs, including industry-sponsored continuing medical education and 

advertising directed toward the prescribers of drugs or the general public, are strictly regulated in the European Union 
under Directive 2001/83/EC. The applicable regulations aim to ensure that information provided by holders of marketing 
authorizations regarding their products is truthful, balanced and accurately reflects the safety and efficacy claims 
authorized by the EMA or by the competent authority of the authorizing member state. Failure to comply with these 
requirements can result in adverse publicity, warning letters, corrective advertising and potential civil and criminal 
penalties. 

37 

Patent Term Extension 

In order to compensate the patentee for delays in obtaining a marketing authorization for a patented product, an 

SPC may be granted extending the exclusivity period for that specific product by up to five years. Applications for SPCs 
must be made to the relevant patent office in each E.U. member state and the granted certificates are valid only in the 
member state of grant. An application has to be made by the patent owner within six months of the first marketing 
authorization being granted in the European Union (assuming the patent in question has not expired, lapsed or been 
revoked) or within six months of the grant of the patent (if the marketing authorization is granted first). In the context of 
SPCs, the term “product” means the active ingredient or combination of active ingredients for a medicinal product and 
the term “patent” means a patent protecting such a product or a new manufacturing process or application for it. The 
duration of an SPC is calculated as the difference between the patent’s filing date and the date of the first marketing 
authorization, minus five years, subject to a maximum term of five years. 

A six month pediatric extension of an SPC may be obtained where the patentee has carried out an agreed pediatric 
investigation plan, the authorized product information includes information on the results of the studies and the product 
is authorized in all member states of the European Union. 

Brexit and the Regulatory Framework in the United Kingdom  

In June 2016, the electorate in the United Kingdom voted in favor of leaving the European Union (commonly 
referred to as “Brexit”). Following the formation of a majority Conservative government in December 2019, the United 
Kingdom approved the withdrawal agreement and left the European Union on January 31, 2020, with a transitional 
period that expired on December 31, 2020. In connection with Brexit, the United Kingdom and the European Union 
entered into a trade agreement known as the Trade and Cooperation Agreement, which is provisionally applicable as of 
January 1, 2021 but has not yet been ratified by the European Parliament. This agreement is intended to govern the legal 
relationship between the European Union and the United Kingdom post-Brexit. We are currently evaluating the potential 
impacts of the new Trade and Cooperation Agreement on our business.  

Since the regulatory framework for pharmaceutical products in the United Kingdom covering quality, safety and 

efficacy of pharmaceutical products, clinical trials, marketing authorization, commercial sales and distribution of 
pharmaceutical products was, prior to Brexit, derived from European Union directives and regulations, and the 
implementation of the Trade and Cooperation Agreement  could materially impact the future regulatory regime which 
applies to products and the approval of product candidates in the United Kingdom. It remains to be seen how, if at all, 
Brexit and the implementation of the Trade and Cooperation Agreement will impact regulatory requirements for product 
candidates and products in the United Kingdom. 

Pharmaceutical Coverage, Pricing and Reimbursement 

Sales of products approved by the FDA will depend, in part, on the extent to which the costs of the products will 

be covered by third-party payors, including government health programs in the United States such as Medicare and 
Medicaid, commercial health insurers and managed care organizations. The process for determining whether a payor will 
provide coverage for a product may be separate from the process for setting the price or reimbursement rate that the 
payor will pay for the product once coverage is approved. Third-party payors may limit coverage to specific products on 
an approved list, or formulary, which might not include all of the approved products for a particular indication. 

In order to secure coverage and reimbursement for any product that might be approved for sale, a company may 

need to conduct expensive pharmacoeconomic studies in order to demonstrate the medical necessity and cost-
effectiveness of the product, in addition to the costs required to obtain FDA or other comparable regulatory approvals. A 
payor’s decision to provide coverage for a drug product does not imply that an adequate reimbursement rate will be 
approved. Third-party reimbursement may not be sufficient to maintain price levels high enough to realize an 
appropriate return on investment in product development. 

38 

In the European Union, pricing and reimbursement schemes vary widely from country to country. Some countries 

provide that drug products may be marketed only after a reimbursement price has been agreed. Some countries may 
require the completion of additional studies that compare the cost-effectiveness of our product candidate to currently 
available therapies (so called health technology assessment, or HTA) in order to obtain reimbursement or pricing 
approval. For example, the European Union provides options for its member states to restrict the range of drug products 
for which their national health insurance systems provide reimbursement and to control the prices of medicinal products 
for human use. E.U. member states may approve a specific price for a drug product or it may instead adopt a system of 
direct or indirect controls on the profitability of the company placing the drug product on the market. Other member 
states allow companies to fix their own prices for drug products but monitor and control prescription volumes and issue 
guidance to physicians to limit prescriptions. The downward pressure on health care costs in general, particularly 
prescription drugs, has become intense. As a result, increasingly high barriers are being erected to the entry of new 
products. In addition, there can be considerable pressure by governments and other stakeholders on prices and 
reimbursement levels, including as part of cost containment measures. Political, economic and regulatory developments 
may further complicate pricing negotiations, and pricing negotiations may continue after reimbursement has been 
obtained. Reference pricing used by various E.U. member states, and parallel distribution (arbitrage between low-priced 
and high-priced member states), can further reduce prices. Any country that has price controls or reimbursement 
limitations for drug products may not allow favorable reimbursement and pricing arrangements. 

Healthcare Law and Regulation 

If our product candidates are approved in the United States, we will have to comply with various U.S. federal and 

state laws, rules and regulations pertaining to healthcare fraud and abuse, including anti-kickback laws and physician 
self-referral laws, rules and regulations. Violations of the fraud and abuse laws are punishable by criminal and civil 
sanctions, including, in some instances, exclusion from participation in federal and state healthcare programs, including 
Medicare and Medicaid. These laws include: 

• 

• 

• 

• 

• 

the federal anti-kickback statute prohibits, among other things, persons from knowingly and willfully 
soliciting, offering, receiving or paying remuneration, directly or indirectly, in cash or in kind, to induce or 
reward either the referral of an individual for, or the purchase, order or recommendation of, any good or 
service, for which payment may be made, in whole or in part, under a federal healthcare program such as 
Medicare and Medicaid; 

the federal False Claims Act imposes civil penalties, and provides for civil whistleblower or qui tam actions, 
against individuals or entities for knowingly presenting, or causing to be presented, to the federal 
government, claims for payment that are false or fraudulent or making a false statement to avoid, decrease or 
conceal an obligation to pay money to the federal government; 

the federal Health Insurance Portability and Accountability Act of 1996, or HIPAA, imposes criminal and 
civil liability for executing a scheme to defraud any healthcare benefit program or making false statements 
relating to healthcare matters; 

HIPAA, as amended by the Health Information Technology for Economic and Clinical Health Act, or 
HITECH Act, and its implementing regulations, also imposes obligations, including mandatory contractual 
terms, with respect to safeguarding the privacy, security and transmission of individually identifiable health 
information;  

the federal transparency requirements under the Physician Payments Sunshine Act require manufacturers of 
FDA-approved drugs, devices, biologics and medical supplies covered by Medicare or Medicaid to report, 
on an annual basis, to the Department of Health and Human Services information related to payments and 
other transfers of value to physicians and teaching hospitals and physician ownership and investment 
interests; and 

39 

• 

analogous state and foreign laws and regulations, such as state anti-kickback and false claims laws, may 
apply to sales or marketing arrangements and claims involving healthcare items or services reimbursed by 
nongovernmental third-party payors, including private insurers. 

Some state laws require pharmaceutical companies to comply with the pharmaceutical industry’s voluntary 

compliance guidelines, or the relevant compliance guidance promulgated by the federal government, in addition to 
requiring drug manufacturers to report information related to payments to physicians and other health care providers or 
marketing expenditures to the extent that those laws impose requirements that are more stringent than the Physician 
Payments Sunshine Act. State and foreign laws also govern the privacy and security of health information in some 
circumstances, many of which differ from each other in significant ways and often are not preempted by HIPAA, thus 
complicating compliance efforts. 

Employees 

As of January 15, 2021, we employed 90 full-time employees, of whom 23 hold Ph.D. or M.D. degrees, or the 

foreign equivalent. Of these employees, 51 were engaged in research and development and 39 were engaged in general 
and administrative functions. Our headquarters are in Boston, MA and we have a research site in Gothenburg Sweden. 
Our employees in Sweden are subject to a collective bargaining agreement. We consider our relationship with our 
employees to be good. 

The Company recognizes that our industry is specialized and dynamic, and a significant aspect of our success is 

our continued ability to execute our human capital strategy of attracting, engaging, developing and retaining highly 
skilled talent. There is fierce competition both within our industry and in the geographic locations in which we have 
offices for highly skilled talent, and we offer a robust set of benefits, career-enhancing learning experiences and 
initiatives aligned with our mission, vision, and values in order to attract qualified prospective employees and to retain 
and motivate our employees. We offer competitive compensation for our employees and strongly embrace a pay for 
performance philosophy in setting and adjusting compensation.  

Our Code of Business Conduct and Ethics clearly outlines our unwavering commitment to diversity and inclusion, 
where all employees are welcomed in an environment designed to make them feel comfortable, respected, and accepted 
regardless of their age, race, national origin, gender, religion, disability or sexual orientation. We have a set of policies 
explicitly setting forth our expectations for nondiscrimination and a harassment-free work environment. We are also a 
proud equal opportunity employer and cultivate a highly collaborative and entrepreneurial culture. 

Corporate Information 

Our internet address is http://www.albireopharma.com. The information contained on, or that can be accessed 

through, our website is not a part of or incorporated by reference in this Annual Report on Form 10-K. Our annual 
reports on Form 10-K, quarterly reports on Form 10-Q, current reports on Form 8-K, and all amendments to those 
reports, are available to you free of charge through the Media & Investors section of our website as soon as reasonably 
practicable after such materials have been electronically filed with, or furnished to, the SEC. 

Item 1A.  RISK FACTORS 

Risk Factor Summary 

We are providing the following summary of the risk factors contained in this Annual Report on Form 10-K to 
enhance the readability and accessibility of our risk factor disclosures. We encourage you to carefully review the full risk 
factors contained in this Annual Report on Form 10-K in their entirety for additional information regarding the material 

40 

 
 
 
factors that make an investment in our securities speculative or risky. These risks and uncertainties include, but are not 
limited to, the following: 

•  We have incurred significant losses since our inception. We expect to continue to incur losses and may never 

generate profits from operations or maintain profitability. 

•  We will need substantial additional funding. If we are unable to raise capital when needed, we could be forced 

to delay, reduce or eliminate our product development programs or commercialization efforts. 

•  We depend heavily on the success of our lead product candidate, odevixibat, which we are developing initially 
for the treatment of patients with PFIC and potentially also for other pediatric cholestatic liver diseases and 
disorders. If we are unable to commercialize odevixibat or experience significant delays in doing so, our 
business will be materially harmed. 

• 

If clinical trials of odevixibat or any of our other product candidates fail to demonstrate safety and efficacy to 
the satisfaction of the FDA or the EMA, or do not otherwise produce favorable results satisfactory to the FDA 
or EMA, we may incur additional costs or experience delays in completing, or ultimately be unable to 
complete, the development and commercialization of the applicable product candidate. 

•  Favorable results seen to date in clinical trials of odevixibat, including our Phase 3 trial of odevixibat in 

patients with PFIC, may not be predictive of favorable results in our ongoing BOLD pivotal trial of odevixibat 
in biliary atresia or our ongoing ASSERT pivotal trial of odevixibat in ALGS. 

•  The clinical trial designs, durations, endpoints and outcomes that will be required to obtain marketing 

approval of odevixibat to treat PFIC patients are uncertain and, in any case, may vary among the FDA, EMA 
and other regulatory authorities outside of the United States and Europe. Based on feedback that we have 
received from the FDA and the EMA, we expect both regulatory authorities to place a greater emphasis on the 
totality of the data from our Phase 3 clinical trial, including secondary endpoints, than may generally be 
expected. As a result, there is risk that, even though the primary endpoint of our Phase 3 clinical trial of 
odevixibat for FDA evaluation purposes and the primary endpoint for EMA evaluation purposes were both 
met with statistical significance, the applicable regulatory authority may not find the overall results of our 
Phase 3 trial to be sufficient to support marketing approval of odevixibat to treat PFIC, a symptom of PFIC 
such as pruritus or any other indication, and we may never receive marketing approval. 

•  We cannot be assured that either the FDA or EMA will complete their respective reviews of our submissions 
for approval of odevixibat within the target timelines, including the FDA’s Prescription Drug User Fee Act 
goal date of July 20, 2021, as a potential result of the impact of the COVID-19 pandemic or otherwise. 

•  The design of our Phase 3 clinical trial of odevixibat in patients with PFIC does not conform precisely in all 

respects to the recommendations or preferences expressed by either the FDA or EMA. 

• 

• 

• 

If we experience delays or difficulties in the enrollment of patients in our pivotal clinical trials of odevixibat 
in patients with biliary atresia and ALGS, our receipt of marketing approval for odevixibat in those 
indications could be delayed or prevented. 

If the commercial opportunity in PFIC is smaller than we anticipate, or if odevixibat receives approval to treat 
only a specific subpopulation of patients with PFIC or only a specific symptom of PFIC such as pruritus, our 
future revenue from odevixibat may be adversely affected and our business may suffer. 

If we are unable to establish sales and marketing capabilities or enter into agreements with third parties to 
market and sell odevixibat or any of our other current or potential future product candidates, we may not be 
successful in commercializing the applicable product candidate if it receives marketing approval. 

•  We face substantial competition, which may result in others discovering, developing or commercializing 

products to treat our target indications or markets before or more successfully than we do. 

41 

•  Use of third parties to manufacture our product candidates may increase the risk that we will not have 

sufficient quantities of our product candidates or products or such quantities at an acceptable cost, which 
could delay, prevent or impair our development or commercialization efforts. 

• 

If we are unable to obtain and maintain patent protection for our technology and products, or if the scope of 
the patent protection is not sufficiently broad, our competitors could develop and commercialize technology 
and products similar or identical to ours, and our ability to successfully commercialize our technology and 
products may be adversely affected. 

•  Even if we complete the necessary clinical trials, the marketing approval process is expensive, time 

consuming and uncertain and may prevent us from obtaining approvals for the commercialization of some or 
all of our product candidates. If we are not able to obtain, or if there are delays in obtaining, required 
marketing approvals, we will not be able to commercialize our product candidates, and our ability to generate 
revenue will be materially impaired. 

•  The outbreak of the novel strain of coronavirus, SARS-CoV-2, which causes COVID-19, could adversely 
impact our business, including our preclinical studies and clinical trials and the commercialization of any 
approved product. 

The terms of our loan and security agreement with Hercules Capital, Inc. require us to meet certain operating 
covenants and place restrictions on our operating and financial flexibility. If we raise additional capital through debt 
financing, the terms of any new debt could further restrict our ability to operate our business. 

Risk Factors 

An investment in shares of our common stock involves a high degree of risk. You should carefully consider the 

following information about these risks, together with the other information appearing elsewhere in this Annual Report 
on Form 10-K, including our consolidated financial statements and related notes thereto, before deciding to invest in 
our common stock. The occurrence of any of the following risks could have a material adverse effect on our business, 
financial condition, results of operations and future growth prospects. In these circumstances, the market price of our 
common stock could decline, and you may lose all or part of your investment. 

Risks Related to Our Financial Position and Need for Additional Capital 

We have incurred significant losses since our inception. We expect to continue to incur losses and may never 

generate profits from operations or maintain profitability. 

Since inception, we have incurred significant operating losses. Our net loss was approximately $107.6 million for 
the year ended December 31, 2020 and approximately $62.7 million for the year ended December 31, 2019. We had an 
accumulated deficit of $266.8 million as of December 31, 2020. To date, we have financed our operations primarily 
through issuances of shares of common stock, preference shares or convertible loan notes, upfront fees paid upon 
entering into or amending license agreements, payments received upon the achievement of specified milestone events 
under the license agreements, grants, venture debt borrowings, and the HCR royalty monetization transactions.. We have 
devoted substantially all of our efforts to research and development, including clinical trials. We have not completed the 
development of any drugs, although our licensee, EA Pharma has received approval in Japan of our product candidate 
elobixibat to treat chronic constipation. We expect to continue to incur significant expenses and increasing operating 
losses for at least the next few years as we continue our development of, and seek marketing approvals for, our product 
candidates, prepare for and begin the commercialization of any approved products, and add infrastructure and personnel 
to support our product development efforts and operations as a public company in the United States. The net losses we 
incur may fluctuate significantly from quarter to quarter and year to year. 

42 

Our ability to generate profits from operations and thereafter to remain profitable depends heavily on: 

• 

• 

• 

• 

• 

• 

• 

• 

• 

• 

• 

• 

• 

• 

• 

• 

• 

• 

any unfavorable development or delay in our odevixibat program in PFIC, including the review or approval 
of our odevixibat marketing applications we submitted in the fourth quarter of 2020 in the United States and 
Europe for PFIC and the costs and timing of our pre-commercialization preparations; 

the costs, design, duration and any potential delays of the pivotal clinical trial of odevixibat in biliary atresia 
and pivotal trial of odevixibat in ALGS; 

 the scope, number, progress, initiation, duration, cost, results and timing of clinical trials and nonclinical 
studies of our current or future product candidates; 

whether and to what extent milestone events are achieved under our license agreement with EA Pharma or 
any potential future licensee or collaborator; 

the outcomes and timing of regulatory reviews, approvals or other actions; 

our ability to obtain marketing approval for our product candidates; 

our ability to establish and maintain additional licensing, collaboration or similar arrangements on favorable 
terms and whether and to what extent we retain development or commercialization responsibilities under any 
new licensing, collaboration or similar arrangement; 

the success of any other business, product or technology that we acquire or in which we invest; 

our ability to maintain, expand and defend the scope of our intellectual property portfolio; 

our ability to manufacture any approved products on commercially reasonable terms; 

our ability to establish a sales and marketing organization or suitable third-party alternatives for any 
approved product;  

the number and characteristics of product candidates and programs that we pursue. 

the potential impacts of the COVID-19 pandemic on our business; 

the costs of acquiring, licensing or investing in businesses, product candidates and technologies; 

our need and ability to hire additional management and scientific and medical personnel; 

the costs to operate as a public company in the United States, including the need to implement additional 
financial and reporting systems and other internal systems and infrastructure for our business; 

market acceptance of our product candidates, to the extent any are approved for commercial sale; and 

the effect of competing technological and market developments. 

Based on our current plans, we do not expect to generate significant revenue from product sales unless and until 

we or a potential future licensee or collaborator obtains marketing approval for, and commercializes, one or more of our 
current or potential future product candidates (other than elobixibat as a treatment for chronic constipation in Japan), 
which we do not expect to occur until at least the second half of 2021, if at all. Neither we nor a licensee may ever 
succeed in obtaining marketing approval for, or commercializing, our product candidates besides elobixibat as a 

43 

treatment for chronic constipation in Japan and, even if we do, may never generate revenues that are significant enough 
to generate profits from operations. Even if we do generate profits from operations, we may not be able to sustain or 
increase profitability on a quarterly or annual basis. Our failure to generate profits from operations and remain profitable 
would decrease our value and could impair our ability to raise capital, expand our business, maintain our research and 
development efforts, diversify our product offerings or continue our operations. A decline in our value could also cause 
you to lose all or part of your investment. 

We will need substantial additional funding. If we are unable to raise capital when needed, we could be forced 

to delay, reduce or eliminate our product development programs or commercialization efforts. 

We expect our research and development expenses to increase substantially in future periods, particularly as we 
advance the development of our lead product candidate odevixibat beyond its development as a treatment for patients 
with PFIC into other pediatric cholestatic liver diseases and disorders, such as our development of odevixibat as a 
treatment for biliary atresia and as a treatment for ALGS. In addition, we expect that our research and development 
expenses would increase if we initiate additional preclinical programs for potential future product candidates. In 
addition, if we obtain marketing approval for any of our product candidates that are not then subject to licensing, 
collaboration or similar arrangements with third parties, we expect to continue to incur significant commercialization 
expenses related to product sales, marketing, distribution and manufacturing. Furthermore, we expect to incur additional 
costs associated with operating as a public company in the United States. Accordingly, we will need to obtain substantial 
additional funding in connection with our continuing operations. If we are unable to raise capital when needed or on 
attractive terms, we could be forced to delay, reduce or eliminate our research and development programs or any future 
commercialization efforts. 

Our future capital requirements will depend on many factors, including: 

• 

• 

• 

• 

• 

• 

• 

• 

• 

whether the FDA and the EMA determines that our Phase 3 clinical trial of odevixibat in PFIC is or is not 
sufficient for regulatory approval to treat PFIC, a symptom of PFIC, a specific PFIC subtype(s) or otherwise; 

our ability to establish a sales and marketing organization for the potential commercialization of odevixibat 
in patients with PFIC, or a sales and marketing organization or suitable third-party alternatives for any other 
product candidate, if approved; 

the costs, design, duration and any potential delays of the pivotal trial of odevixibat in biliary atresia or the 
pivotal trial of odevixibat in ALGS; 

the same factors that our ability to generate profits from operations and thereafter to remain profitable 
depend heavily on, as described above under “— We have incurred significant losses since our inception. 
We expect to continue to incur losses and may never generate profits from operations or maintain 
profitability.”; 

the scope, number, progress, duration, cost, results and timing of clinical trials and nonclinical studies of our 
current or future product candidates; 

the outcomes and timing of regulatory reviews, approvals or other actions; 

our ability to obtain marketing approval for our product candidates; 

our ability to establish and maintain additional licensing, collaboration or similar arrangements on favorable 
terms and whether and to what extent we retain development or commercialization responsibilities under any 
new licensing, collaboration or similar arrangement; 

the success of any other business, product or technology that we acquire or in which we invest; 

44 

• 

• 

• 

• 

• 

• 

• 

• 

our ability to maintain, expand and defend the scope of our intellectual property portfolio; 

our ability to manufacture any approved products on commercially reasonable terms; 

the number and characteristics of product candidates and programs that we pursue; 

the costs of acquiring, licensing or investing in businesses, product candidates and technologies; 

our need and ability to hire additional management and scientific and medical personnel; 

the costs to operate as a public company in the United States, including the need to implement additional 
financial and reporting systems and other internal systems and infrastructure for our business; 

market acceptance of our product candidates, to the extent any are approved for commercial sale; and 

the effect of competing technological and market developments. 

Conducting preclinical testing and clinical trials is a time-consuming, expensive and uncertain process that 
takes years to complete, and we may never generate the necessary data or results required to obtain marketing approval 
and achieve product sales. In addition, our product candidates, if approved, may not achieve commercial success. Our 
commercial revenues, if any, will be derived from sales of products that will not be commercially available for sale by us 
until at least the second half of 2021, if at all. Accordingly, we will need to continue to rely on additional financing to 
achieve our business objectives. In addition, we may seek additional capital due to favorable market conditions or 
strategic considerations, even if we believe that we have sufficient funds for our current or future operating plans. 
Additional financing may not be available to us on acceptable terms, or at all. The unavailability of additional financing 
on acceptable terms, or at all, would have an adverse effect on your investment. 

Raising additional capital may cause dilution to our investors, restrict our operations or require us to relinquish 

rights to our technologies or product candidates. 

Until such time, if ever, as we can generate substantial product revenues, we expect to finance our cash needs 

through a combination of equity offerings, licensing, collaboration or similar arrangements, grants and debt financings. 
We do not have any committed external source of funds. On May 7, 2020, we filed a universal shelf registration on 
Form S-3 with the SEC, which was declared effective on May 18, 2020, pursuant to which we registered for sale up to 
$200 million of any combination of our common stock, preferred stock, debt securities, warrants, rights and/or units 
from time to time and at prices and on terms that we may determine, including up to $50 million of our common stock 
available for issuance pursuant to an at-the-market offering program sales agreement that we entered into with Cowen 
and Company, LLC, or Cowen, in May 2020. The agreement was terminated on September 9, 2020. As of December 31, 
2020, $40.0 million of securities remain available for issuance under the shelf registration statement. We refer to this 
registration statement as the 2020 Form S-3. On or about February 25, 2021, we expect to file a new universal shelf 
registration on Form S-3 with the SEC, pursuant to which we expect to register for sale up to $400 million of any 
combination of our common stock, preferred stock, debt securities, warrants, rights and/or units from time to time and at 
prices and on terms that we may determine, which we refer to as the 2021 Form S-3. On or about February 25, 2021, we 
intend to enter into a new sales agreement, which we refer to as the 2021 Sales Agreement, with respect to an at-the-
market offering program under which we may offer and sell, from time to time at our sole discretion, shares of our 
common stock having an aggregate offering price of up to $100 million. Our issuance and sale, if any, of shares under 
the 2021 Sales Agreement that we intend to enter into are subject to the effectiveness of the 2021 Form S-3. We make no 
assurances as to if or when the 2021 Form S-3 will become effective or, if it does become effective, as to the continued 
effectiveness of the 2021 Form S-3. No additional securities registered under the 2020 Form S-3 will be offered or sold 
after the date of effectiveness of the 2021 Form S-3.  This report shall not constitute an offer to sell or the solicitation of 
an offer to buy any shares under the 2021 Sales Agreement that we intend to enter into or any securities under the 2021 
Form S-3 that we intend to file with the SEC, nor shall there be any sale of such securities in any state in which such 
offer, solicitation or sale would be unlawful prior to registration or qualification under the securities laws of any such 
state. 

45 

We may seek to raise additional capital at any time. To the extent that we raise additional capital through the sale 

of equity or convertible debt securities, your ownership interest will be diluted, and the terms of these securities may 
include liquidation or other preferences that adversely affect your rights as a holder of our common stock. Debt 
financing, if available, may involve agreements that include covenants limiting or restricting our ability to take specific 
actions, such as incurring additional debt, making capital expenditures or declaring dividends or other distributions. 

If we raise additional funds through licensing, collaboration or similar arrangements, we may have to relinquish 

valuable rights to our technologies, future revenue streams, research and development programs or product candidates or 
to grant licenses on terms that may not be favorable to us. If we are unable to raise additional funds through equity or 
debt financings or other arrangements when needed, we may be required to delay, limit, reduce or terminate our product 
development or future commercialization efforts or grant rights to develop and market product candidates that we would 
otherwise prefer to develop and market ourselves. 

Inadequate funding for the FDA, the SEC and other government agencies, or a work slowdown or stoppage at 

those agencies as part of a broader federal government shutdown, could hinder their ability to hire and retain key 
leadership and other personnel, prevent new products and services from being developed or commercialized in a 
timely manner, or otherwise prevent those agencies from performing normal business functions on which the 
operation of our business may rely, which could negatively impact our business.  

The ability of the FDA to review and approve new products can be affected by a variety of factors, including 
government budget and funding levels, ability to hire and retain key personnel and accept the payment of user fees, and 
statutory, regulatory and policy changes. Average review times at the agency have fluctuated in recent years as a result. 
In addition, funding of government agencies on which our operations may rely, including those that fund research and 
development activities, is subject to the political process, which is inherently fluid and unpredictable.  

Disruptions at the FDA and other agencies may also slow the time necessary for new drugs to be reviewed and/or 

approved by necessary government agencies, which would adversely affect our business. For example, over the last 
several years, the U.S. government has shut down several times and certain regulatory agencies, such as the FDA and the 
SEC, have had to furlough critical FDA, SEC and other government employees and stop critical activities. If a prolonged 
government shutdown recurs, it could significantly impact the ability of the FDA to timely review and process our 
regulatory submissions, which could have a material adverse effect on our business. Further, future government 
shutdowns could impact our ability to access the public markets and obtain necessary capital in order to properly 
capitalize and continue our operations. 

The continuing outbreak of the novel strain of coronavirus, SARS-CoV-2, which causes COVID-19, could 

adversely impact our business, including our preclinical studies and clinical trials. 

Public health crises such as pandemics or similar outbreaks could adversely impact our business. In 

December 2019, a novel strain of coronavirus, SARS-CoV-2, which causes coronavirus disease 2019 (COVID-19), 
surfaced in Wuhan, China. Since then, COVID-19 has spread to countries across the world, including the United States. 
In response to the spread of COVID-19, we have closed our executive offices with our administrative employees 
continuing their work outside of our offices, restricted on-site staff to only those required to execute their job 
responsibilities and limited the number of staff in any given research and development laboratory. 

As a result of the COVID-19 outbreak, or similar pandemics, we have and may in the future experience 

disruptions that could severely impact our business, preclinical studies and clinical trials, including: 

• 

• 

• 

delays or difficulties in enrolling patients in our clinical trials; 

delays or difficulties in clinical site initiation, including difficulties in recruiting clinical site investigators and 
clinical site staff; 

delays or disruptions in preclinical studies or clinical trials due to unforeseen circumstances at contract 
research organizations and vendors along their supply chain; 

46 

 
 
 
 
 
 
• 

• 

• 

• 

• 

• 

increased rates of patients withdrawing from our clinical trials following enrollment as a result of contracting 
COVID-19, being forced to quarantine, or not being willing to travel to clinical trial sites; 

diversion of healthcare resources away from the conduct of clinical trials, including the diversion of 
hospitals serving as our clinical trial sites and hospital staff supporting the conduct of our clinical trials; 

interruption of key clinical trial activities, such as clinical trial site data monitoring, due to limitations on 
travel imposed or recommended by federal or state governments, employers and others or interruption of 
clinical trial subject visits and study procedures (particularly any procedures that may be deemed non-
essential), which may impact the integrity of subject data and clinical study endpoints; 

interruption or delays in the operations of the FDA and comparable foreign regulatory agencies, which may 
impact approval timelines, as described further below under “Risks Related to the Development and 
Commercialization of Our Product Candidates - Our pending NDA seeking approval to market 
odevixibat for the treatment of patients with PFIC may be delayed as a result of ongoing COVID-19 travel 
restrictions and safety protocols that could affect FDA’s ability to conduct a pre-approval inspection of 
the drug product’s manufacturing facility, or as a result of operational delays at the FDA or the EMA due 
to a redeployment of staff or other resources as part of the global response to the COVID-19 pandemic.” 

interruption of, or delays in receiving, supplies of our product candidates from our contract manufacturing 
organizations due to staffing shortages, production slowdowns or stoppages and disruptions in delivery 
systems; and 

limitations on employee resources that would otherwise be focused on the conduct of our preclinical studies 
and clinical trials, including because of sickness of employees or their families, the desire of employees to 
avoid contact with large groups of people, continued reliance on working from home or mass transit 
disruptions. 

These and other factors arising from the COVID-19 pandemic could worsen in countries that are already afflicted 

with COVID-19, could continue to spread to additional countries, or could return to countries where the pandemic has 
been partially contained, each of which could further adversely impact our ability to conduct clinical trials and our 
business generally, and could have a material adverse impact on our operations and financial condition and results. 

In addition, the trading prices for our common stock and the securities of other biopharmaceutical companies have 
been highly volatile as a result of the COVID-19 pandemic. As a result, we may face difficulties raising capital through 
sales of our common stock or such sales may be on unfavorable terms. The COVID-19 outbreak continues to rapidly 
evolve. The extent to which the outbreak may impact our business, preclinical studies and clinical trials will depend on 
future developments, which are highly uncertain and cannot be predicted with confidence, such as the ultimate 
geographic spread of the disease, the duration of the outbreak, travel restrictions and actions to contain the outbreak or 
treat its impact, such as social distancing and quarantines or lock-downs in the United States and other countries, 
business closures or business disruptions and the effectiveness of actions taken in the United States and other countries 
to contain and treat the disease. 

The terms of our loan and security agreement with Hercules Capital require us to meet certain operating 
covenants and place restrictions on our operating and financial flexibility. If we raise additional capital through debt 
financing, the terms of any new debt could further restrict our ability to operate our business. 

On June 8, 2020, we entered into a loan and security agreement, or the Loan Agreement, with Hercules Capital, 
Inc., in its capacity as administrative and collateral agent for itself and the other Lenders party to the Loan Agreement. 
The loan advanced under the Loan Agreement, or the Term Loan, is secured by a security interest covering our assets, 
other than our intellectual property and other customary collateral exclusions. The Loan Agreement contains customary 
affirmative and negative covenants and events of default. Affirmative covenants include, among others, covenants 
requiring us to maintain our legal existence and comply with all applicable laws, deliver certain financial reports, 

47 

 
 
 
 
 
 
 
 
maintain a minimum cash balance, and maintain insurance coverage. Negative covenants include, among others, 
covenants restricting us from transferring any part of our business or intellectual property, incurring additional 
indebtedness, engaging in mergers or acquisitions, repurchasing shares, paying dividends or making other distributions, 
making investments, and creating other liens on our assets, including our intellectual property, in each case subject to 
customary exceptions. If we raise any additional debt financing, the terms of such additional debt could further restrict 
our operating and financial flexibility. These restrictions may include, among other things, limitations on borrowing and 
specific restrictions on the use of our assets, as well as prohibitions on our ability to create liens, pay dividends, redeem 
capital stock or make investments. If we default under the terms of the Loan Agreement or any future debt facility, the 
Lenders may accelerate all of our repayment obligations and take control of our pledged assets, potentially requiring us 
to renegotiate our agreement on terms less favorable to us or to immediately cease operations. Further, if we are 
liquidated, the Lenders’ right to repayment would be senior to the rights of the holders of our common stock. The 
Lenders could declare a default upon the occurrence of any event that it interprets as a material adverse effect as defined 
under the Loan Agreement. Any declaration by the Lenders of an event of default could significantly harm our business 
and prospects and could cause the price of our common stock to decline. If we raise any additional debt financing, the 
terms of such additional debt could further restrict our operating and financial flexibility. 

Repayment of the Term Loan will require a significant amount of cash, and we may not have sufficient cash 

flow from our business to make payments on our indebtedness. 

Our ability to pay the principal of and/or interest on the Term Loan depends on our future performance, which is 

subject to economic, financial, competitive and other factors beyond our control. Our business may not generate cash 
flow from operations in the future sufficient to service the Term Loan or other future indebtedness and make necessary 
capital expenditures. If we are unable to generate such cash flow, we may be required to adopt and implement one or 
more alternatives, such as selling assets, restructuring indebtedness or obtaining additional debt financing or equity 
financing on terms that may be onerous or highly dilutive. Our ability to refinance the Term Loan or other future 
indebtedness will depend on the capital markets and our financial condition at such time. We may not be able to engage 
in any of these activities or engage in these activities on desirable terms, which could result in a default on our debt 
obligations, including the Term Loan. 

Risks Related to the Development and Commercialization of Our Product Candidates 

We depend heavily on the success of our lead product candidate, odevixibat, which we are developing initially 

for the treatment of patients with PFIC and potentially also for other pediatric cholestatic liver diseases and 
disorders. If we are unable to commercialize odevixibat or experience significant delays in doing so, our business will 
be materially harmed. 

Odevixibat is in Phase 3 clinical development. Elobixibat has been approved in Japan for the treatment of chronic 
constipation, but we will not receive additional cash in respect of elobixibat in Japan as a result of our RIAA. Our other 
most advanced product candidates are in Phase 2 or earlier-stage development. 

Our ability to generate product revenues, which may not occur until at least the end of 2021, if at all, will depend 

heavily on the approval of our NDA by the FDA and our MAA by the EMA and the successful commercialization of 
odevixibat as a treatment for patients with PFIC. Our ability to generate product revenues also depends on the successful 
development and commercialization of odevixibat to treat patients with biliary atresia or ALGS or A3907 or A2342 to 
treat adult liver and viral diseases. The success of each of these product candidates will depend on a number of factors, 
including the following: 

• 

• 

receipt of marketing approval from the FDA and the EMA for odevixibat for the treatment of patients with 
PFIC and marketing approvals for other indications or product candidates from applicable regulatory 
authorities; 

our ability to obtain additional capital, whether from potential future licensing, collaboration or similar 
arrangements or from any future offering of our debt or equity securities; 

48 

• 

• 

• 

• 

• 

• 

• 

• 

• 

• 

our ability to identify and enter into potential future licenses or other collaboration arrangements with third 
parties and the terms of the arrangements; 

completion of clinical development with successful outcomes; 

establishing commercial manufacturing arrangements with third-party manufacturers; 

obtaining and maintaining patent and trade secret protection and regulatory exclusivity; 

protecting our rights in our intellectual property portfolio; 

establishing sales, marketing and distribution capabilities; 

generating commercial sales of odevixibat, A3907 or A2342, as applicable, if and when approved, whether 
alone or in collaboration with others; 

acceptance of odevixibat, A3907 or A2342, as applicable, if and when approved, by patients, the medical 
community and third-party payors; 

effectively competing with other therapies; and 

maintaining an acceptable safety profile of odevixibat, A3907 or A2342, as applicable, following approval. 

If we do not achieve one or more of these factors in a timely manner or at all, we could experience significant 
delays or an inability to successfully commercialize odevixibat, A3907 or A2342, which would materially harm our 
business. 

If clinical trials of odevixibat or any of our other product candidates fail to demonstrate safety and efficacy to 
the satisfaction of the U.S. Food and Drug Administration, or the FDA, or the European Medicines Agency, or the 
EMA, or do not otherwise produce favorable results, we may incur additional costs or experience delays in 
completing, or ultimately be unable to complete, the development and commercialization of the applicable product 
candidate. 

In connection with obtaining marketing approval from regulatory authorities for the sale of any product candidate, 

we must complete preclinical development and then conduct extensive clinical trials to demonstrate the safety and 
efficacy of our product candidates in humans. Clinical trials are expensive, difficult to design and implement, can take 
many years to complete and are uncertain as to outcome. A failure of one or more clinical trials can occur at any stage of 
testing. The outcome of preclinical testing and early clinical trials may not be predictive of the success of later clinical 
trials. In particular, the small number of subjects and patients in our early clinical trials may make the results of these 
clinical trials less predictive of the outcome of later clinical trials. The design of a clinical trial can determine whether its 
results will support approval of a product candidate, and flaws in the design of a clinical trial may not become apparent 
until the clinical trial is well advanced or completed. There is no assurance that we will be able to design and execute a 
clinical trial to support marketing approval. Moreover, preclinical and clinical data are often susceptible to varying 
interpretations and analyses, and many companies that have believed their product candidates performed satisfactorily in 
preclinical studies and clinical trials have nonetheless failed to obtain marketing approval of their product candidates. 

If we are required to conduct additional or longer clinical trials or other testing of odevixibat or any of our other 
product candidates beyond those that we currently contemplate, if we are unable to successfully complete our clinical 
trials or other testing, or if the results of these clinical trials or tests are not positive or are only modestly positive or if 
there are safety concerns, we may: 

• 

be delayed in obtaining marketing approval for our product candidates; 

49 

• 

• 

• 

• 

• 

not obtain marketing approval at all; 

obtain approval for indications or patient populations that are not as broad as we intended or desired; 

obtain approval with labeling that includes significant use or distribution restrictions or safety warnings, 
including boxed warnings; 

be subject to additional post-marketing testing requirements or restrictions; or 

have the product removed from the market after obtaining marketing approval. 

Following consultation with the FDA and EMA, we used change in pruritus as the primary endpoint for 
purposes of FDA evaluation and as a key secondary endpoint for EMA evaluation in our recently completed Phase 3 
trial in PFIC patients. There was no clinical experience with the two outcome instruments that we used in the trial 
and either may not be considered adequately reliable or valid for use in PFIC patients. 

In September 2020, we announced topline results from our Phase 3 trial in PFIC, and in December 2020, we 

announced that we submitted an NDA to the FDA and an MAA to the EMA, with the EMA granting accelerated 
assessment, seeking approval of odevixibat for the treatment of patients with PFIC. The Phase 3 trial in PFIC met its two 
primary endpoints. In January 2021 we announced the FDA accepted our NDA, granting priority review and a user fee 
goal date under PDUFA of July 20, 2021. The primary endpoint for FDA evaluation, and a key secondary endpoint for 
EMA evaluation, was an assessment of change in pruritus. Because the assessment of pruritus relies on subjective 
caregiver feedback, it is challenging to evaluate and measure consistently and, for any patient, a caregiver-reported 
outcome may vary from the patient’s self-reported outcome. The measure of pruritus can be influenced by factors 
outside of our control and can vary widely from measurement point to measurement point for a particular patient, from 
patient to patient and from site to site within a clinical trial. 

We developed caregiver-reported and patient-reported outcome instruments to assess pruritus in the Phase 3 PFIC 

trial. These outcome instruments, and the manner in which they are analyzed, represent novel endpoints and 
measurement methodologies with which the FDA, EMA and other regulatory authorities have no experience. 
Notwithstanding the positive results in the Phase 3 trial, the FDA or EMA may ultimately determine that either of these 
outcome instruments is not adequately reliable or valid for use with PFIC patients, whether because it was not applied by 
clinical investigators sufficiently consistently, was not sufficiently sensitive to detect varying degrees of pruritus, or for 
any other reason. If this were to occur, our ability to obtain marketing approval for odevixibat would be delayed and we 
may never obtain marketing approval for odevixibat. 

Our pending NDA seeking approval to market odevixibat for the treatment of patients with PFIC may be 

delayed as a result of ongoing COVID-19 travel restrictions and safety protocols that could affect FDA’s ability to 
conduct a pre-approval inspection of the drug product’s manufacturing facility, or as a result of operational delays at 
the FDA or the EMA due to a redeployment of staff or other resources as part of the global response to the 
COVID-19 pandemic. 

In response to the COVID-19 pandemic, in March 2020, the FDA announced its intention to temporarily postpone 
most inspections of foreign manufacturing facilities and products, as well as routine surveillance inspections of domestic 
manufacturing facilities, and it also provided guidance regarding the conduct of clinical trials, which has since been 
further updated several times. As of June 23, 2020, the FDA noted it was continuing to ensure timely reviews of 
applications for medical products during the COVID-19 pandemic in line with its user fee performance goals and 
conducting mission-critical domestic and foreign inspections to ensure compliance of manufacturing facilities with FDA 
quality standards. The FDA subsequently publicized its development and use of an internal rating system called the 
COVID-19 Advisory Rating system, to assist in determining when and where it is safest to conduct such inspections 
based on data about the virus’s trajectory in a given country, state and locality and the rules and guidelines that are put in 
place by foreign, state and local governments. As of February 2021, FDA is either continuing to, on a case-by-case basis, 
conduct only “mission-critical” inspections, or, where possible to do so safely, resuming prioritized domestic 

50 

inspections, which generally include pre-approval inspections (PAIs). Foreign PAIs that are not deemed mission-critical 
remain postponed, while those deemed mission-critical will be considered for inspection on a case-by-case basis. FDA 
will use similar data to inform resumption of other prioritized operations abroad as it becomes feasible and advisable to 
do so.  

During the global response to the COVID-19 pandemic, moreover, there have been strategic redeployments of 

government resources to priority projects, including FDA and EMA resources and staff, which could have an impact on 
the timeline for review and approval of new marketing applications. Since the start of the pandemic, the FDA’s new drug 
review programs continued to meet key performance goals related to working with applicants and approving NDAs and 
NDA supplements, although the agency has also stated that the uncertainty of the COVID-19 situation may make it 
difficult to sustain that level of performance indefinitely. The FDA may not be able to maintain its normal pace with 
respect to new drug applications and delays or setbacks are possible in the future. The FDA has told industry that it 
intends to be as transparent as possible about its workload and performance metrics as the situation evolves, and also that 
it intends to communicate proactively with applicants during the review cycle regarding the need for a pre-approval 
inspection and whether such PAI is considered “mission-critical.”  

Should FDA determine that a PAI is necessary for approval of an NDA or NDA supplement and such an 
inspection cannot be completed during the review cycle due to restrictions on travel or other safety protocols, FDA has 
stated that it generally intends to issue a complete response letter. Further, if there is inadequate information to make a 
determination on the acceptability of a facility, FDA may defer action on the application until an inspection can be 
completed. Such decisions will be based on the totality of the information available to the FDA, including considerations 
of whether it can obtain existing inspection reports from trusted foreign regulatory partners through mutual recognition 
and confidentiality agreements and/or secure additional records from the applicant, the manufacturing facility, or other 
inspected entities. Accordingly, FDA has encouraged applicants to effectively communicate with all their facilities and 
sites to ensure timely responses to any inquiries from FDA for information needed to support its assessment of pending 
drug applications. FDA has also stated that it is using all available tools and sources of information to support regulatory 
decisions on NDAs such as the historical compliance status of a manufacturing facility and other risk-benefit 
considerations pertaining to the proposed new drug product and its manufacturing process and facilities. In addition, 
whether or not FDA considers a facility inspection to be “mission-critical” involves several factors related to the public 
health benefits of the proposed new drug product, including but not limited to whether the candidate has been granted 
breakthrough therapy designation and whether the candidate is intended to treat or prevent a serious disease or medical 
condition for which there is no other appropriate substitute. Regulatory authorities outside the United States, including 
but not limited to the EMA, may adopt similar restrictions or other policy measures in response to the COVID-19 
pandemic. We cannot guarantee that our NDA for odevixibat will be approved before or on its user fee goal date, or that 
a necessary PAI inspection of one or both of our contract manufacturers for odevixibat will not have the potential to 
delay FDA approval of our NDA or lead to the issuance of a complete response letter. We are actively monitoring 
developments with respect to these matters, particularly because the contract manufacturers for odevixibat are located 
outside the U.S., and an inspection of one or both facilities will be required before our NDA for odevixibat can be 
approved. 

The clinical trial designs, durations, endpoints and outcomes that will be required to obtain marketing approval 

of odevixibat to treat PFIC patients are uncertain and, in any case, may vary among the FDA, EMA and other 
regulatory authorities outside of the United States and Europe. Based on feedback that we have received from the 
FDA and the EMA, we expect both regulatory authorities to place a greater emphasis on the totality of the data from 
our Phase 3 clinical trial, including secondary endpoints, than may generally be expected. As a result, there is risk 
that, even though the primary endpoints of our Phase 3 clinical trial of odevixibat for FDA evaluation purposes and  
for EMA evaluation purposes were met with statistical significance, the applicable regulatory authority may not find 
the overall results of our Phase 3 trial to be sufficient to support marketing approval of odevixibat to treat PFIC, a 
symptom of PFIC such as pruritus or any other indication, and we may never receive marketing approval.  

No product is currently approved for the treatment of PFIC in the United States, the European Union or, to our 

knowledge, any other jurisdiction, and there is limited clinical experience in PFIC. Accordingly, there is not a well-
established development path that, with positive outcomes in clinical trials, would be reasonably assured of receiving 
marketing approval for these indications. 

51 

Our Phase 3 PFIC program included a single randomized, double blind, placebo controlled, multicenter clinical 

trial and an open label long-term extension study. In September 2020, we announced topline results from PEDFIC 1, our 
Phase 3 clinical trial for odevixibat, given once per day as an oral capsule or sprinkled over food, in patients ages 
6 months to 18 years with PFIC types 1 and 2, which was conducted at 33 global sites. Both doses of odevixibat tested 
were statistically significant for each of the U.S. and E.U. primary endpoints. Odevixibat was well tolerated, with an 
overall adverse event incidence similar to placebo. There were no drug-related serious adverse events, or SAEs, reported 
during the study. In December 2020, we announced that we submitted an NDA to the FDA and an MAA to the EMA, 
seeking approval of odevixibat for the treatment of patients with PFIC. In January 2021 we announced the FDA accepted 
our NDA, with priority review and a user fee goal date under PDUFA of July 20, 2021. 

Based on feedback that we have received from the FDA and the EMA, we expect both regulatory authorities to 
place a greater emphasis on the totality of the data from our Phase 3 clinical trial, including secondary endpoints, than 
may generally be expected. As a result, there is risk that, even though the primary endpoint of our Phase 3 clinical trial 
of odevixibat for FDA evaluation purposes or for EMA evaluation purposes was met with statistical significance, the 
applicable regulatory authority may not find the overall results of our Phase 3 trial to be sufficient to support marketing 
approval of odevixibat to treat PFIC, a symptom of PFIC such as pruritus or any other indication, and we may never 
receive marketing approval. 

Furthermore, the FDA has informed us that showing a clinically meaningful effect only on pruritus could support 

approval for the treatment of pruritus associated with PFIC and has indicated to us that meaningful improvements on one 
or more additional clinical benefit endpoints and/or corroborative real-world clinical evidence would be required to 
support approval for the treatment of PFIC itself. Certain secondary endpoints that we used in our Phase 3 trial may be 
considered clinical benefit endpoints, including progression to surgery and change in growth markers. 

For PEDFIC 1, serum bile acid responder rate was the primary endpoint for purposes of EMA evaluation and a 

key secondary endpoint for FDA evaluation. To support the clinical utility of reduction in serum bile acids in the 
treatment of patients with PFIC, we are supporting an independent study pooling and analyzing long-term PFIC patient 
data from a number of leading PFIC academic centers, which we refer to as the PFIC Study Group. Should the data that 
are being accumulated by the PFIC Study Group come to a different conclusion than we anticipate about the relationship 
of serum bile acids to beneficial clinical outcomes, the EMA or the FDA may determine the results of the Phase 3 PFIC 
study not to be adequate to support approval. 

Also, it is possible that any marketing authorization we may receive from the EMA for odevixibat for the 
treatment of PFIC could be conditional on post-authorization studies and not be considered a full authorization. Our 
ability to obtain and maintain conditional marketing authorization in the European Union will be limited to specific 
circumstances and subject to several conditions and obligations, if obtained at all, including the completion of a clinical 
outcomes trial to confirm the clinical benefit of odevixibat in PFIC. Conditional marketing authorizations based on 
incomplete clinical data may be granted for a limited number of listed medicinal products for human use, including 
products designated as orphan medicinal products under European Union law, if (1) the risk-benefit balance of the 
product is positive, (2) it is likely that the applicant will be in a position to provide the required comprehensive clinical 
trial data, (3) unmet medical needs will be fulfilled and (4) the benefit to public health of the immediate availability on 
the market of the medicinal product outweighs the risk inherent in the fact that additional data are still required. Specific 
obligations, including with respect to the completion of ongoing or new studies, and with respect to the collection of 
pharmacovigilance data, may be specified in the conditional marketing authorization. Conditional marketing 
authorizations are valid for one year, and may be renewed annually, if the risk-benefit balance remains positive, and after 
an assessment of the need for additional or modified conditions. 

The FDA and EMA generally require two pivotal clinical trials to support marketing approval of a drug. In 
December 2020, we announced that we submitted an NDA to the FDA and an MAA to the EMA, seeking approval of 
odevixibat for the treatment of patients with PFIC. In January 2021 we announced the FDA accepted our NDA, with 
priority review and a user fee goal date under PDUFA of July 20, 2021. Our single Phase 3 clinical trial in patients with 
PFIC, together with data from a long-term, open label extension study, forms the primary support for our applications for 
marketing approval of odevixibat in both the United States and European Union for treatment of patients with PFIC. If 
the FDA or EMA requires us to conduct additional clinical trials beyond the ones that we currently contemplate in order 

52 

to support marketing approval of odevixibat to treat patients with PFIC in the United States or European Union, it would 
result in a more expensive and potentially longer development program for odevixibat than we currently contemplate, 
which could delay our ability to generate product revenues with odevixibat, interfere with our ability to enter into any 
potential licensing or collaboration arrangements with respect to this program, cause our value to decline, and limit our 
ability to obtain additional financing that may be necessary to complete additional trials. Even though a single Phase 3 
trial of odevixibat serves as the basis, together with safety data from an extension study to evaluate long-term outcomes, 
for an application for marketing approval for odevixibat in PFIC, either the FDA or the EMA may require that we meet 
the primary endpoint or endpoints in the trial at a higher level of statistical significance than would otherwise be required 
for a trial to be successful, which would reduce the likelihood of a positive trial. 

The design of our Phase 3 clinical trial of odevixibat in patients with PFIC did not conform precisely in all 

respects to the recommendations or preferences expressed by either the FDA or EMA. 

Although the feedback on our Phase 3 clinical trial design in patients with PFIC that we had received from the 
FDA and EMA is generally consistent, it was not identical and the design of our Phase 3 clinical trial of odevixibat in 
patients with PFIC does not conform precisely in all respects to the recommendations or preferences expressed by either 
regulatory authority. As a result, there is increased risk that, even though we view the results of our trial as favorable, the 
FDA or EMA may not find the overall results to be sufficient to support marketing approval of odevixibat to treat PFIC, 
a symptom of PFIC such as pruritus or any other indication. 

Favorable results seen to date in clinical trials of odevixibat, including our Phase 3 trial of odevixibat in 

patients with PFIC, may not be predictive of favorable results in our ongoing BOLD clinical trial of odevixibat in 
biliary atresia or our ongoing ASSERT clinical trial of odevixibat in ALGS. 

A number of companies in the pharmaceutical and biotechnology industries have suffered significant setbacks in 

later-stage clinical trials, even after promising results in earlier trials or in preclinical studies. Similarly, companies have 
experienced disappointing outcomes in later phases of a multiphase clinical trial, even after promising results in an early 
phase of the trial. Odevixibat has been evaluated in our Phase 3 trial in patients with PFIC, an open label Phase 2 trial in 
children with chronic cholestasis and pruritus and in an investigator-initiated Phase 2 clinical trial for the treatment of 
PBC. Our Phase 3 trial in PFIC met its two primary endpoints, demonstrating that odevixibat reduced serum bile acid 
responses (sBAs) (p=0.003) and improved pruritus assessments (p=0.004) with a single digit diarrhea rate. Data from 
our Phase 2 trial in children with chronic cholestasis and pruritus showed a reduction in serum bile acids in a substantial 
majority of patients and improvement in pruritus that was significantly correlated with the reduction in serum bile acids. 
In addition, based on data from the PBC trial that we received from the investigator, nine patients with pruritus received 
odevixibat and all of them reported a reduction in pruritus. If the favorable findings on pruritus and serum bile acids seen 
in our Phase 3 trial and these two Phase 2 trials are not replicated in any other future trial of odevixibat in patients with 
PFIC or other pediatric cholestatic liver disease or disorder, we may not obtain marketing approval for odevixibat to treat 
any indication, in which case our business would be materially and adversely affected. 

Our Phase 3 trial of odevixibat in patients with PFIC involved a greater number of patients, different outcome 

measures, doses and treatment duration and had other differences in trial design, in addition to the difference in patient 
population, compared with either the prior pediatric chronic cholestasis trial or PBC trial. For example, our Phase 3 trial 
of odevixibat was a randomized, double blind, placebo controlled, multicenter, clinical trial designed to enroll 
approximately 60 patients with PFIC (type 1 or 2), with a treatment duration of 24 weeks. The primary endpoint for 
FDA evaluation, and a key secondary endpoint for EMA evaluation, will be an assessment of change in pruritus. The 
primary endpoint for EMA evaluation, and a key secondary endpoint for FDA evaluation, will be serum bile acid 
responder rate, where a responder is a patient who achieves either a reduction in serum bile acid levels of 70% or more 
from baseline or a reduction of serum bile acid levels at least to an absolute level that is specified in between 50 and 
100 µmol/l. Although we have assessed the effects of odevixibat on serum bile acids in prior clinical trials of odevixibat, 
we have not previously utilized this serum bile acid responder rate endpoint. Moreover, we did not have any prior data 
regarding the effect of a placebo in patients with PFIC on serum bile acid levels, pruritus or any other outcome variable 
to guide the planning for our Phase 3 PFIC trial, which increased the risk that the trial would not be powered adequately 
to show a statistically significant separation between odevixibat and placebo. 

53 

If we experience delays or difficulties in the enrollment of patients in our pivotal clinical trials of odevixibat in 

patients with biliary atresia and Alagille syndrome, our receipt of marketing approval for odevixibat in those 
indications could be delayed or prevented. 

Recruiting patients for orphan pediatric liver diseases is challenging. We have previously experienced enrollment 
delays in our clinical trials of odevixibat. If we are unable to locate and enroll a sufficient number of eligible patients to 
participate in clinical trials of our product candidates, we may not be able to initiate or continue the clinical trials. In 
particular, if we experience enrollment delays in our pivotal trials  of odevixibat in patients with biliary atresia and 
Alagille syndrome, our cash resources may not be sufficient to enable us to fund the trial to completion, which could 
cause our value to decline and limit our ability to obtain additional financing. 

Potential clinical trial participants may not be adequately diagnosed or identified with the diseases that we are 
targeting and may not meet the inclusion criteria for our trials.  Biliary atresia, Alagille syndrome and other pediatric 
cholestatic liver diseases or disorders for which we may develop odevixibat  are rare diseases or disorders with a limited 
patient populations, which could result in slow enrollment of clinical trial participants. Further, there are only a limited 
number of specialist physicians that treat these diseases and disorders, and major clinical centers that treat these diseases 
and disorders are concentrated in a few geographic regions. 

Patient enrollment is affected by many factors, including: 

• 

• 

• 

• 

• 

• 

• 

• 

• 

• 

• 

size of the target patient population; 

severity of the disease or disorder under investigation; 

eligibility criteria for the clinical trial in question; 

other clinical trials being conducted at the same time involving patients who have the disease or disorder 
under investigation; 

perceived risks and benefits of the product candidate under study; 

approval and availability of other therapies to treat the disease or disorder that is being investigated in the 
clinical trial; 

willingness or unwillingness to participate in a placebo controlled clinical trial; 

efforts to facilitate timely enrollment in clinical trials; 

patient referral practices of physicians; 

the ability to monitor patients adequately during and after treatment; and 

proximity and availability of clinical trial sites for prospective patients. 

Our inability to enroll a sufficient number of patients in our planned clinical trials of odevixibat, or any of our 
other product candidates, would result in significant delays or may require us to abandon one or more clinical trials 
altogether. 

54 

If the commercial opportunity in PFIC is smaller than we anticipate, or if odevixibat receives approval to treat 
only a specific subpopulation of patients with PFIC or only a specific symptom of PFIC such as pruritus, our future 
revenue from odevixibat may be adversely affected and our business may suffer. 

If the size of the commercial opportunities in any of our target indications is smaller than we anticipate, we may 
not be able to achieve profitability and growth. We are developing odevixibat initially as a treatment for patients with 
PFIC and potentially also as a treatment for patients with other pediatric cholestatic liver diseases and disorders, such as 
biliary atresia and ALGS. PFIC and these other diseases and disorders are each rare, with a limited patient population. 
Moreover, we expect that the addressable PFIC patient population for odevixibat is only a subset of the overall patient 
population, specifically patients who have not yet received partial external biliary diversion, or PEBD, surgery or liver 
transplant surgery or patients who have had PEBD reversal surgery. We are not aware of any available patient registries 
for PFIC, and we rely on various estimates and assumptions to estimate the addressable PFIC population. In addition, 
there are different subtypes of PFIC and the beneficial effects of odevixibat may vary among patients with different 
subtypes or among children of different ages. Our Phase 3 PEDFIC 1 clinical trial enrolled patients with PFIC, 
type 1 or 2, but not PFIC, type 3. Based on FDA feedback, odevixibat may ultimately receive regulatory approval, if at 
all, as a treatment for only the PFIC subtypes studied. It is also possible that odevixibat may ultimately receive 
regulatory approval, if at all, as a treatment for children with PFIC of some ages but not others. Moreover, we are using 
change in pruritus as the primary endpoint of our Phase 3 trial for purposes of FDA evaluation. The FDA has informed 
us that showing a clinically meaningful effect only on pruritus could support approval for the treatment of pruritus 
associated with PFIC and has indicated to us that meaningful improvements on one or more additional clinical benefit 
endpoints and/or corroborative real-world clinical evidence would be required to support approval for the treatment of 
PFIC itself. If the commercial opportunity in PFIC is smaller than we anticipate, whether because our estimates of the 
addressable patient population prove to be incorrect, because odevixibat receives marketing approval, if at all, as a 
treatment for some but not all PFIC subtypes, for children of some ages but not others, for pruritus associated with PFIC 
but not PFIC itself or for any other reason, our future revenue from odevixibat may be adversely affected and our 
business may suffer. 

It is critical to our ability to grow and become profitable that we successfully identify patients with PFIC and any 

other rare cholestatic liver diseases and disorders that we may target in the future. Our projections of the number of 
people who have PFIC or our other potential target cholestatic liver diseases and disorders, as well as the subset who 
have the potential to benefit from treatment with odevixibat, are based on a variety of sources, including third-party 
estimates and analyses in the scientific literature, and may prove to be incorrect. Further, new information may emerge 
that changes our estimate of the prevalence of these diseases or the number of patient candidates for odevixibat. The 
effort to identify patients with PFIC or our other potential target indications is ongoing, and we cannot accurately predict 
the number of patients for whom treatment might be possible. Additionally, the addressable patient population for 
odevixibat may be limited or may not be amenable to treatment with odevixibat, and new patients may become 
increasingly difficult to identify or gain access to, which would adversely affect our results of operations and our 
business. Further, even if we obtain significant market share for odevixibat, we may never achieve profitability because 
the potential target patient population for odevixibat is small. 

If we experience any of a number of possible unforeseen events in connection with our clinical trials, potential 
marketing approval or commercialization of our product candidates, or entry into licensing, collaboration or similar 
arrangements, could be delayed or prevented. 

We may experience numerous unforeseen events during, or as a result of, clinical trials that could delay or prevent 

our ability to receive marketing approval or commercialize our product candidates, including: 

• 

• 

clinical trials of our product candidates may produce negative or inconclusive results, and we may decide, or 
regulators may require us, to conduct additional clinical trials or abandon product development programs; 

the number of patients required for clinical trials of our product candidates may be larger than we anticipate, 
enrollment in these clinical trials may be slower than we anticipate or participants may drop out of these 
clinical trials at a higher rate than we anticipate; 

55 

• 

• 

• 

• 

• 

• 

• 

• 

• 

we may be unable to recruit and enroll a sufficient number of patients in our clinical trials to ensure adequate 
statistical power to detect any statistically significant treatment effects; 

our third-party contractors may fail to comply with regulatory requirements or meet their contractual 
obligations to us in a timely manner, or at all; 

regulators, institutional review boards or independent ethics committees may not authorize us or our 
investigators to commence a clinical trial or conduct a clinical trial at a prospective trial site; 

we may experience delays in reaching, or we may fail to reach, agreement on acceptable clinical trial 
contracts or clinical trial protocols with prospective trial sites; 

we may have to suspend or terminate clinical trials of our product candidates for various reasons, including a 
finding that the participants are being exposed to unacceptable health risks or undesirable side effects; 

regulators, institutional review boards or independent ethics committees may require that we or our 
investigators suspend or terminate clinical research for various reasons, including noncompliance with 
regulatory requirements or a finding that the participants are being exposed to unacceptable health risks; 

the cost of clinical trials of our product candidates may be greater than we anticipate; 

the supply or quality of our product candidates or other materials necessary to conduct clinical trials of our 
product candidates may be insufficient or inadequate; and 

our product candidates may have undesirable side effects or other unexpected characteristics, causing us or 
our investigators, regulators, institutional review boards or independent ethics committees to suspend or 
terminate the clinical trials. 

For example, in March 2015, Ferring International Center S.A., or Ferring, stopped early two Phase 3 clinical trials 

of elobixibat that Ferring had been conducting pursuant to a now-terminated license agreement with us due to an issue 
related to the distribution of study drug to study sites that was unrelated to the performance of elobixibat. We were 
unable as a result of the stopping of the trials to obtain data for the total number of patients for which the trials were 
designed, and the abbreviated trials are not sufficient to support an application for marketing approval. 

Our product development costs will increase if we experience delays in testing or marketing approvals. We do not 

know whether any preclinical studies or clinical trials will begin as planned, will need to be restructured or will be 
completed on schedule, or at all. Significant preclinical study or clinical trial delays also could shorten any periods 
during which we may have the exclusive right to commercialize our product candidates or allow our competitors to bring 
products to market before we do and impair our ability to successfully commercialize our product candidates, which may 
harm our business and results of operations. 

The benefit of IBAT inhibition in the treatment of patients with PFIC or any of our other target indications is 

unproven, and we do not know whether we will be able to develop any products of commercial value for these 
indications. 

Odevixibat is an ileal bile acid transporter, or IBAT, inhibitor. There is no marketed drug in the United States or 

Europe that relies on IBAT inhibition for the treatment of PFIC or any other indication for which we plan to develop 
odevixibat. Shire plc, or Shire, reported that, SHP625, now known as maralixibat, which it licensed to and is now being 
developed by Mirum Pharmaceuticals, and has been reported to be an IBAT inhibitor, failed to meet the respective 
primary endpoints of Phase 2 clinical trials in multiple adult and pediatric indications. We cannot assure you that we will 
be able to replicate or improve upon our findings from preclinical studies and early clinical trials in later-stage clinical 
trials of odevixibat for any of our other target indications or that our focus on IBAT inhibition as a medically useful 

56 

mechanism of action will result in the development of a commercially viable drug that safely and effectively treats PFIC 
or any of our other target indications.  

If serious or unacceptable side effects are identified during the development of odevixibat, A3907, A2342 or any 

other product candidate, we may need to abandon or limit our development of that product candidate. 

All of our product candidates are in clinical or preclinical development (other than elobixibat for chronic 
constipation, which has been approved in Japan) and their risk of failure is high. It is impossible to predict when or if 
any of our product candidates will prove effective or safe in humans or will receive marketing approval. If our product 
candidates are associated with undesirable side effects or have other unexpected, unacceptable characteristics, we may 
need to abandon their development or limit development to certain uses or subpopulations in which the undesirable side 
effects or other characteristics are less prevalent, less severe or more acceptable from a risk-benefit perspective. Many 
investigational products that initially showed promise in clinical or earlier stage testing have later been found to cause 
side effects or other safety issues that prevented further development. 

For example, the investigator for the investigator-initiated Phase 2 clinical trial of odevixibat in PBC determined to 

conclude the trial prior to its intended completion, citing gastrointestinal side effects. If the GI side effects cited by the 
investigator in the PBC trial are predictive of an inadequate tolerability profile of odevixibat, the overall commercial 
opportunity for odevixibat may be lower than we expect. Even if we receive regulatory approval for odevixibat, if the 
approved dose of odevixibat is not well tolerated, odevixibat may not achieve market acceptance by physicians, patients, 
third-party payors or others in the medical community, which would materially and adversely affect our business. 

Even if odevixibat, A3907, A2342 or any potential future product candidate of ours receives marketing 

approval, it may fail to achieve the degree of market acceptance by physicians, patients, third-party payors and others 
in the medical community necessary for commercial success. 

If odevixibat, A3907, A2342 or any potential future product candidate of ours receives marketing approval, the 
approved product may nonetheless fail to gain sufficient market acceptance by physicians, patients, third-party payors 
and others in the medical community. If an approved product does not achieve an adequate level of acceptance, we may 
not generate significant product revenues or any profits from operations. The degree of market acceptance of our product 
candidates, if approved for commercial sale, will depend on a number of factors, including: 

• 

• 

• 

• 

• 

• 

• 

• 

• 

the efficacy and potential advantages compared to alternative treatments or competitive products; 

the prevalence and severity of any side effects; 

whether physicians will be willing to prescribe odevixibat to patients with PFIC notwithstanding that the 
primary endpoint in our Phase 3 clinical trial of odevixibat in patients with PFIC is change in pruritus (FDA) 
or serum bile acid responder rate (EMA), as opposed to a direct measure of reducing or eliminating 
progressive liver disease; 

the ability to offer our product candidates for sale at competitive prices; 

convenience and ease of administration compared to alternative treatments; 

the willingness of the target patient population to try and adhere to new therapies and of physicians to 
prescribe these therapies; 

the strength of marketing and distribution support; 

the adequacy of supply of our product candidates; 

the availability of third-party coverage and adequate reimbursement; 

57 

• 

• 

• 

the timing of any such marketing approval in relation to other product approvals; 

support from patient advocacy groups; and 

any restrictions on concomitant use of other medications. 

Our ability to negotiate, secure and maintain third-party coverage and reimbursement for our product candidates 

may be affected by political, economic and regulatory developments in the United States, the European Union and other 
jurisdictions. Governments continue to impose cost containment measures, and third-party payors are increasingly 
challenging prices charged for medicines and examining their cost effectiveness, in addition to their safety and efficacy. 
These and other similar developments could significantly limit the degree of market acceptance of odevixibat, A3907, 
A2342 or any potential future product candidate of ours that receives marketing approval. 

If we are unable to establish sales and marketing capabilities or enter into agreements with third parties to 

market and sell odevixibat or any of our other current or potential future product candidates, we may not be 
successful in commercializing the applicable product candidate if it receives marketing approval. 

We have limited experience as a company in the sale or marketing of pharmaceutical products. To achieve 
commercial success for any approved product, we must either develop a sales and marketing organization or outsource 
these functions to third parties. We are building the capabilities to commercialize odevixibat in the United States and 
Europe in anticipation of marketing approval for odevixibat to treat PFIC in those jurisdictions. Outside of the United 
States and Europe, we plan to selectively utilize collaboration, distribution or other marketing arrangements with third 
parties to commercialize odevixibat. Also, we intend to selectively seek licensing, collaboration or similar arrangements 
to assist us in furthering the development or commercialization of product candidates, such as A3907 or A2342, 
targeting large primary care markets that must be served by large sales and marketing organizations. There are risks 
involved with establishing our own sales and marketing capabilities and entering into arrangements with third parties to 
perform these services. For example, recruiting and training a sales force is expensive and time consuming and could 
delay any product launch. If the commercial launch of a PFIC or any other product candidate for which we recruit a sales 
force and establish marketing capabilities is delayed or does not occur for any reason, we would have prematurely or 
unnecessarily incurred these commercialization expenses. This may be costly, and our investment would be lost if we 
cannot retain or reposition our sales and marketing personnel. 

Factors that may inhibit our efforts to commercialize our products on our own include: 

• 

• 

• 

• 

our inability to recruit, train and retain adequate numbers of effective sales and marketing personnel; 

the inability of sales personnel to obtain access to or persuade adequate numbers of physicians to prescribe 
any future products; 

the lack of complementary products to be offered by sales personnel, which may put us at a competitive 
disadvantage relative to companies with more extensive product lines; and 

unforeseen costs and expenses associated with creating an independent sales and marketing organization. 

If we enter into arrangements with third parties to perform sales and marketing services, our product revenues or 
the profitability of these product revenues are likely to be lower than if we were to market and sell any products that we 
develop ourselves. In addition, we may not be successful in entering into arrangements with third parties to sell and 
market our product candidates or may be unable to do so on terms that are acceptable to us. We likely will have little 
control over such third parties, and any of them may fail to devote the necessary resources and attention to sell and 
market our products effectively. If we do not establish sales and marketing capabilities successfully, either on our own or 
in collaboration with third parties, we will not be successful in commercializing our product candidates. 

58 

We face substantial competition, which may result in others discovering, developing or commercializing 

products to treat our target indications or markets before or more successfully than we do. 

The development and commercialization of new drug products is highly competitive. We face competition with 

respect to our current product candidates and any products we may seek to develop or commercialize in the future from 
major pharmaceutical companies, specialty pharmaceutical companies and biotechnology companies worldwide. 

Competitors may also include academic institutions, government agencies and other public and private research 

organizations that conduct research, seek patent protection and establish collaborative arrangements for research, 
development, manufacturing and commercialization. Many of our competitors have significantly greater financial 
resources and expertise in research and development, manufacturing, preclinical testing, conducting clinical trials, 
obtaining approvals from regulatory authorities and marketing approved products than we do. Mergers and acquisitions 
in the pharmaceutical and biotechnology industries may result in even more resources being concentrated among a 
smaller number of our competitors. Smaller and other early-stage companies may also prove to be significant 
competitors, particularly through collaborative arrangements with large and established companies. These third parties 
compete with us in recruiting and retaining qualified scientific and management personnel, establishing clinical trial sites 
and patient registration for clinical trials, as well as in acquiring technologies that may be complementary to or necessary 
for our programs. 

Our commercial opportunities could be reduced or eliminated if our competitors develop and commercialize 

products that are more effective, safer, have fewer or less severe side effects, are approved for broader indications or 
patient populations, or are more convenient or less expensive than any products that we develop and commercialize. Our 
competitors may also obtain marketing approval for their products more rapidly than we may obtain approval for our 
products, which could result in our competitors establishing a strong market position before we are able to enter the 
market. 

In particular, we are aware of other companies that are developing product candidates that, like our product 
candidates odevixibat and elobixibat, act via IBAT inhibition. As noted above, Mirum is conducting a Phase 3 clinical 
trial of maralixibat in PFIC, for which it has reported that it expects to complete enrollment in the second quarter of 2021 
and announce topline data by year-end 2021. As of February 2021, Mirum Pharmaceuticals announced the completion of 
a rolling NDA submission for maralixibat in ALGS, and is planning for a potential launch in ALGS in the second half of 
2021. We understand that Mirum’s MAA for maralixibat for the treatment of patients with PFIC type 2 has been 
accepted for review by the EMA. If approved, our product candidates will compete for a share of the existing market 
with numerous other products. We believe that the primary competitive products for use in indications that we are 
currently targeting with our most advanced product candidates include the following. 

• 

• 

For PFIC and many other cholestatic liver diseases, there are currently no approved drug treatments. First-
line treatment for PFIC is typically off-label ursodeoxycholic acid, or UDCA, which is approved in France 
only for PFIC type 3, and in the United States and elsewhere for the treatment of PBC. PFIC patients often 
require surgical intervention such as PEBD surgery or liver transplant. As noted above, Mirum has initiated 
a Phase 3 clinical trial with maralixibat in PFIC, and plans to seek FDA approval for maralixibat as a 
treatment for pruritus associated with ALGS. We understand that Mirum’s MAA for maralixibat for the 
treatment of patients with PFIC type 2 has been accepted for review by the EMA. In addition, Intercept 
Pharmaceuticals’ obeticholic acid, which is approved in the United States in combination with UDCA, or as 
a monotherapy for patients unable to tolerate UDCA, to treat PBC, is in Phase 2 development as a treatment 
for biliary atresia. GlaxoSmithKline’s linerixibat is at the Phase 2 development stage as a treatment for 
pruritus associated with ALGS. In July 2019, Genfit SA received orphan designations from the FDA and 
EMA elafibranor for the treatment of PBC. Genfit has announced plans to advance elafibranor into a Phase 3 
program for PBC in 2020. Gilead Sciences, Inc.’s FXR agonist, cilofexor, has completed Phase 2 
development for PBC, and is in Phase 3 development for PSC. 

For the pruritus that is characteristic of many cholestatic liver diseases, symptomatic off-label treatment 
with: UDCA; bile acid sequestrants, such as generic cholestyramine (marketed as, Efensol, Ipocol, 

59 

Kolestran, Lipocol, Olestyr, Prevalite or Quantalan in various countries), marketed by Upsher-Smith 
Laboratories, Inc., Par Pharmaceutical Companies, Inc. and Sandoz, the generic pharmaceuticals division of 
Novartis AG; rifampin, an antibiotic derivative; or naltrexone, an opioid antagonist. 

For chronic constipation: linaclotide, a guanylate cyclase-C agonist, is marketed by Ironwood 
Pharmaceuticals, Inc. and Allergan as Linzess in the United States and as Constella (for the treatment of a 
related condition, irritable bowel syndrome with constipation, or IBS-C) in Europe. Linaclotide is also 
marketed by Astellas Pharma Inc. as Linzess in Japan for the treatment of IBS-C; lubiprostone, a type-2 
chloride channel marketed as Amitiza by Takeda Pharmaceutical Company Limited in the United States and 
select countries in Europe and by Mylan N.V. in Japan; prucalopride, a motility agent marketed by Shire in 
the United States as Motegrity and in the European Union as Resolor; and numerous over the counter 
products, including psyllium husk (such as Metamucil), methylcellulose (such as Citrucel), calcium 
polycarbophil (such as FiberCon), lactulose (such as Cephulac), polyethylene glycol (such as MiraLax), 
sennosides (such as Exlax), bisacodyl (such as Ducolax), docusate sodium (such as Colace), magnesium 
hydroxide (such as Milk of Magnesia), saline enemas (such as Fleet) and sorbitol. 

Plecanatide, marketed as Trulance by Bausch Health Companies, Inc. is approved in the United States to 
treat CIC and IBS-C. Ardelyx, Inc.’s product, IBSRELA (tenapanor) was approved by the FDA for the 
treatment of IBS-C in September 2019.  

• 

• 

Even if we are able to commercialize odevixibat, A3907, A2342 or any other product candidate that we develop, 

the product may become subject to unfavorable pricing regulations, third-party reimbursement practices or 
healthcare reform initiatives, which would harm our business. 

The regulations that govern marketing approvals, pricing, coverage and reimbursement for new drug products vary 

widely from country to country. Current and future legislation may significantly change the approval requirements in 
ways that could involve additional costs and cause delays in obtaining approvals. Some countries require approval of the 
sale price of a drug before it can be marketed. In many countries, the pricing review period begins after marketing or 
product licensing approval is granted and, in some markets, prescription pharmaceutical pricing remains subject to 
continuing governmental control even after initial approval is granted. As a result, we might obtain marketing approval 
for a product in a particular country, but then be subject to price regulations that delay our commercial launch of the 
product, possibly for lengthy time periods, and negatively impact the revenues we are able to generate from the sale of 
the product in that country. Adverse pricing limitations may hinder our ability to recoup our investment in one or more 
product candidates, even if our product candidates obtain marketing approval. 

Our ability to commercialize odevixibat, A3907, A2342 or any other product candidate successfully also will 

depend in part on the extent to which coverage and adequate reimbursement for these products and related treatments 
will be available from government health administration authorities, private health insurers and other organizations. 
Government authorities and other third-party payors, such as private health insurers and health maintenance 
organizations, decide which medications they will pay for and establish reimbursement levels. A primary trend in the 
U.S. and E.U. healthcare industries and elsewhere is cost containment. Government authorities and other third-party 
payors have attempted to control costs by limiting coverage and the amount of reimbursement for particular medications. 
Increasingly, third-party payors are requiring that drug companies provide them with predetermined discounts from list 
prices and are challenging the prices charged for medical products. We cannot be sure that coverage and reimbursement 
will be available for odevixibat, A3907, A2342 or any other product that we commercialize and, if coverage and 
reimbursement is available, the level of reimbursement. Reimbursement may impact the demand for, or the price of, any 
product candidate for which we obtain marketing approval. Obtaining and maintaining adequate reimbursement for 
odevixibat may be particularly difficult because of the higher prices typically associated with drugs directed at smaller 
populations of patients. In addition, third-party payors are likely to impose strict requirements for reimbursement of a 
higher priced drug, and any launch of a competitive product is likely to create downward pressure on the price initially 
charged. If reimbursement is not available or is available only to a limited degree, we may not be able to successfully 
commercialize any product candidate for which we obtain marketing approval. 

60 

There may be significant delays in obtaining coverage and reimbursement for newly approved drugs, and coverage 
may be more limited than the purposes for which the drug is approved by the applicable regulatory authority. Moreover, 
eligibility for coverage and reimbursement does not imply that any drug will be paid for in all cases or at a rate that 
covers our costs, including research, development, intellectual property, manufacturing, sale and distribution expenses. 
Interim reimbursement levels for new drugs, if applicable, may also not be sufficient to cover our costs and may not be 
made permanent. Reimbursement rates may vary according to the use of the drug and the clinical setting in which it is 
used, may be based on reimbursement levels already set for lower cost drugs, and may be incorporated into existing 
payments for other services. Net prices for drugs may be reduced by mandatory discounts or rebates required by 
government healthcare programs or private payors and by any future relaxation of laws that presently restrict imports of 
drugs from countries where they may be sold at lower prices than in the United States. In the United States, third-party 
payors often rely upon Medicare coverage policy and payment limitations in setting their own reimbursement policies. In 
the European Union, reference pricing systems and other measures may lead to cost containment and reduced prices. Our 
inability to promptly obtain coverage and adequate reimbursement rates from both government-funded and private 
payors for any approved products that we develop could have a material adverse effect on our operating results, our 
ability to raise capital needed to commercialize products and our overall financial condition. 

Governments outside the United States tend to impose strict price controls, which may adversely affect our 

revenues, if any. 

In some countries, particularly the member states of the European Union, the pricing of prescription 
pharmaceuticals is subject to governmental control. In these countries, pricing negotiations with governmental 
authorities can take considerable time after the receipt of marketing approval for a product. In addition, there can be 
considerable pressure by governments and other stakeholders on prices and reimbursement levels, including as part of 
cost containment measures. Political, economic and regulatory developments may further complicate pricing 
negotiations, and pricing negotiations may continue after reimbursement has been obtained. Reference pricing used by 
various E.U. member states and parallel distribution, or arbitrage between low-priced and high-priced member states, 
can further reduce prices. In some countries, we may be required to conduct a clinical trial or other studies that compare 
the cost-effectiveness of our product candidate to other available therapies in order to obtain or maintain reimbursement 
or pricing approval. Publication of discounts by third-party payors or authorities may lead to further pressure on prices or 
reimbursement levels within the country of publication and other countries. If reimbursement of our products is 
unavailable or limited in scope or amount, or if pricing is set at unsatisfactory levels, our business could be adversely 
affected. 

Product liability lawsuits against us could cause us to incur substantial liabilities and to limit 

commercialization of any products that we may develop. 

We face an inherent risk of product liability exposure related to the testing of our product candidates in human 
clinical trials and will face an even greater risk with respect to commercial sales of any products that we may develop. If 
we cannot successfully defend ourselves against claims that our product candidates or products caused injuries, we will 
incur substantial liabilities. Regardless of merit or eventual outcome, liability claims may result in: 

• 

• 

• 

• 

• 

• 

reduced resources of our management to pursue our business strategy; 

decreased demand for any products that we may develop; 

injury to our reputation and significant negative media attention; 

withdrawal of clinical trial participants or sites; 

significant costs to defend the related litigation; 

substantial monetary awards to clinical trial participants or patients; 

61 

• 

• 

• 

loss of revenue; 

increased insurance costs; and 

the inability to commercialize any products that we may develop. 

We have separate liability insurance policies that cover each of our clinical trials, as well as a global 

product/clinical trial policy. These policies provide coverage in varying amounts, with the global policy having a per 
occurrence and aggregate limit of $10 million. The amount of insurance that we currently hold may not be adequate to 
cover all liabilities that we may incur. We will need to increase our insurance coverage when and if we begin conducting 
more expansive clinical development of, or commercializing, odevixibat, A3907, A2342 or any potential future product 
candidate of ours. Insurance coverage is increasingly expensive. We may not be able to maintain insurance coverage at a 
reasonable cost or in an amount adequate to satisfy any liability that may arise. 

We may expend our limited resources to pursue a particular product candidate and fail to capitalize on product 

candidates that may be more profitable or for which there is a greater likelihood of success. 

Because we have limited financial and managerial resources, we focus on specific product candidates. Currently, 

we are focusing our resources predominantly on odevixibat. As a result, we may forego or delay pursuit of opportunities 
with potential future product candidates that later could prove to have greater commercial potential. Our resource 
allocation decisions may cause us to fail to capitalize on viable commercial products or profitable market opportunities. 
Our spending on current and future research and development programs and product candidates may not yield any 
commercially viable products. 

We have historically based our research and development efforts on IBAT inhibitors, including odevixibat and 

elobixibat, to treat cholestatic liver diseases and CIC and on our proprietary formulations of, A3907 and A2342, as  
potential treatments for adult liver or viral diseases. Notwithstanding our investment to date and anticipated future 
investment, we have not yet developed, and may never successfully develop, any marketed drugs using these 
approaches. As a result of pursuing the development of product candidates using our proprietary technologies, we may 
fail to develop product candidates or address indications based on other scientific approaches that may offer greater 
commercial potential or for which there is a greater likelihood of success. Research programs to identify new product 
candidates require substantial technical, financial and human resources. These programs may initially show promise in 
identifying potential product candidates, yet fail to yield product candidates for clinical development. 

If we do not accurately evaluate the commercial potential or target market for a particular product candidate, we 

may relinquish valuable rights to that product candidate through licensing, collaboration or other royalty or similar 
arrangements in cases in which it would have been more advantageous for us to retain sole development and 
commercialization rights to such product candidate. 

If we do not accurately evaluate the commercial potential or target market for a particular product candidate, we 

may relinquish valuable rights to that product candidate through licensing, collaboration or other royalty or similar 
arrangements in cases in which it would have been more advantageous for us to retain sole development and 
commercialization rights to such product candidate. 

Risks Related to Our Dependence on Third Parties 

We rely on EA Pharma for the successful commercialization of elobixibat to treat chronic constipation in 
Japan and for the successful development and commercialization of elobixibat to treat chronic constipation in other 
select markets in Asia. If EA Pharma does not successfully commercialize elobixibat in Japan, we may not receive 
any future payments under our license agreement with EA Pharma. 

We entered into a license agreement with EA Pharma (formerly known as Ajinomoto Pharmaceuticals) for 
elobixibat in April 2012. In January 2018, the Japanese Ministry of Health, Labour and Welfare, or MHLW, approved a 

62 

new drug application filed by EA Pharma for elobixibat for the treatment of chronic constipation. EA Pharma co-
markets elobixibat in Japan with another company, Mochida Pharmaceutical Co., Ltd, or Mochida, and co-promotes 
elobixibat in Japan with Eisai Co., Ltd. 

In December 2017, the Company entered into a royalty interest acquisition agreement (RIAA) with HealthCare 
Royalty Partners III, L.P. (HCR) pursuant to which it sold to HCR the right to receive all royalties from sales in Japan 
and sales milestones achieved from any covered territory potentially payable to the Company under the Agreement, up 
to a specified maximum “cap” amount of $78.8 million, based on the funds the Company received from HCR to date. 
The Company received $44.5 million from HCR, net of certain transaction expenses, under the RIAA. On June 8, 2020, 
we entered into an amendment to the RIAA with HCR pursuant to which HCR agreed to pay us an additional $14.8 
million, net of certain transactions expenses, in exchange for the elimination of the (i) $78.8 million cap amount on 
HCR’s rights to receive royalties on sales in Japan and sales milestones for elobixibat in certain other territories that may 
become payable by EA Pharma and (ii) $15.0 million payable to us if a specified sales milestone is achieved for 
elobixibat in Japan. The Company is obligated to make royalty interest payments to HCR under the RIAA only to the 
extent it receives future Japanese royalties, sales milestones or other specified payments from EA Pharma. Although the 
Company sold its rights to receive royalties from the sales of elobixibat in Japan, as a result of its ongoing involvement 
in the cash flows related to these royalties, the Company will continue to account for these royalties as revenue. Upon 
receipt of the payments from HCR the Company recorded the $59.3 million as a liability related to sale of future 
royalties (royalty obligation). The royalty obligation will be accreted using the effective interest rate method. 

The Company records estimated royalties due for the current period in accrued other expenses until the payment is 
received from EA Pharma at which time the Company then remits payment to HCR. In order to determine the accretion 
of the royalty obligation, the Company is required to estimate the total amount of future royalty payments to be received 
and submitted to HCR. The sum of these amounts less the $59.3 million proceeds the Company received will be 
recorded as interest expense over the life of the royalty obligation. At December 31, 2020, the Company’s estimate of its 
total interest expense resulted in an annual effective interest rate of approximately 19.0%.  

The Company periodically assesses the estimated royalty payments to HCR and to the extent such payments are 
greater or less than its initial estimates or the timing of such payments is materially different than its original estimates, 
the Company will prospectively adjust the accretion of interest on the royalty obligation. There are a number of factors 
that could materially affect the amount and the timing of royalty payments, most of which are not within the Company’s 
control. Such factors include, but are not limited to, the rate of elobixibat prescriptions, the number of doses 
administered, the introduction of competing products, manufacturing or other delays, patent protection, adverse events 
that result in governmental health authority imposed restrictions on the use of the drug products, significant changes in 
foreign exchange rates as the royalties remitted to HCR are in U.S. dollars while sales of elobixibat are in Japanese yen, 
and sales never achieving forecasted numbers, which would result in reduced royalty payments and reduced non-cash 
interest expense over the life of the royalty obligation. To the extent future royalties result in an amount less than the 
liability, the Company is not obligated to fund any such shortfall. 

EA Pharma is responsible for all commercialization of elobixibat in its licensed field (namely, all prophylactic or 
therapeutic uses of a pharmaceutical product for gastrointestinal diseases and disorders, symptoms of constipation of all 
causes or postoperative ileus, in colonoscopy cleansing procedures and, in specified circumstances, select liver diseases) 
in Japan and for all future development and commercialization of elobixibat in its licensed field, and has substantial 
control over the conduct and timing of development efforts with respect to elobixibat in these countries. We have little 
control over the amount and timing of resources that EA Pharma devotes, or Mochida devotes, to the commercialization 
of elobixibat in Japan or to the development of elobixibat in these other countries. If EA Pharma or, where applicable, 
Mochida fails to devote sufficient financial and other resources, the commercialization of elobixibat in Japan and the 
development and potential commercialization of elobixibat otherwise in EA Pharma’s licensed territory would be 
adversely affected. If this occurs, royalties that we could receive on any future elobixibat product sales could be delayed 
or reduced. 

EA Pharma has the right to terminate the elobixibat agreement on a country-by-country basis or in its entirety for 

an uncured material breach by us or in specified bankruptcy or similar events. EA Pharma also has the right, with 

63 

180 days’ notice, to terminate the agreement in its entirety or on a country-by-country basis (except for Japan) for any 
reason. 

If EA Pharma terminates the elobixibat license agreement at any time, for any reason, it would negatively impact 

both the likelihood that we would receive any future payments under our license agreement with EA Pharma and the 
development of elobixibat in EA Pharma’s licensed territory outside of Japan, would materially harm our business and 
could accelerate our need for additional capital. In particular, we would be required to seek a replacement licensee for 
Japan under the RIAA. 

If we do not pursue the development and potential commercialization of elobixibat for the treatment of CIC in 

the United States or Europe, whether through a licensing, collaboration or similar arrangement or otherwise, the 
revenue that we will generate based on elobixibat may be lower. 

We have commercial rights to elobixibat in the United States, Europe and otherwise outside of the territories 

licensed to EA Pharma. We do not have any current plan to seek a license or other partnering transaction with a third 
party for elobixibat for CIC in the United States or Europe. The cost and duration of the additional clinical trial or trials 
that would be required by the FDA and EMA to support marketing approval of elobixibat to treat CIC is currently 
uncertain. Even if we were to seek to establish licensing, collaboration or similar arrangement with a third party for the 
United States or Europe, the uncertain regulatory requirements may interfere with our ability to do so on acceptable 
terms, or at all. We do not anticipate that we will conduct future clinical trials of elobixibat in CIC for the United States 
or Europe independently, whether or not we elect to seek a suitable third-party arrangement. If we do not enter into 
suitable third-party arrangements and do not ourselves conduct clinical trials of elobixibat in CIC for the United States or 
Europe, the revenue that we will generate based on elobixibat in CIC will be limited to future payments that we receive, 
if any, under our agreements with EA Pharma, which will reduce the overall commercial potential of elobixibat and may 
harm our business. 

We rely on third parties to conduct our clinical trials and those third parties may not perform satisfactorily, 

including failing to meet deadlines for the completion of such clinical trials. 

We do not independently conduct clinical trials for our product candidates. We rely on third parties, such as 
contract research organizations, clinical data management organizations, medical institutions, clinical investigators and 
government agencies, to perform this function. Any of these third parties may terminate their engagements with us at 
any time. If we need to enter into alternative arrangements, it would delay our product development activities. 

Our reliance on these third parties for clinical development activities reduces our control over these activities but 

does not relieve us of our responsibilities. For example, we remain responsible for ensuring that each of our clinical trials 
is conducted in accordance with the general investigational plan and protocols for the clinical trial. Moreover, the FDA 
and foreign regulatory authorities require us to comply with standards, commonly referred to as Good Clinical Practice, 
or GCP, for conducting, recording and reporting the results of clinical trials to assure that data and reported results are 
credible and accurate and that the rights, integrity of data and confidentiality of clinical trial participants are protected. 
We are also required to register clinical trials subject to FDA regulation and, with some exceptions, post the results of 
completed clinical trials on a government-sponsored database, www.ClinicalTrials.gov, within certain timeframes. 
Failure to do so can result in fines, adverse publicity and civil and criminal sanctions. The National Institutes of Health 
also has announced plans to require sponsors to post results of clinical trials for unapproved products, including 
unfavorable results in clinical trials for unapproved uses of approved products. 

Furthermore, third parties that we rely on for our clinical development activities may also have relationships with 

other entities, some of which may be our competitors. If these third parties do not successfully carry out their contractual 
duties, meet expected deadlines or conduct our clinical trials in accordance with regulatory requirements or our stated 
protocols, we will not be able to obtain, or may be delayed in obtaining, marketing approvals for our product candidates 
and will not be able to, or may be delayed in our efforts to, successfully commercialize our product candidates. Our 
product development costs will increase if we experience delays in testing or obtaining marketing approvals. 

64 

We also rely on other third parties to store and distribute drug supplies for our clinical trials. Any performance 

failure on the part of our distributors could delay clinical development or marketing approval of our product candidates 
or commercialization of our products, producing additional losses and depriving us of potential product revenue. 

Use of third parties to manufacture our product candidates may increase the risk that we will not have 

sufficient quantities of our product candidates or products or such quantities at an acceptable cost, which could 
delay, prevent or impair our development or commercialization efforts. 

We do not own or operate manufacturing facilities for the production of clinical or commercial supplies of our 
product candidates. We have limited personnel with experience in drug manufacturing and lack the resources and the 
capabilities to manufacture any of our product candidates on a clinical or commercial scale. We currently rely on third 
parties for supply of the active pharmaceutical ingredients, or API, in our product candidates. Our strategy is to 
outsource all manufacturing of our product candidates and any approved products to third parties. 

We do not currently have any agreements with third-party manufacturers for the long-term clinical or commercial 

supply of any of our product candidates. We currently engage a single third-party manufacturer to provide API for 
odevixibat and elobixibat. We also currently engage single third-party manufacturers to provide fill and finish services 
for the final drug product formulation of our product candidate in development. We may in the future be unable to enter 
into agreements for commercial supply with third-party manufacturers on acceptable terms, or at all. In addition, while 
we believe that there are alternative sources available to manufacture our product candidates, in the event that we seek 
such alternative sources, we may not be able to enter into replacement arrangements without delays or additional 
expenditures. We cannot estimate these delays or costs with certainty but, if they were to occur, they could cause a delay 
in our development and commercialization efforts. If our third party manufacturing agreements are terminated or if the 
sources of supply from such arrangements are inadequate and we must seek supply agreements from alternative sources, 
we may be unable to enter into such agreements or do so on commercially reasonable terms, which could delay a product 
launch or subject our commercialization efforts to significant supply risk. 

Even if we are able to establish and maintain arrangements with third-party manufacturers, reliance on third-party 

manufacturers entails additional risks, including: 

• 
• 
• 
• 

reliance on the third party for regulatory compliance and quality assurance; 
the possible breach of the manufacturing agreement by the third party; 
the possible misappropriation of our proprietary information, including our trade secrets and know-how; and 
the possible termination or nonrenewal of the agreement by the third party at a time that is costly or 
inconvenient for us. 

Manufacturers of our product candidates are obliged to operate in accordance with FDA-mandated current good 
manufacturing practices, or cGMP’s. The manufacture of pharmaceutical products in compliance with cGMPs requires 
significant expertise and capital investment, including the development of advanced manufacturing techniques and 
process controls. Manufacturers of pharmaceutical products often encounter difficulties in production, including 
difficulties with volume production, production costs and yields, laboratory testing, quality control, including stability of 
the product or product candidate, or quality assurance testing, or suffer shortages of qualified personnel, as well as 
compliance with strictly enforced cGMP requirements, other federal and state regulatory requirements and foreign 
regulations, any of which could result in our inability to manufacture sufficient quantities to meet clinical timelines for a 
particular product candidate, to obtain marketing approval for the product candidate or to commercialize the product 
candidate. If our manufacturers were to encounter any of these difficulties or otherwise fail to comply with their 
obligations to us or under applicable regulations, our ability to provide product for commercial sale or product 
candidates in our clinical trials would be jeopardized. 

In addition, the facilities used by our contract manufacturers or other third party manufacturers to manufacture our 
product candidates must be approved by the FDA pursuant to inspections conducted following our request for regulatory 
approval for our product candidates from the FDA. These requirements include, among other things, quality control, 
quality assurance and the maintenance of records and documentation. Manufacturers of our product candidates may be 
unable to comply with these cGMP requirements and with other FDA, state and foreign regulatory requirements. The 

65 

 
 
 
 
 
FDA or similar foreign regulatory agencies may also implement new standards at any time, or change their interpretation 
and enforcement of existing standards for manufacture, packaging or testing of products. We have little control over our 
manufacturers' compliance with these regulations and standards. A failure of any of our current or future contract 
manufacturers to establish and follow cGMPs and to document their adherence to such practices may lead to significant 
delays in clinical trials or in obtaining regulatory approval of product candidates or the ultimate launch of products, if 
approved, into the market. Failure by our current or future third-party manufacturers or us to comply with applicable 
regulations could result in sanctions being imposed on us, including fines, injunctions, civil penalties, failure of the 
government to grant pre-market approval of drugs, delays, suspension or withdrawal of approvals, seizures or recalls of 
products, operating restrictions, and criminal prosecutions. If the safety of any product supplied is compromised due to 
our manufacturers' failure to adhere to applicable laws or for other reasons, we may not be able to obtain regulatory 
approval for or successfully commercialize our products and we may be held liable for any injuries sustained as a result. 
Any of these factors could cause a delay of clinical studies, regulatory submissions, approvals or commercialization of 
our product candidates or approved products, entail higher costs or impair our reputation. 

Our product candidates and any products that we may develop may compete with other product candidates and 
products for access to manufacturing facilities. There are a limited number of manufacturers that operate under cGMP 
regulations and that might be capable of manufacturing for us. 

If the third parties that we engage to manufacture product for our preclinical tests and clinical trials cease to 
continue to do so for any reason, we likely would experience delays in advancing these clinical trials while we identify 
and qualify replacement suppliers and we may be unable to obtain replacement supplies on terms that are favorable to 
us. In addition, if we are not able to obtain adequate supplies of our product candidates or the drug substances used to 
manufacture them, it will be more difficult for us to develop our product candidates and compete effectively. 
Furthermore, any delay or interruption in the supply of commercial quantities of approved product could have a material 
adverse impact on our revenue from product sales and any delay or interruption in the supply of clinical trial materials 
could delay the completion of our clinical trials, increase the costs associated with maintaining our clinical development 
programs and, depending upon the period of delay, require us to commence new clinical trials or redo work that has 
already been done, in either case at significant additional expense to us, or to terminate the clinical trials completely. 

Our current and anticipated future dependence upon others for the manufacture of our product candidates may 
adversely affect our future profit margins and our ability to develop product candidates and commercialize any products 
that receive marketing approval on a timely and competitive basis. 

We may depend on additional collaborations, licenses or similar arrangements with third parties for the 
development and commercialization of some of our product candidates. If those collaborations are not successful, we 
may not be able to capitalize on the market potential of these product candidates. 

We have licensed rights to develop and commercialize elobixibat for CIC and other gastrointestinal diseases and 

disorders to EA Pharma in Japan and other select markets in Asia. We may in the future enter into other licensing, 
collaboration or similar arrangements for the development and commercialization of odevixibat, elobixibat, A3907, 
A2342 or any potential future product candidate of ours for any or all indications and for any or all territories, except for 
the rights currently subject to EA Pharma’s license with respect to elobixibat. 

Our likely counterparties for any licensing, collaboration or similar arrangement include large and mid-size 
pharmaceutical companies, regional and national pharmaceutical companies and biotechnology companies. Except for 
our agreement with EA Pharma, we are not currently party to any such arrangement for odevixibat, elobixibat A3907 or 
A2342. However, if we do enter into any such arrangements with any third parties in the future, we will likely have 
limited control over the amount and timing of resources that our collaborators dedicate to the development or 
commercialization of the applicable product candidate. Our ability to generate revenues from these arrangements will 
depend on our collaborators’ abilities and efforts to successfully perform the functions assigned to them in these 
arrangements. 

66 

 
 
 
 
Any licensing, collaboration or similar arrangement involving our product candidates would pose numerous risks 

to us, including the following: 

• 

• 

• 

• 

• 

• 

• 

• 

• 

• 

• 

collaborators have significant discretion in determining the efforts and resources that they will apply to these 
collaborations and may not perform their obligations as expected; 

collaborators may deemphasize or not pursue development and commercialization of our product candidates 
or may elect not to continue or renew development or commercialization programs based on clinical trial 
results, changes in the collaborators’ strategic focus, including as a result of a sale or disposition of a 
business unit or development function, or available funding, or external factors such as an acquisition that 
diverts resources or creates competing priorities; 

collaborators may delay clinical trials, provide insufficient funding for a clinical trial program, stop a clinical 
trial or abandon a product candidate, repeat or conduct new clinical trials or require a new formulation of a 
product candidate for clinical testing; 

collaborators may independently develop, or develop with third parties, products that compete directly or 
indirectly with our products or product candidates if the collaborators believe that competitive products are 
more likely to be successfully developed or can be commercialized under terms that are more economically 
attractive than ours; 

a collaborator with marketing and distribution rights to multiple products may not commit sufficient 
resources to the marketing and distribution of our product relative to other products; 

collaborators may not properly maintain or defend our intellectual property rights or may use our proprietary 
information in such a way as to invite litigation that could jeopardize or invalidate our intellectual property 
or proprietary information or expose us to potential litigation; 

collaborators may infringe the intellectual property rights of third parties, which may expose us to litigation 
and potential liability; 

disputes may arise between us and a collaborator as to the ownership of intellectual property arising during 
the collaboration; 

we may grant exclusive rights to our collaborators, which would prevent us from collaborating with others 
or make us a less attractive collaboration partner by narrowing the scope of potential collaborations into 
which we may enter; 

disputes may arise between us and a collaborator that result in the delay or termination of the research, 
development or commercialization of our products or product candidates or that result in costly litigation or 
arbitration that diverts management attention and resources; and 

collaborations may be terminated and, if terminated, may result in a need to identify and enter into a new 
licensing, collaboration or similar arrangement or obtain additional capital to pursue further development or 
commercialization of the applicable product candidates. 

Collaboration agreements may not lead to development or commercialization of product candidates in the most 

efficient manner or at all. If a collaborator of ours were to be involved in a business combination, the continued pursuit 
and emphasis on our product development or commercialization program could be delayed, diminished or terminated. 

67 

If we are not able to establish additional collaborations, we may have to alter our development and 

commercialization plans. 

Our product development programs and the potential commercialization of our product candidates will require 
substantial additional cash to fund expenses. For some of our product candidates, we may decide to collaborate with 
pharmaceutical and biotechnology companies for the development and potential commercialization of those product 
candidates. 

We face significant competition in seeking appropriate collaborators. Whether we reach a definitive agreement for 

a collaboration will depend, among other things, upon our assessment of the collaborator’s resources and expertise, the 
terms and conditions of the proposed collaboration and the proposed collaborator’s evaluation of a number of factors. 
Those factors may include the design or results of clinical trials, the likelihood of approval by regulatory authorities, the 
potential market for the subject product candidate, the costs and complexities of manufacturing and delivering such 
product candidate to patients, the potential of competing products, the existence of uncertainty with respect to our 
ownership of technology, which can exist if there is a challenge to such ownership without regard to the merits of the 
challenge, and industry and market conditions generally. The collaborator may also consider alternative product 
candidates or technologies for similar indications that may be available to collaborate on and whether such a 
collaboration could be more attractive than the one with us for our product candidate. We may also be restricted under 
future license agreements from entering into agreements on certain terms with potential collaborators. Collaborations are 
complex and time-consuming to negotiate and document. In addition, there have been a significant number of business 
combinations among large pharmaceutical companies that have resulted in a reduced number of potential future 
collaborators. 

We may not be able to negotiate collaborations on a timely basis, on acceptable terms, or at all. If we are unable to 
do so, we may have to curtail the development of a product candidate, reduce or delay our development program or one 
or more of our other development programs, delay our potential commercialization or reduce the scope of any sales or 
marketing activities, or increase our expenditures and undertake development or commercialization activities at our own 
expense. If we do not have sufficient funds, we may not be able to further develop our product candidates or bring them 
to market and generate product revenue. 

Risks Related to Our Intellectual Property  

If we are unable to obtain and maintain patent protection for our technology and products, or if the scope of 

the patent protection is not sufficiently broad, our competitors could develop and commercialize technology and 
products similar or identical to ours, and our ability to successfully commercialize our technology and products may 
be adversely affected. 

Our success depends in large part on our ability to obtain and maintain patent protection in the United States and 
other countries with respect to our proprietary technology and products. We seek to protect our proprietary position by 
filing patent applications in the United States, in Europe and in certain additional jurisdictions related to our novel 
technologies and product candidates that are important to our business. This process is expensive and time-consuming, 
and we may not be able to file and prosecute all necessary or desirable patent applications at a reasonable cost or in a 
timely manner. It is also possible that we will fail to identify patentable aspects of our research and development output 
before it is too late to obtain patent protection. Moreover, if we license technology or product candidates from third 
parties in the future, these license agreements may not permit us to control the preparation, filing and prosecution of 
patent applications, or to maintain or enforce the patents, covering the licensed technology or product candidates. These 
agreements could also give our licensors the right to enforce the licensed patents without our involvement, or to decide 
not to enforce the patents at all. Therefore, in these circumstances, these patents and applications may not be prosecuted 
or enforced in a manner consistent with the best interests of our business. 

The patent position of biotechnology and pharmaceutical companies generally is highly uncertain, involves 
complex legal and factual questions and has in recent years been the subject of much litigation. As a result, the issuance, 
scope, validity, enforceability and commercial value of our patent rights are highly uncertain. Our pending and future 

68 

patent applications may not result in patents being issued which protect our technology or products, in whole or in part, 
or which effectively prevent others from commercializing competitive technologies and products. Changes in either the 
patent laws or interpretation of the patent laws in the United States and other countries may diminish the value of our 
patents, narrow the scope of our patent protection or make enforcement more difficult or uncertain. 

The laws of other countries may not protect our patent rights to the same extent as the laws of the United States. 

Consequently, we may not be able to prevent third parties from practicing our inventions in all countries outside the 
United States, or from selling or importing products made using our inventions in and into the United States or other 
jurisdictions. Many companies have encountered significant difficulties in protecting and defending intellectual property 
rights in such foreign jurisdictions. The legal systems of certain countries, including certain developing countries, do not 
favor the enforcement of patents, trade secrets and other intellectual property protection, particularly those relating to 
biotechnology, which could make it difficult for us to stop the infringement of our patent rights or the marketing of 
competing products in violation of our intellectual property and proprietary rights generally.  For example, European 
patent law restricts the patentability of methods of treatment of the human body more than U.S. law does. For this or 
other reasons, we may not pursue or obtain patent protection in all major markets or may not obtain protection that 
enables us to prevent the entry of third parties onto the market. 

Additionally, proceedings to enforce our intellectual property and proprietary rights in foreign jurisdictions could 
result in substantial costs and divert our efforts and attention from other aspects of our business, could put our patents at 
risk of being invalidated or interpreted narrowly, could put our patent applications at risk of not issuing and could 
provoke third parties to assert claims against us. We may not prevail in any lawsuits that we initiate, and the damages or 
other remedies awarded, if any, may not be commercially meaningful. Accordingly, our efforts to enforce our 
intellectual property and proprietary rights around the world may be inadequate to obtain a significant commercial 
advantage from the intellectual property that we develop or license.  

Further, many countries have compulsory licensing laws under which a patent owner may be compelled to grant 
licenses to third parties. In addition, many countries limit the enforceability of patents against government agencies or 
government contractors. In these countries, the patent owner may have limited remedies, which could materially 
diminish the value of such patent. If we are forced to grant a license to third parties with respect to any patents relevant 
to our business, our competitive position may be impaired, and our business, financial condition, results of operations 
and prospects may be adversely affected. 

Assuming the other requirements for patentability are met, currently, the first to file a patent application is 
generally entitled to the patent. However, prior to March 16, 2013, in the United States, the first to invent was entitled to 
the patent. Publications of discoveries in the scientific literature often lag behind the actual discoveries, and patent 
applications in the United States and other jurisdictions are typically not published until 18 months after filing, or in 
some cases not at all. Therefore, we cannot know with certainty whether we were the first to make the inventions 
claimed in our U.S. patents or pending U.S. patent applications filed prior to March 16, 2013. 

Moreover, we may be subject to a third party preissuance submission of prior art to the U.S. Patent and Trademark 

Office, or the USPTO, or become involved in opposition, derivation, reexamination, reissue, inter partes review, post 
grant review, interference proceedings or other patent office proceedings, court litigation or International Trade 
Commission proceedings, in the United States or elsewhere, challenging our patent rights or the patent rights of others. 
An adverse determination in any such submission, proceeding or litigation concerning our patent rights could reduce the 
scope of or prevent the enforceability of, or invalidate, our patent rights, allowing third parties to commercialize our 
technology or products, or equivalent or similar technology or products, and so to compete directly with us, without 
payment to us, or, where such proceedings involve third-party patents, result in our inability to manufacture or 
commercialize products without infringing third-party patent rights. In addition, if the breadth or strength of protection 
provided by our patents and patent applications is threatened or narrowed by operation of any of the foregoing, such an 
event could dissuade companies from collaborating with us to license, develop or commercialize current or potential 
future product candidates of ours. 

69 

Even if our patent applications issue as patents, they may not issue in a form that will provide us with adequate 
protection to prevent competitors from competing with us or otherwise to provide us with any competitive advantage. 
Our competitors may be able to circumvent our owned or licensed patents by developing similar, improved or alternative 
technologies or products in a noninfringing manner. Moreover, method-of-treatment patent claims are more difficult to 
enforce than composition-of-matter claims for reasons including off-label sale, potential divided infringement issues and 
use of the subject compound in noninfringing manners. Physicians are permitted to prescribe an approved product for 
uses that are not described in the product’s labeling. Although off-label prescriptions may infringe our method-of-
treatment patents, the practice is common across medical specialties and such infringement is difficult to prevent or 
prosecute. Off-label sales would limit our ability to generate revenue from the sale of our product candidates, if 
approved for commercial sale. In addition, if a third party were able to design around our dosage-form and formulation 
patents and create a different formulation and dosage form that is not covered by our patents or patent applications, we 
would likely be unable to prevent that third party from manufacturing and marketing our product. 

In addition, other companies may attempt to circumvent any regulatory data protection or market exclusivity that 

we obtain under applicable legislation, such as orphan drug exclusivity in the United States, which may require us to 
allocate significant resources to preventing such circumvention. Legal and regulatory developments in the European 
Union and elsewhere may also result in clinical trial data submitted as part of a marketing authorization application 
becoming publicly available. Such developments could enable other companies to use our clinical trial data to assist in 
their own product development and to obtain marketing authorizations in the European Union and in other jurisdictions. 
Such developments may also require us to allocate significant resources to prevent other companies from circumventing 
or violating our intellectual property rights. Our attempts to prevent third parties from circumventing our intellectual 
property and other rights may ultimately be unsuccessful. We may also fail to take the required actions or pay the 
necessary fees to maintain our patents. 

The issuance of a patent is not conclusive as to its inventorship, scope, validity or enforceability, and our owned 
and licensed patents may be challenged in the courts or patent offices in the United States, Europe and elsewhere. Such 
challenges may result in loss of exclusivity or in patent claims being narrowed, invalidated or held unenforceable, in 
whole or in part, which could limit our ability to stop others from using or commercializing similar or identical 
technology and products, or limit the duration of the patent protection of our technology and products. Future changes in 
U.S. statutory or case law beyond our control could affect some or all of the foregoing possibilities. Given the amount of 
time required for the development, testing and regulatory review of new product candidates, patents protecting such 
candidates might expire before or shortly after such candidates are commercialized. This could be the case even after 
giving effect to patent term extensions and data exclusivity provisions preventing third parties from relying on clinical 
trial data filed by us for marketing approval in support of their own applications for such approval. As a result, our patent 
portfolio may not provide us with sufficient rights to exclude others from commercializing products similar or identical 
to ours. 

We may become involved in lawsuits or other enforcement proceedings to protect or enforce our patents or 

other intellectual property, which could be expensive, time consuming and potentially unsuccessful. 

Competitors may infringe our patents, trademarks, copyrights or other intellectual property. To counter 

infringement or unauthorized use, we may be required to file claims, which can be expensive and time consuming. Any 
claims we assert against perceived infringers could provoke these parties to assert counterclaims against us alleging that 
we infringe their intellectual property or that our patent and other intellectual property rights are invalid or 
unenforceable, including for antitrust reasons. As a result, in a patent infringement proceeding, a court or administrative 
body may decide that a patent of ours is invalid or unenforceable, in whole or in part, or may construe the patent’s 
claims narrowly and so refuse to stop the other party from using the technology at issue on the grounds that our patents 
do not cover the competitor technology in question. Even if we are successful in a patent infringement action, the 
unsuccessful party may subsequently raise antitrust issues and bring a follow-on action. Antitrust issues may also 
provide a bar to settlement or constrain the permissible settlement terms. Further, settlement agreements in the 
pharmaceutical sector are the subject of ongoing review by the antitrust authorities in the European Union. 

70 

Third parties may initiate legal proceedings alleging that we are infringing their intellectual property rights, the 

outcome of which would be uncertain and could have a material adverse effect on the success of our business. 

Our commercial success depends upon our ability and the ability of our current or potential future licensees or 

collaborators to develop, manufacture, market and sell our product candidates and use our proprietary technologies 
without infringing the intellectual property and other proprietary rights of third parties. There is considerable intellectual 
property litigation in the biotechnology and pharmaceutical industries, and we may become party to, or threatened with, 
future adversarial proceedings or litigation regarding intellectual property rights with respect to our products and 
technology, including interference, derivation, inter partes review, reexamination, reissue or post-grant review 
proceedings before the USPTO. The risks of being involved in such litigation and office proceedings may also increase 
as our product candidates approach commercialization, and as our business gains greater visibility operating as a 
publicly traded company in the United States. Third parties may assert infringement claims against us based on existing 
or future intellectual property rights and so restrict our freedom to operate. Third parties may also seek injunctive relief 
against us, whereby they would attempt to prevent us from practicing our technologies altogether pending the outcome 
of any litigation against us. We may not be aware of all such intellectual property rights potentially relating to our 
product candidates prior to their assertion against us. For example, we have not conducted an in depth freedom-to-
operate search or analysis for odevixibat. Any freedom-to-operate search or analysis previously conducted may not have 
uncovered all relevant patents and pending patent applications, and there may be pending or future patent applications 
that, if issued, would block us from commercializing odevixibat or any other product candidate. Thus, we do not know 
with certainty whether odevixibat or any other product candidate, or our commercialization of any such product 
candidate, does not and will not infringe any third party’s intellectual property. 

If we are found to infringe a third party’s intellectual property rights, or in order to avoid or settle litigation, we 
could be required to obtain a license to enable us to continue developing and marketing our products and technology. 
However, we may not be able to obtain any required license on commercially reasonable terms, or at all. Even if we 
were able to obtain a license, it could be nonexclusive, thereby giving our competitors access to the same technologies 
that we have then licensed, and could require us to make substantial payments. Absent a license, we could be forced, 
including by court order, to cease commercializing the infringing technology or product. In addition, we could be found 
liable for monetary damages, including treble damages and attorneys’ fees if we are found to have willfully infringed a 
patent or other intellectual property right. A finding of infringement could prevent us from commercializing our product 
candidates or force us to cease some of our business operations, which could materially harm our business. Claims that 
we have misappropriated the confidential information or trade secrets of third parties, or claims that we derived 
inventions from another, could have a similar negative impact on our business. 

We may be subject to claims by third parties asserting that we or our employees have misappropriated their 

intellectual property, or claiming ownership of what we regard as our own intellectual property. 

Many of our employees were previously employed at universities or other biotechnology or pharmaceutical 

companies. Although we try to ensure that our employees do not use the proprietary or otherwise confidential 
information or know-how of others in their work for us, we may be subject to claims that we or these employees have 
without authorization used or disclosed intellectual property, including trade secrets or other proprietary or confidential 
information, of any such employee’s former employer. Litigation may be necessary to defend against these claims. 

In addition, while we typically require our employees and contractors who may be involved in the development of 
intellectual property to execute agreements assigning such intellectual property to us and agreeing to cooperate and assist 
us with securing and defending our intellectual property, we may be unsuccessful in executing such an agreement with 
each party who in fact develops intellectual property that we regard as our own. These assignment agreements may not 
be self-executing or may be breached, and we may be forced to bring claims against third parties, or defend claims they 
may bring against us, to determine the ownership of what we regard as our intellectual property. 

If we fail in prosecuting or defending any such claims, in addition to paying monetary damages, we may lose 
valuable intellectual property rights or personnel. Even if we are successful in prosecuting or defending against such 
claims, litigation could result in substantial costs and be a distraction to management. 

71 

Intellectual property litigation could cause us to spend substantial resources and could distract our personnel 

from their normal responsibilities. 

Even if resolved in our favor, litigation or other legal proceedings relating to intellectual property claims may 
cause us to incur significant expenses and could distract our technical and management personnel from their normal 
responsibilities. In addition, there could be public announcements of the results of hearings, motions or other interim 
proceedings or developments. If securities analysts or investors perceive these results to be negative, it could have a 
substantial adverse effect on the price of our common stock. Such litigation or proceedings could substantially increase 
our operating losses and reduce the resources available for development, sales, marketing or distribution activities. We 
may not have sufficient financial or other resources to adequately conduct such litigation or proceedings. Some of our 
competitors may be able to sustain the costs of such litigation or proceedings more effectively than we can because of 
their greater financial resources. Accordingly, costs and lost management time, as well as uncertainties resulting from 
the initiation and continuation of patent litigation or other proceedings, could have a material adverse effect on our 
ability to compete in the marketplace. 

If we do not obtain protection under the Hatch-Waxman Act and similar legislation outside of the United States 

by extending the patent terms and obtaining data exclusivity for our product candidates, our business may be 
materially harmed. 

Depending upon the timing, duration and specifics of FDA marketing approval of odevixibat, A3907, A2342 or 

potential future product candidates of ours, if any, one or more of our U.S. patents may be eligible for limited patent 
term restoration under the Drug Price Competition and Patent Term Restoration Act of 1984, referred to as the Hatch-
Waxman Act. The Hatch-Waxman Act permits a patent restoration term of up to five years as compensation for patent 
term lost during product development and the FDA regulatory review process. However, we may not be granted an 
extension if, for example, we fail to apply within applicable deadlines, we fail to apply prior to expiration of relevant 
patents or if we otherwise fail to satisfy applicable requirements. Moreover, the applicable time period or the scope of 
patent protection afforded could be less than we request. If we are unable to obtain patent term extension or restoration 
or the term of any such extension is less than we request, the period during which we will have the right to exclusively 
market our products will be shortened and our competitors may obtain approval of competing products following our 
patent expiration, and our revenue could be reduced, possibly materially. In the event that we are unable to obtain any 
patent term extension, the issued U.S. composition of matter patent for odevixibat is expected to expire in 2022 
assuming it withstands any challenge. In the event that we are unable to obtain any patent term extension, the issued U.S. 
composition of matter patent for elobixibat is expected to expire in 2022, assuming it withstands any challenge. We 
expect that the other U.S. patents and patent applications for odevixibat and elobixibat, if issued, and if the appropriate 
maintenance, renewal, annuity or other governmental fees are paid, would expire from 2031 to 2039. 

If we are unable to protect the confidentiality of our trade secrets, our business and competitive position would 

be harmed. 

In addition to seeking patents for some of our technology and products, we also rely on trade secrets, including 

unpatented know-how, technology and other proprietary and confidential information, to maintain our competitive 
position. We seek to protect these trade secrets, in part, by entering into nondisclosure and confidentiality agreements 
with parties who have access to them, such as our employees, corporate collaborators, outside scientific collaborators, 
contract manufacturers, consultants, advisors and other third parties. However, we cannot guarantee that we have 
executed these agreements with each party that may have or have had access to our trade secrets or that the agreements 
we have executed will provide adequate protection. Any party with whom we have executed such an agreement may 
breach that agreement and disclose our proprietary or confidential information, including our trade secrets, and we may 
not be able to obtain adequate remedies for such breaches. Enforcing a claim that a party illegally disclosed or 
misappropriated a trade secret is difficult, expensive and time-consuming, and the outcome is unpredictable. In addition, 
some courts inside and outside the United States are less willing or unwilling to protect trade secrets. If any of our trade 
secrets were to be lawfully obtained or independently developed by a competitor, we would have no right to prevent 
them, or those to whom they communicate the trade secret, from using that technology or information to compete with 
us. If any of our trade secrets, particularly unpatented know-how, were to be obtained or independently developed by a 
competitor, our competitive position would be harmed. 

72 

Risks Related to Regulatory Approval and Marketing of Our Product Candidates 

A rare pediatric disease designation may not lead to the receipt of a rare pediatric disease priority review 

voucher, even if odevixibat is approved. 

The FDA has awarded rare pediatric disease priority review vouchers, or PRVs, to sponsors of drug products 
intended to treat rare pediatric disease products if the treatment and product application meet certain criteria. Under this 
program, upon the approval of a qualifying NDA or biologics license application, BLA, for the treatment or prevention 
of a rare pediatric disease, the sponsor of the application may be eligible for a PRV that can be used to obtain priority 
review for a subsequent NDA or BLA. The PRV may be sold or transferred an unlimited number of times. The FDA has 
granted rare pediatric disease designation for odevixibat for PFIC. The PRV program is now set to expire at the end of 
2024, or 2026 for products that receive rare pediatric disease designation by 2024.Therefore, there is no guarantee that 
we will receive a PRV for odevixibat even if it is approved by the FDA to treat a rare pediatric disease. 

If prior to any marketing approval in the European Union of odevixibat to treat PFIC, Mirum’s maralixibat is 

approved in the European Union to treat PFIC and at the time of approval maintains its designation as an orphan 
medicinal product, and if odevixibat is deemed to be a similar medicinal product, within the meaning of E.U. law, to 
maralixibat, we may not be able to obtain marketing approval of odevixibat in the European Union for a significant 
period of time. In addition, odevixibat may not be entitled to orphan drug exclusivity for odevixibat in the United 
States or European Union notwithstanding its current orphan designation. 

Regulatory authorities in some jurisdictions, including the United States and European Union, may designate 

drugs for relatively small patient populations as orphan drugs. The FDA has granted orphan drug designation to 
odevixibat, which is an IBAT inhibitor, for the treatment of PFIC, biliary atresia, PBC and ALGS, and the European 
Commission has designated odevixibat as an orphan medicinal product for the treatment of PFIC, biliary atresia, PBC 
and ALGS. Mirum’s maralixibat, which has also been reported to be an IBAT inhibitor, has also been granted orphan 
drug designation by the FDA and as an orphan medicinal product by the European Commission. 

Generally, if a designated orphan medicinal product receives the first marketing approval in the European Union 
for the orphan indication for which it has been designated and maintains under applicable criteria its designation as an 
orphan medicinal product at the time of approval, the product is entitled to a period of market exclusivity in the 
European Union. Subject to certain exceptions, this market exclusivity precludes the EMA from accepting another 
marketing application for a “similar medicinal product” for the same indication for 10 years, which can be reduced to 
six years if a drug no longer meets the criteria for orphan drug designation (including if the drug is sufficiently profitable 
so that market exclusivity is no longer justified). Under E.U. law, a “similar medicinal product” is a medicinal product 
that contains a similar active substance or substances as contained in the authorized orphan medicinal product and that is 
intended for the same therapeutic indication and a “similar active substance” is an active substance that is identical or 
has the same principal molecular structural features (but not necessarily all of the same molecular features) and acts via 
the same mechanism as the authorized orphan medicinal product. 

Maralixibat has been evaluated to date in a greater number of PFIC patients than has odevixibat. If (1) prior to 

marketing approval, if any, of odevixibat to treat PFIC in the European Union, maralixibat is approved in the European 
Union to treat PFIC and at the time of approval maintains its designation as an orphan medicinal product, (2) odevixibat 
is deemed to be a similar medicinal product to maralixibat and (3) we are not able to establish that odevixibat provides a 
significant benefit to patients compared with maralixibat, we may not be able to obtain marketing approval of odevixibat 
in the European Union for at least several years. We understand that Mirum’s MAA for maralixibat for the treatment of 
patients with PFIC type 2 has been accepted for review by the EMA. 

Moreover, we may not be able to obtain orphan drug exclusivity in the United States or the European Union for 
odevixibat for PFIC or any other indication, notwithstanding the fact that odevixibat has been designated as an orphan 
drug in the United States or an orphan medicinal product in the European Union. For example, if a competitive product 
that is the same drug as odevixibat is shown to be clinically superior, any orphan drug exclusivity that we have obtained 
in the United States will not block the approval of such competitive product. In addition, orphan drug exclusivity will not 

73 

prevent the approval in the United States of a product that is the same drug as our product candidate if the FDA finds 
that we cannot assure the availability of sufficient quantities of the drug to meet the needs of the persons with the disease 
or condition for which the drug was designated. Moreover, if prior to marketing approval, if any, of odevixibat to treat 
PFIC in the European Union, maralixibat or any other product is approved in the European Union to treat PFIC, 
odevixibat may not be entitled to orphan drug exclusivity if we are not able to establish that it provides a significant 
benefit to patients compared with maralixibat. Finally, even if we obtain orphan drug exclusivity for a product, that 
exclusivity may not effectively protect the product from competition because different drug products can be approved 
for the same condition. 

Even if we complete the necessary clinical trials, the marketing approval process is expensive, time consuming 
and uncertain and may prevent us from obtaining approvals for the commercialization of some or all of our product 
candidates. If we are not able to obtain, or if there are delays in obtaining, required marketing approvals, we will not 
be able to commercialize our product candidates, and our ability to generate revenue will be materially impaired. 

Our product candidates, including odevixibat, A3907 and A2342, and the activities associated with their 

development and commercialization, including their design, testing, manufacture, safety, efficacy, recordkeeping, 
labeling, storage, approval, advertising, promotion, sale and distribution, are subject to comprehensive regulation by the 
FDA and by comparable authorities in other countries. Failure to obtain marketing approval for a product candidate will 
prevent us from commercializing the product candidate. In December 2020, we announced that we submitted an NDA to 
the FDA and an MAA to the EMA, seeking approval of odevixibat for the treatment of patients with PFIC. Other than 
the approval of elobixibat received by EA Pharma for the treatment of chronic constipation in Japan, we have not 
received approval to market odevixibat, A3907, A2342 or any other product candidate from regulatory authorities in any 
jurisdiction. 

We have only limited experience in filing and supporting the applications necessary to obtain marketing approvals 
for product candidates and have relied on third-party contract research organizations to assist us in this process. Securing 
marketing approval requires the submission of extensive preclinical and clinical data and supporting information to 
regulatory authorities for each therapeutic indication to establish the product candidate’s safety and effectiveness. 
Securing marketing approval also requires the submission of information about the product manufacturing process to, 
and inspection of manufacturing facilities by, the regulatory authorities. Regulatory authorities may determine that 
odevixibat, A3907, A2342 or any potential future product candidate of ours is not effective, is only moderately effective 
or has undesirable or unintended side effects, toxicities, safety profiles or other characteristics that preclude us from 
obtaining marketing approval or that prevent or limit commercial use. 

The process of obtaining marketing approvals is expensive, may take many years, if approval is obtained at all, 
and can vary substantially based upon a variety of factors, including the type, complexity and novelty of the product 
candidates involved. Changes in marketing approval policies during the development period, changes in or the 
enactment of additional statutes or regulations, or changes in regulatory review for each submitted product application, 
may cause delays in the approval or rejection of an application. Regulatory authorities have substantial discretion in the 
approval process and may refuse to accept any application or may decide that our data are insufficient for approval and 
require additional preclinical studies, clinical trials or other trials. In addition, varying interpretations of the data obtained 
from preclinical and clinical testing could delay, limit or prevent marketing approval of a product candidate. Any 
marketing approval we ultimately obtain may be limited or subject to restrictions or post-approval commitments that 
render the approved product not commercially viable. If we experience delays in obtaining approval or if we fail to 
obtain approval of our product candidates, the commercial prospects for our product candidates may be harmed and our 
ability to generate revenues will be materially impaired. 

Additionally, on June 23, 2016, the electorate in the United Kingdom voted in favor of leaving the European 
Union, commonly referred to as “Brexit.” On March 29, 2017, the country formally notified the European Union of its 
intention to withdraw pursuant to Article 50 of the Lisbon Treaty. The withdrawal of the United Kingdom from the 
European Union took effect on January 31, 2020, the effective date of the withdrawal agreement, with a transition period 
that ended on December 31, 2020. Since a significant proportion of the regulatory framework in the United Kingdom 
was, prior to Brexit, derived from European Union directives and regulations, Brexit and the new Trade and Cooperation 
Agreement between the European Union and the United Kingdom that took provisional effect on January 1, 2021 could 

74 

materially impact the regulatory regime with respect to the approval of any product candidates in the United Kingdom. 
Any delay in obtaining, or an inability to obtain, any marketing approvals, as a result of Brexit or otherwise, would 
prevent us from commercializing any product candidates in the United Kingdom and restrict our ability to generate 
revenue and achieve and sustain profitability. If any of these outcomes occur, we may be forced to restrict or delay 
efforts to seek regulatory approval in the United Kingdom and/or European Union for our product candidates, which 
could significantly and materially harm our business. 

Our failure to obtain marketing approval in jurisdictions other than the United States and Europe would 
prevent our product candidates from being marketed in these other jurisdictions. Any approval that we are granted 
for our product candidates in the United States or Europe would not assure approval of product candidates in the 
other or in any other jurisdiction. 

In order to market and sell odevixibat, A3907, A2342 or any potential future product candidate of ours in 
jurisdictions other than the United States or Europe, we or a current or potential future licensee or collaborator must 
obtain separate marketing approvals and comply with numerous and varying regulatory requirements. The approval 
procedure varies among countries and can involve additional testing. The time required to obtain approval may differ 
from that required to obtain FDA or EMA approval. The regulatory approval process outside the United States and 
Europe generally includes all of the risks associated with obtaining FDA and EMA approval. In addition, some countries 
outside the United States and Europe require approval of the sales price of a drug before it can be marketed. In many 
countries, separate procedures must be followed to obtain reimbursement. We or a current or potential future licensee or 
collaborator may not obtain marketing, pricing or reimbursement approvals outside the United States and Europe on a 
timely basis, if at all. 

Approval by the FDA does not ensure approval by the EMA, approval by the EMA does not assure approval by 

the FDA, and approval of either or both of the FDA and EMA does not assure approval by regulatory authorities in other 
countries or jurisdictions. Likewise, approval by any regulatory authority in any country or jurisdiction outside the 
United States or Europe, such as Japan, does not assure approval by regulatory authorities in other countries or 
jurisdictions or by the FDA or EMA. We and any current or potential future licensee or collaborator may not be able to 
file for marketing approvals and may not receive necessary approvals to commercialize our product candidates in any 
market. Marketing approvals in countries outside the United States and Europe do not ensure pricing approvals in those 
countries or in any other countries, and marketing approvals and pricing approvals do not ensure that reimbursement will 
be obtained. 

Our ability to obtain and maintain conditional marketing authorizations in the European Union is limited to 

specific circumstances and subject to several conditions and obligations. A failure to renew any conditional approval 
that we obtain prior to full approval for the applicable indication would prevent us from continuing to market our 
products. 

Conditional marketing authorizations in the European Union based on incomplete clinical data may be granted for 

a limited number of listed medicinal products for human use, including products designated as orphan medicinal 
products under E.U. law, if (1) the risk-benefit balance of the product is positive, (2) it is likely that the applicant will be 
in a position to provide the required comprehensive clinical trial data, (3) unmet medical needs will be fulfilled and 
(4) the benefit to public health of the immediate availability on the market of the medicinal product outweighs the risk 
inherent in the fact that additional data are still required. Specific obligations, including with respect to the completion of 
ongoing or new studies or trials, and with respect to the collection of pharmacovigilance data, may be specified in the 
conditional marketing authorization. Conditional marketing authorizations are valid for one year and may be renewed 
annually, if the risk-benefit balance remains positive, and after an assessment of the need for additional or modified 
conditions. Even if we obtain conditional approval for odevixibat for the treatment of PFIC or any other pediatric 
cholestatic liver disease or disorder, we may not be able to renew such conditional approval. 

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Even if we obtain marketing approval for our product candidates, the terms of approvals and ongoing 
regulation of our products may limit how we manufacture or market our products and compliance with such 
requirements may involve substantial resources, which could materially impair our ability to generate profit. 

Even if marketing approval of a product candidate is granted, an approved product and its manufacturer and 
marketer are subject to ongoing review and extensive regulation, including the possible requirement to implement a risk 
evaluation and mitigation strategy or to conduct costly post-marketing studies or clinical trials and surveillance to 
monitor the safety or efficacy of the product. We must also comply with requirements concerning advertising and 
promotion for any of our product candidates for which we obtain marketing approval. Promotional communications with 
respect to prescription drugs are subject to a variety of legal and regulatory restrictions and must be consistent with the 
information in the product’s approved labeling. Thus, we will not be able to promote any products we develop for 
indications or uses for which they are not approved. In addition, manufacturers of approved products and those 
manufacturers’ facilities are required to ensure that quality control and manufacturing procedures conform to cGMP, 
which include requirements relating to quality control and quality assurance as well as the corresponding maintenance of 
records and documentation and reporting requirements. We and our contract manufacturers could be subject to periodic 
unannounced inspections by the FDA to monitor and ensure compliance with cGMP. 

Accordingly, to the extent we receive marketing approval for one or more of our product candidates, we and our 
contract manufacturers will continue to expend time, money and effort in all areas of regulatory compliance, including 
manufacturing, production, product surveillance and quality control. If we are not able to comply with post-approval 
regulatory requirements, we could have marketing approval for any of our products withdrawn by regulatory authorities 
and our ability to market any future products could be limited, which could adversely affect our ability to achieve or 
sustain profitability. Thus, the cost of compliance with post-approval regulations may have a negative effect on our 
operating results and financial condition. 

Any product candidate for which we obtain marketing approval will be subject to strict enforcement of post-

marketing requirements and we could be subject to substantial penalties, including withdrawal of our products from 
the market, if we fail to comply with all regulatory requirements or if we experience unanticipated problems with our 
products, when and if any of them are approved. 

Any product candidate for which we obtain marketing approval, along with the manufacturing processes, post-
approval clinical data, labeling, advertising and promotional activities for such product, will be subject to continual 
requirements of and review by the FDA and other regulatory authorities. These requirements include, but are not limited 
to, restrictions governing promotion of an approved product, submissions of safety and other post-marketing information 
and reports, registration and listing requirements, cGMP requirements relating to manufacturing, quality control, quality 
assurance and corresponding maintenance of records and documents, and requirements regarding the distribution of 
samples to physicians and recordkeeping. 

The FDA closely regulates compliance with all requirements governing prescription drug products, including 
requirements pertaining to marketing and promotion of drugs in accordance with the provisions of the approved labeling 
and manufacturing of products in accordance with cGMP requirements. Violations of such requirements may lead to 
investigations alleging violations of the Food, Drug and Cosmetic Act and other statutes, including the False Claims Act 
and other federal and state health care fraud and abuse laws as well as state consumer protection laws. Our failure to 
comply with all regulatory requirements, and later discovery of previously unknown adverse events or other problems 
with our products, manufacturers or manufacturing processes, may yield various results, including: 

• 

• 

• 

• 

litigation involving patients taking our products; 

restrictions on such products, manufacturers or manufacturing processes; 

restrictions on the labeling or marketing of a product; 

restrictions on product distribution or use; 

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• 

• 

• 

• 

• 

• 

• 

• 

• 

• 

• 

• 

requirements to conduct post-marketing studies or clinical trials; 

warning or untitled letters; 

withdrawal of the products from the market; 

refusal to approve pending applications or supplements to approved applications that we submit; 

recall of products; 

fines, restitution or disgorgement of profits or revenues; 

suspension or withdrawal of marketing approvals; 

damage to relationships with any potential collaborators; 

unfavorable press coverage and damage to our reputation; 

refusal to permit the import or export of our products; 

product seizure; or 

injunctions or the imposition of civil or criminal penalties. 

Our noncompliance, or noncompliance by any future licensee or collaborator, with regulatory requirements 
relating to safety monitoring or pharmacovigilance, to the development of products for the pediatric population or to the 
protection of personal information can lead to significant penalties and sanctions. 

Fast track designation by the FDA may not actually lead to a faster development or regulatory review or 

approval process. 

If a drug is intended for the treatment of a serious or life threatening condition and the drug demonstrates the 
potential to address unmet medical needs for the condition, the drug sponsor may apply for FDA fast track designation. 
The designation offers the sponsor opportunities for interactions with the FDA review team and the possibility of a 
rolling review for certain portions of the marketing application. The FDA has granted fast track designation for 
odevixibat for pruritus associated with PFIC, but there is no assurance that odevixibat will receive marketing approval 
from the FDA or that approval will be granted within any particular timeframe. We may also seek fast track designation 
for other current or potential future product candidates of ours. Even if the FDA grants fast track designation to one or 
more of these product candidates, we may not experience a faster development process, review or approval compared to 
conventional FDA procedures. In addition, the FDA may withdraw fast track designation that may in the future be 
granted to any of our product candidates if it believes that the designation is no longer supported by data from our 
clinical development program for that product candidate. Fast track designation alone does not guarantee qualification 
for the FDA’s priority review procedures. 

Priority review designation by the FDA may not lead to a faster regulatory review or approval process and, in 

any event, does not assure FDA approval. 

If the FDA determines that a product candidate intended to treat a serious disease, if approved, would provide a 

significant improvement in safety or effectiveness of the treatment of the disease, the FDA may designate the drug 
application for that product candidate for priority review. A priority review designation means that the goal for the FDA 
to review the marketing application is six months from the date of NDA acceptance for filing, rather than the standard 
review period of ten months from the date of NDA acceptance for filing. A priority review designation does not 

77 

necessarily mean a faster regulatory review process or necessarily confer any advantage with respect to approval 
compared to conventional FDA procedures. Receiving a priority review designation from the FDA does not guarantee 
approval of the drug application within the six-month review cycle or any time thereafter. 

Our relationships with customers, healthcare providers and professionals and third-party payors will be subject 

to applicable anti-kickback, fraud and abuse and other healthcare laws and regulations, which could expose us to 
criminal sanctions, civil penalties, contractual damages, reputational harm and diminished profits and future 
earnings. 

Healthcare providers, physicians and third-party payors play a primary role in the recommendation and 

prescription of any product candidate, including odevixibat, A3907 or A2342, for which we obtain marketing approval. 
Our future arrangements with customers, healthcare providers and professionals, and third-party payors may expose us 
to broadly applicable federal and state fraud and abuse and other healthcare laws and regulations that may constrain the 
business or financial arrangements and relationships through which we market, sell or distribute any product candidate 
for which we obtain marketing approval. 

The federal anti-kickback statute prohibits, among other things, persons from knowingly and willfully soliciting, 

offering, receiving or paying remuneration, directly or indirectly, in cash or in kind, to induce or reward either the 
referral of an individual for, or the purchase, order or recommendation of, any good or service, for which payment may 
be made, in whole or in part, under a federal healthcare program such as Medicare and Medicaid. This statute has been 
broadly interpreted to apply to manufacturer arrangements with prescribers, purchasers and pharmacy benefit managers, 
among others. Several other countries, including the United Kingdom, have enacted similar anti-kickback laws and 
regulations. 

The federal False Claims Act imposes civil penalties, and provides for civil whistleblower or qui tam actions, 
against individuals or entities for knowingly presenting, or causing to be presented, to the federal government, claims for 
payment that are false or fraudulent or making a false statement to avoid, decrease or conceal an obligation to pay money 
to the federal government. Both the government and qui tam relators have brought False Claims Act actions against 
pharmaceutical companies on the theory that their practices have caused false claims to be submitted to the government. 

The federal Health Insurance Portability and Accountability Act of 1996, or HIPAA, imposes criminal and civil 

liability for executing a scheme to defraud any healthcare benefit program or for knowingly and willfully falsifying, 
concealing or covering up a material fact or making any materially false statement in connection with the delivery of or 
payment for healthcare benefits, items or services. HIPAA, as amended by the Health Information Technology for 
Economic and Clinical Health Act, or HITECH Act, and its implementing regulations, also imposes obligations, 
including mandatory contractual terms, with respect to safeguarding the privacy, security and transmission of 
individually identifiable health information. 

The federal Physician Payments Sunshine Act requirements under the Patient Protection and Affordable Care Act 

of 2010, as amended by the Health Care and Education Reconciliation Act of 2010, referred to together as the 
Affordable Care Act, require manufacturers of FDA-approved drugs, devices, biologics and medical supplies covered by 
Medicare or Medicaid to report to the Department of Health and Human Services information related to payments and 
other transfers of value made to or at the request of covered recipients, such as physicians and teaching hospitals, and 
physician ownership and investment interests in such manufacturers. Among other payments, the law requires payments 
made to physicians and teaching hospitals for clinical trials be disclosed. 

Analogous state laws and regulations, such as state anti-kickback and false claims laws, may apply to sales or 

marketing arrangements and claims involving healthcare items or services reimbursed by nongovernmental third-party 
payors, including private insurers. Some state laws require pharmaceutical companies to comply with the pharmaceutical 
industry’s voluntary compliance guidelines, or the relevant compliance guidance promulgated by the federal 
government, in addition to requiring drug manufacturers to report information related to payments to physicians and 
other health care providers or marketing expenditures to the extent that those laws impose requirements that are more 
stringent than the Physician Payments Sunshine Act. State and foreign laws also govern the privacy and security of 

78 

health information in some circumstances, many of which differ from each other in significant ways and often are not 
preempted by HIPAA, thus complicating compliance efforts. 

Efforts to ensure that our business arrangements with third parties will comply with applicable healthcare laws and 

regulations will involve substantial costs. It is possible that governmental authorities will conclude that our business 
practices may not comply with current or future statutes, regulations or case law involving applicable fraud and abuse or 
other healthcare laws and regulations. If our operations are found to be in violation of any of these laws or any other 
governmental regulations that may apply, we may be subject to significant civil, criminal and administrative penalties, 
damages, fines, exclusion from government funded healthcare programs, such as Medicare and Medicaid, and the 
curtailment or restructuring of our operations. Violation of certain of these laws could also result in exclusion, 
suspension and debarment from government funded healthcare programs. Exclusion, suspension or debarment would 
significantly impact our ability to commercialize, sell or distribute any product candidate for which we obtain regulatory 
approval. If any of the physicians or other providers or entities with whom we expect to do business are found to be not 
in compliance with applicable laws, they may be subject to criminal, civil or administrative sanctions, including 
exclusions from government funded healthcare programs. 

Legislation may increase the difficulty and cost for us to obtain marketing approval of and commercialize our 

product candidates and affect the prices we may obtain for any product that receives marketing approval. 

In the United States and in some other jurisdictions, there have been a number of legislative and regulatory 
changes and proposed changes regarding the healthcare system that could prevent or delay marketing approval of 
odevixibat, A3907, A2342 or any potential future product candidates of ours, restrict or regulate post-approval activities, 
or affect our ability to profitably sell any product candidates, including odevixibat A3907, or A2342, for which we 
obtain marketing approval. In addition, increased scrutiny by the U.S. Congress of the FDA’s approval process may 
significantly delay or prevent marketing approval, as well as subject us to more stringent product labeling and post-
marketing testing and other requirements. 

In the United States, the Medicare Prescription Drug, Improvement, and Modernization Act of 2003, or the 
Medicare Modernization Act, changed the way Medicare covers and pays for pharmaceutical products. The legislation 
expanded Medicare coverage for drug purchases by the elderly and introduced a new reimbursement methodology based 
on average sales prices for physician administered drugs. In addition, this legislation provided authority for limiting the 
number of drugs that will be covered in any therapeutic class. Cost reduction initiatives and other provisions of this 
legislation could decrease the coverage and price that we receive for any approved products. While the Medicare 
Modernization Act applies only to drug benefits for Medicare beneficiaries, private payors often follow Medicare 
coverage policy and payment limitations in setting their own reimbursement rates. Therefore, any reduction in 
reimbursement that results from the Medicare Modernization Act may result in a similar reduction in payments from 
private payors. 

In March 2010, the Affordable Care Act, or ACA, became law in the United States. The ACA is a sweeping law 

intended to broaden access to health insurance, reduce or constrain the growth of healthcare spending, enhance remedies 
against fraud and abuse, add new transparency requirements for health care and health insurance industries, impose new 
taxes and fees on the health industry and impose additional health policy reforms. Congress has expressed an intention to 
repeal or repeal and replace the ACA, and as a result certain sections of the ACA have not been fully implemented or 
effectively repealed. The uncertainty around the future of the ACA, and in particular the impact to reimbursement levels, 
may lead to uncertainty or delay in the purchasing decisions of our customers, which may in turn negatively impact our 
product sales. If there are not adequate reimbursement levels, our business and results of operations could be adversely 
affected. Similarly, there are a number of state and local legislative and regulatory efforts related to drug pricing, 
including a drug price transparency law in Vermont that applies to pharmaceutical manufacturers, that may have an 
impact on our business. 

In addition, the Drug Supply Chain Security Act imposes new obligations on manufacturers of pharmaceutical 
products related to product tracking and tracing. Legislative and regulatory proposals have been made to expand post-
approval requirements and restrict sales and promotional activities for pharmaceutical products. We are unsure whether 

79 

additional legislative changes will be enacted, or whether the current regulations, guidance or interpretations will be 
changed, or whether such changes will have any impact on our business. 

In the European Union, similar political, economic and regulatory developments may affect our ability to 
profitably commercialize our products. In addition to continuing pressure on prices and cost containment measures, 
legislative developments at the European Union or E.U. member state level may result in significant additional 
requirements or obstacles that may increase our operating costs. 

We expect that the ACA, as well as other healthcare reform measures that may be adopted in the future, may result 

in more rigorous coverage criteria and lower reimbursement, and additional downward pressure on the price that we 
receive for any approved product. Any reduction in reimbursement from Medicare or other government-funded 
programs may result in a similar reduction in payments from private payors. The implementation of cost containment 
measures or other healthcare reforms could result in reduced demand for our product candidates or additional pricing 
pressures, and may prevent us from being able to generate revenue, attain profitability or commercialize our drugs. 

We are subject to anti-corruption laws, as well as export control laws, data protection laws, customs laws, 
sanctions laws and other laws governing our operations. If we fail to comply with these laws, we could be subject to 
civil or criminal penalties, other remedial measures and legal expenses, which could adversely affect our business, 
results of operations and financial condition. 

Our operations are subject to anti-corruption laws, including the U.S. Foreign Corrupt Practices Act, or the FCPA, 

and other anti-corruption laws that apply in countries where we do business and may do business in the future. The 
FCPA and these other laws generally prohibit us, our officers, and our employees and intermediaries from bribing, being 
bribed or making other prohibited payments to government officials or other persons to obtain or retain business or gain 
some other business advantage. We may in the future operate in jurisdictions that pose a high risk of potential FCPA 
violations and we may participate in collaborations and relationships with third parties whose actions could potentially 
subject us to liability under the FCPA or local anti-corruption laws. In addition, we cannot predict the nature, scope or 
effect of future regulatory requirements to which our international operations might be subject or the manner in which 
existing laws might be administered or interpreted. 

We are also subject to other laws and regulations governing our international operations, including regulations 

administered by the government of the United States and authorities in the European Union, including applicable export 
control regulations, economic sanctions on countries and persons, customs requirements and currency exchange 
regulations, collectively referred to as the Trade Control laws. In addition, various statutes and rules in Europe and 
elsewhere around the world regulate privacy and data protection, which affect our collection, use, storage, and transfer of 
information both abroad and in the United States. New laws and regulations are periodically being enacted in this area, 
which remains in a state of flux. Monitoring and complying with these laws requires substantial financial resources.  In 
particular, the European Union’s General Data Protection Regulation, or GDPR, took effect in May 2018, and will 
require us to meet new and more stringent requirements regarding the handling of personal data about European Union 
residents. The GDPR is a complex law and the regulatory guidance is still evolving.  Furthermore, many of the countries 
within the European Union are still in the process of drafting supplementary data protection legislation in key fields 
where the GDPR allows for national variation, including the fields of clinical study and other health-related information.  
Failure to meet GDPR requirements could result in penalties of up to 4% of our worldwide revenue. While we have 
taken steps to comply with the GDPR, including reviewing our security procedures, updating our website, revising our 
clinical study informed consent forms, and entering into data processing agreements with relevant contractors, we cannot 
assure you that our efforts to remain in compliance will be fully successful. 

There is no assurance that we will be completely effective in ensuring our compliance with all applicable anti-

corruption laws, including the FCPA or other legal requirements, including Trade Control laws. If we are not in 
compliance with the FCPA and other anti-corruption laws or Trade Control laws, we may be subject to criminal and civil 
penalties, disgorgement and other sanctions and remedial measures, and legal expenses, which could have an adverse 
impact on our business, financial condition, results of operations and liquidity. Likewise, any investigation of any 

80 

potential violations of the FCPA, other anti-corruption laws or Trade Control laws by U.S. or other authorities could also 
have an adverse impact on our reputation, our business, results of operations and financial condition. 

We rely significantly on information technology and any failure, inadequacy, interruption or security lapse of 

that technology or loss of data, including any cyber security incidents, could compromise sensitive information 
related to our business, prevent us from accessing critical information or expose us to liability which could harm our 
ability to operate our business effectively and adversely affect our business and reputation. 

In the ordinary course of our business, we, our contract research organizations and other third parties on which we 

rely collect and store sensitive data, including legally protected patient health information, personally identifiable 
information about our employees, intellectual property, and proprietary business information. We manage and maintain 
our applications and data utilizing on-site systems. These applications and data encompass a wide variety of business-
critical information including research and development information and business and financial information. 

The secure processing, storage, maintenance and transmission of this critical information is vital to our operations 
and business strategy. Despite the implementation of security measures, our internal computer systems and those of third 
parties with which we contract are vulnerable to damage from cyber-attacks, computer viruses, breaches unauthorized 
access, interruptions due to employee error or malfeasance or other disruptions, or damage from natural disasters, 
terrorism, war and telecommunication and electrical failures. Any such event could compromise our networks and the 
information stored there could be accessed by unauthorized parties, publicly disclosed, lost or stolen. We have measures 
in place that are designed to detect and respond to such security incidents and breaches of privacy and security mandates. 
Any such access, disclosure or other loss of information could result in legal claims or proceedings, liability under laws 
that protect the privacy of personal information, government enforcement actions and regulatory penalties. Unauthorized 
access, loss or dissemination could also disrupt our operations, including our ability to conduct research, development 
and commercialization activities, process and prepare company financial information, manage various general and 
administrative aspects of our business and damage our reputation, in addition to possibly requiring substantial 
expenditures of resources to remedy, any of which could adversely affect our business. The loss of clinical trial data 
could result in delays in our regulatory approval efforts and significantly increase our costs to recover or reproduce the 
data. In addition, there can be no assurance that we will promptly detect any such disruption or security breach, if at all. 
To the extent that any disruption or security breach were to result in a loss of, or damage to, our data or applications, or 
inappropriate disclosure of confidential or proprietary information, we could incur liability and our product research, 
development and commercialization efforts could be delayed. 

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Risks Related to Our Business Operations, Employee Matters and Managing Growth 

If we fail to maintain an effective system of internal control over financial reporting, we may not be able to 

accurately report our financial results or prevent fraud. As a result, stockholders could lose confidence in our 
financial and other public reporting, which would harm our business and the trading price of our common stock.  

Effective internal control over financial reporting is necessary for us to provide reliable financial reports and, 

together with adequate disclosure controls and procedures, is designed to prevent fraud. Pursuant to Section 404 of the 
Sarbanes-Oxley Act, or Section 404, we are required to furnish a report by our management on the effectiveness of our 
internal control over financial reporting. As a non-accelerated filer, we are not required this year to obtain an attestation 
report on internal control over financial reporting from our independent registered public accounting firm. Ensuring that 
we have adequate internal financial and accounting controls and procedures in place so that we can produce accurate 
financial statements on a timely basis is a costly and time-consuming effort that will need to be evaluated frequently. 
Any failure to implement required new or improved controls, or difficulties encountered in their implementation, could 
cause us to fail to meet our reporting obligations. In addition, any testing by us conducted in connection with 
Section 404 or any future testing by our independent registered public accounting firm may reveal deficiencies in our 
internal control over financial reporting that are deemed to be material weaknesses or that may require prospective or 
retroactive changes to our consolidated financial statements or identify other areas for further attention or improvement. 
Inferior internal controls could also cause investors to lose confidence in our reported financial information, which could 
have a negative effect on the trading price of our common stock. While our management concluded that our internal 
control over financial reporting is effective as of December 31, 2020, there is no assurance that we or our independent 
registered public accounting firm will not identify material weaknesses in the future, which could have a material 
adverse impact on our business and the trading price of our common stock.  

Our future success depends on our ability to retain our chief executive officer and other key executives and to 

attract, retain and motivate qualified personnel. 

We are highly dependent on Ron Cooper, our President and Chief Executive Officer, and other principal members 

of our management and scientific teams. Although we have formal employment agreements with each of our executive 
officers, these agreements do not prevent our executives from terminating their employment with us at any time. We do 
not maintain “key person” insurance on any of our executive officers. The unplanned loss of the services of any of these 
persons could materially impact the achievement of our research, development and commercialization objectives. 

Recruiting and retaining qualified scientific, clinical, manufacturing and sales and marketing personnel, including 

in the United States and Sweden, will also be critical to our success. We may not be able to attract and retain these 
personnel on acceptable terms given the competition among numerous biotechnology and pharmaceutical companies for 
similar personnel. We also experience competition for the hiring of scientific and clinical personnel from universities 
and research institutions. In addition, we rely on consultants and advisors, including scientific and clinical advisors, to 
assist us in formulating our research and development and commercialization strategy. Our consultants and advisors may 
be employed by employers other than us and may have commitments under consulting or advisory contracts with other 
entities that may limit their availability to us. 

We expect to expand our capabilities and, as a result, we may encounter difficulties in managing our growth, 

which could disrupt our operations. 

We expect to experience significant growth in the number of our employees and the scope of our operations, 
particularly in the areas of drug development, regulatory affairs, finance and administration and sales and marketing. To 
manage our anticipated future growth, we must continue to implement and improve our managerial, operational and 
financial systems, expand our facilities and continue to recruit and train additional qualified personnel. Due to our 
limited financial resources and the limited experience of our management team in managing a company with such 
anticipated growth, we may not be able to effectively manage the expansion of our operations or recruit and train 
additional qualified personnel. The physical expansion of our operations may lead to significant costs and may divert our 

82 

management and business development resources. Any inability to manage growth could delay the execution of our 
business plans or disrupt our operations. 

We incur significant costs and demands as a result of operating as a public company. 

We incur significant legal, accounting and other expenses to meet our obligations as a publicly traded company. In 

addition, the Sarbanes-Oxley Act, the Dodd-Frank Act, the listing requirements of the Nasdaq Stock Market and other 
applicable securities rules and regulations impose various requirements on public companies, including establishment 
and maintenance of effective disclosure and financial controls and corporate governance practices. Stockholder activism, 
the current political environment and the current high level of government intervention and regulatory reform may lead 
to substantial new regulations and disclosure obligations, which may lead to additional compliance costs and impact the 
manner in which we operate our business in ways that are not currently anticipated. Our management and other 
personnel will need to devote a substantial amount of time to these compliance initiatives. Moreover, these rules and 
regulations will increase our legal and financial compliance costs and will make some activities more time-consuming 
and costly. For example, these rules and regulations may make it difficult and expensive for us to maintain director and 
officer liability insurance coverage. As a result, it may be more difficult for us to attract and retain qualified individuals 
to serve on our board of directors or as our executive officers, which may adversely affect investor confidence in us and 
could cause our business or stock price to suffer. 

We are exposed to risks related to currency exchange rates. 

We conduct a significant portion of our operations outside of the United States. Because our consolidated financial 

statements are presented in U.S. dollars, changes in currency exchange rates have had and could have in the future a 
significant effect on our operating results when our operating results are translated into U.S. dollars. Exchange rate 
fluctuations between local currencies, the euro and the dollar create risk in several ways, including the following: 
weakening of the dollar may increase the cost of overseas research and development expenses and the cost of sourced 
product components outside the United States; strengthening of the dollar may decrease the value of our revenues 
denominated in other currencies; the exchange rates on-nondollar transactions and cash deposits can distort our financial 
results; and commercial pricing and profit margins may be affected. 

Our present and potential future international operations may expose us to business, political, operational and 

financial risks associated with doing business outside of the United States. 

Our business is subject to risks associated with conducting business internationally. Some of our operations are 

located outside of the United States. Doing business internationally involves a number of risks, including but not limited 
to: 

•  multiple, conflicting and changing laws and regulations such as privacy regulations, tax laws, export and 

import restrictions, employment laws, regulatory requirements, and other governmental approvals, permits 
and licenses; 

• 

• 

• 

• 

• 

failure by us to obtain and maintain regulatory approvals for the use of our products in various countries; 

rejection or qualification of foreign clinical trial data by the competent authorities of other countries; 

additional potentially relevant third-party patent and other intellectual property rights that may be necessary to 
develop and commercialize our products and drug candidates; 

complexities and difficulties in obtaining, maintaining, enforcing and defending our patent and other 
intellectual property rights; 

difficulties in staffing and managing foreign operations; 

83 

• 

• 

• 

• 

• 

• 

• 

complexities associated with managing multiple payor reimbursement regimes, government payors or patient 
self-pay systems; 

limits in our ability to penetrate international markets; 

restrictions on traveling outside the United States; 

financial risks, such as longer payment cycles, difficulty collecting accounts receivable, the impact of local 
and regional financial crises on demand and payment for our products and exposure to foreign currency 
exchange rate fluctuations; 

natural disasters, pandemics, political and economic instability, including wars, terrorism and political unrest, 
outbreak of disease, boycotts, curtailment of trade and other business restrictions, implementation of tariffs; 

certain expenses including, among others, expenses for travel, translation and insurance; and 

regulatory and compliance risks that relate to anti-corruption compliance and record-keeping that may fall 
within the purview of the U.S. Foreign Corrupt Practices Act, its accounting provisions or its anti-bribery 
provisions or provisions of anti-corruption or anti-bribery laws in other countries. 

Any of these factors could harm our ongoing international clinical operations and supply chain, as well as any 

future international expansion  

The United Kingdom’s withdrawal from the European Union could adversely impact operations, make it more 
difficult for us to do business in Europe and impose additional regulatory costs and challenges in securing approval 
of our product candidate. 

On June 23, 2016, the electorate in the United Kingdom voted in favor of leaving the European Union, commonly 
referred to as “Brexit.” Thereafter, on March 29, 2017, the country formally notified the European Union of its intention 
to withdraw pursuant to Article 50 of the Lisbon Treaty. The withdrawal of the United Kingdom from the European 
Union took effect on January 31, 2020, the effective date of the withdrawal agreement, with a transition period that 
ended on December 31, 2020. The United Kingdom entered into a trade agreement known as the Trade and Cooperation 
Agreement, which is provisionally applicable as of January 1, 2021 but has not yet been ratified by the European 
Parliament.  The United Kingdom’s withdrawal could lead to a period of considerable uncertainty and volatility, 
particularly in relation to United Kingdom financial and banking markets. Weakening of economic conditions or 
economic uncertainties tend to harm our business, and if such conditions emerge in the United Kingdom or in the rest of 
Europe, it may have a material adverse effect on operations and sales. 

Currency exchange rates in the pound sterling and the euro with respect to each other and the U.S. dollar have 
already been adversely affected by Brexit and that may continue to be the case. In addition, the United Kingdom and the 
European Union have entered into a new Trade and Cooperation Agreement, which is designed to implement a new 
structure for trade agreements involving United Kingdom and the European Union, which may result in increased trade 
barriers and could make doing business in Europe more difficult. 

We may also face new and additional regulatory costs and challenges from the new Trade and Cooperation 

Agreement that could have a material adverse effect on operations. Since a significant proportion of the regulatory 
framework in the United Kingdom was, prior to Brexit, derived from European Union directives and regulations, the 
new post-Brexit framework could materially impact the regulatory regime with respect to the approval of our product 
candidates in the United Kingdom or the European Union. We are currently evaluating the potential impacts on our 
business of the new Trade and Cooperation Agreement, including provisions relating to the manufacturing of medicinal 
products. Any delays to manufacturing or delays in obtaining, or an inability to obtain, any marketing approvals, as a 
result of Brexit, the Trade and Cooperation Agreement, or otherwise, would prevent us from commercializing product 
candidates in the United Kingdom and/or the European Union and restrict our ability to generate revenue and achieve 

84 

and sustain profitability. If any of these outcomes occur, we may be forced to restrict or delay efforts to seek regulatory 
approval in the United Kingdom and/or European Union for our product candidates, which could significantly and 
materially harm our business. 

Risks Related to Our Common Stock 

Our stock price is expected to continue to be volatile, and the market price of our common stock may drop. 

The market price of our common stock could continue to be subject to significant fluctuations. Market prices for 

securities of clinical-stage pharmaceutical, biotechnology and other life sciences companies have historically been 
particularly volatile. Some of the factors that may cause the market price of our common stock to fluctuate include: 

• 

• 

• 

• 

• 

• 

• 

• 

• 

• 

• 

• 

• 

• 

• 

• 

the progress, scope, cost, duration or results of our current and any future clinical trials of our product 
candidates; 

the timing and success of submission, acceptance and approval of regulatory filings; 

our ability to obtain regulatory approvals for our product candidates, delays or failures to obtain such 
approvals and any restrictions, limitations or warnings in the label of any approved product candidates; 

failure of any of our product candidates, if approved, to achieve commercial success; 

issues in manufacturing our approved products, if any, or product candidates; 

the entry into, or termination of, licensing, collaboration or similar agreements, or other key agreements, and 
the agreement terms; 

the initiation of, material developments in, or conclusion of litigation to enforce or defend any of our 
intellectual property rights or defend against the intellectual property rights of others; 

announcements by commercial partners or competitors of new commercial products, clinical progress or the 
lack thereof, significant contracts, commercial relationships or capital commitments; 

adverse publicity relating to the markets in which we compete, including with respect to other products and 
potential products in such markets; 

the introduction of technological innovations or new therapies that compete with our product candidates or 
products, if any; 

the loss of key employees; 

changes in estimates or recommendations by securities analysts, if any, who cover our common stock; 

low trading volume; 

general and industry-specific economic conditions that may affect our research and development 
expenditures; 

changes in the structure of health care payment systems; 

failure to maintain compliance with listing requirements of The Nasdaq Capital Market; and 

85 

• 

period-to-period fluctuations in our financial results. 

Moreover, the stock markets in general have experienced substantial volatility that has often been unrelated to the 

operating performance of individual companies. These broad market fluctuations may also adversely affect the trading 
price of our common stock. 

In the past, following periods of volatility in the market price of a company’s securities, stockholders have often 

instituted class action securities litigation against those companies. Such litigation, if instituted, could result in 
substantial costs and diversion of management attention and resources, which could significantly harm our profitability 
and reputation. 

We do not anticipate that we will pay any cash dividends in the foreseeable future. 

We currently expect to retain our future earnings to fund the development and growth of our business. As a result, 

capital appreciation, if any, of our common stock will be the sole source of gain, if any, of our stockholders for the 
foreseeable future. 

Provisions in our corporate charter documents and under Delaware law could make an acquisition of us, which 

may be beneficial to our stockholders, more difficult and may prevent attempts by our stockholders to replace or 
remove our current management. 

Provisions in our certificate of incorporation and bylaws may discourage, delay or prevent a merger, acquisition or 

other change in control of us that stockholders may consider favorable, including transactions in which stockholders 
might otherwise receive a premium for their shares. These provisions could also limit the price that investors might be 
willing to pay in the future for shares of our common stock, thereby depressing the market price of our common stock. In 
addition, these provisions may frustrate or prevent any attempts by our stockholders to replace or remove our current 
management by making it more difficult for stockholders to replace members of our board of directors. Because our 
board of directors is responsible for appointing the members of our management team, these provisions could in turn 
affect any attempt by our stockholders to replace current members of our management team. 

Among others, these provisions: 

• 

• 

• 

• 

• 

• 

• 

• 

establish a classified board of directors such that not all members of the board are elected at one time; 

allow the authorized number of our directors to be changed only by resolution of our board of directors; 

limit the manner in which stockholders can remove directors from the board of directors; 

establish advance notice requirements for stockholder proposals that can be acted on at stockholder meetings 
and nominations to our board of directors; 

require that stockholder actions must be effected at a duly called stockholder meeting and prohibit actions by 
our stockholders by written consent; 

limit who may call stockholder meetings; 

authorize our board of directors to issue preferred stock without stockholder approval, which could be used 
to institute a stockholder rights plan or “poison pill” that would work to dilute the stock ownership of a 
potential hostile acquirer, effectively preventing acquisitions that have not been approved by our board of 
directors; and 

require the approval of the holders of at least 75% of the votes that all of our stockholders would be entitled 
to cast to amend or repeal certain provisions of our charter or bylaws. 

86 

In addition, because we are incorporated in Delaware, we are governed by the provisions of Section 203 of the 

Delaware General Corporation Law, which generally prohibits a person who owns 15% or more of our outstanding 
voting stock from merging or combining with us for a period of three years after the date of the transaction in which the 
person acquired 15% or more of our outstanding voting stock, unless the merger or combination is approved in a 
prescribed manner. 

Item 1B. 

UNRESOLVED STAFF COMMENTS 

None. 

Item 2. 

PROPERTIES 

Our portfolio of commercial real estate leases consists of office space for our corporate headquarters in Boston, 

Massachusetts and for administrative space in Göteborg, Sweden, both of which are accounted for as operating leases.  

Item 3. 

LEGAL PROCEEDINGS 

We are not currently a party to any material legal proceedings. 

Item 4. 

MINE SAFETY DISCLOSURES 

Not applicable. 

87 

 
 
 
 
 
Item 5. 

MARKET FOR REGISTRANT’S COMMON EQUITY, RELATED STOCKHOLDER MATTERS 
AND ISSUER PURCHASES OF EQUITY SECURITIES 

PART II 

Market Information 

Our common stock is traded on The Nasdaq Capital Market under the symbol “ALBO”. 

Stockholders 

As of January 31, 2021, we had 19,113,291 outstanding shares of common stock and no outstanding shares of 

preferred stock. As of January 31, 2021, there were approximately 24 holders of record of our common stock. 

Unregistered Sales of Securities 

Not applicable. 

Issuer Purchases of Equity Securities 

We did not purchase any of our registered equity securities during the year ended December 31, 2020. 

Item 6. 

SELECTED FINANCIAL DATA 

Not required for smaller reporting companies. 

88 

 
 
Item 7. 

MANAGEMENT’S DISCUSSION AND ANALYSIS OF FINANCIAL CONDITION AND 
RESULTS OF OPERATIONS 

You should read the following discussion and analysis of our financial condition and results of operations together 

with our consolidated financial statements and the related notes included elsewhere in this Annual Report on 
Form 10-K. Some of the information contained in this discussion and analysis or set forth elsewhere in this Form 10-K, 
including information with respect to our plans and strategy for our business and related financing, includes forward-
looking statements that involve risks and uncertainties. You should read the “Risk Factors” section of this Annual 
Report on Form 10-K (see Part I, Item 1A) for a discussion of important factors that could cause actual results to differ 
materially from the results described in or implied by any forward-looking statement contained in the following 
discussion and analysis. 

Since inception, we have incurred significant operating losses. As of December 31, 2020, we had an accumulated 

deficit of $266.8 million. We expect to continue to incur significant expenses and increasing operating losses as we 
continue our development of, and seek marketing approvals for, our product candidates, prepare for and begin the 
commercialization of any approved products, and add infrastructure and personnel to support our product development 
efforts and operations as a public company in the United States. 

As a clinical-stage company, our revenues, expenses and results of operations are likely to fluctuate significantly 

from quarter to quarter and year to year. We believe that period-to-period comparisons of our results of operations 
should not be relied upon as indicative of our future performance. 

As of December 31, 2020, we had approximately $251.3 million in cash and cash equivalents. 

Financial Operations Overview 

The following discussion sets forth certain components of our consolidated statements of operations as well as 

factors that impact those items. 

Revenue 

We generate revenue from the receipt of royalty revenue, upfront or license fees and milestone payments. License 
agreements with commercial partners generally include nonrefundable upfront fees and milestone payments, the receipt 
of which is dependent upon the achievement of specified development, regulatory or commercial milestone events, as 
well as royalties on product sales of licensed products, if and when such product sales occur, and payments for 
pharmaceutical ingredient or related procurement services. For these agreements, management applies judgment in the 
allocation of total agreement consideration to the performance obligations on a reliable basis that reasonably reflects the 
selling prices that might be expected to be achieved in stand-alone transactions. For additional information about our 
revenue recognition, refer to Note 1 to our consolidated financial statements included in this Annual Report on 
Form 10-K.  

For the years ended December 31, 2020 and 2019, we recognized revenue of $8.3 million and $9.6 million, 
respectively, related to our agreement with EA Pharma. We expect that any future revenue recognized under our license 
agreement with EA Pharma will fluctuate from quarter to quarter and year to year as a result of royalties for the period 
from EA Pharma, as well as the uncertain timing of future milestone payments, if any. 

Operating Expenses 

Research and Development Expenses 

Research and development expenses consist primarily of personnel costs (including salaries, benefits and stock-
based compensation) for employees in research and development functions, costs associated with nonclinical and clinical 
development services, including clinical trials and related manufacturing costs, third-party contract research 

89 

organizations, or CROs, and related services and other outside costs, including fees for third-party professional services 
such as consultants. Our nonclinical studies and clinical studies are performed by CROs. We expect to continue to focus 
our research and development efforts on nonclinical studies and clinical trials of our product candidates. As a result, we 
expect our research and development expenses to continue to increase for the foreseeable future. 

Our direct research and development expenses are tracked on a program-by-program basis and consist primarily of 

external costs such as fees paid to CROs and others in connection with our nonclinical and clinical development 
activities and related manufacturing. We do not allocate employee costs or facility expenses, including depreciation or 
other indirect costs, to specific product development programs because these costs are deployed across multiple product 
development programs and, as such, are not separately classified. 

Successful development of our current and potential future product candidates is highly uncertain. Completion 

dates and costs for our programs can vary significantly by product candidate and are difficult to predict. As a result, we 
cannot estimate with any degree of certainty the costs we will incur in connection with development of any of our 
product candidates. We anticipate we will make determinations as to which programs and product candidates to pursue 
and how much funding to direct to each program and product candidate on an ongoing basis in response to the results of 
ongoing and future clinical trials, our ability to enter into licensing, collaboration and similar arrangements with respect 
to current or potential future product candidates, the success of research and development programs and our assessments 
of commercial potential. 

General and Administrative Expenses 

General and administrative expenses consist primarily of personnel costs (including salaries, benefits and stock-

based compensation) for our executive, finance and other administrative employees. In addition, general and 
administrative expenses include fees for third-party professional services, including consulting, information technology, 
legal and accounting services and other corporate expenses and allocated overhead. 

Other Operating (Income), Expense 

Other operating (income), expense consists primarily of foreign currency exchange gains or losses associated with 

revaluation of intercompany loans.  

Interest Expense, net 

Interest expense, net consists primarily of non-cash interest expense recorded in connection with the sale of future 

royalties, related to sales of elobixibat in Japan in addition to interest expense associated with our note payable. In 
addition, interest expense, net includes interest income associated with our interest-bearing cash and cash equivalents. 

Critical Accounting Policies and Estimates 

Our management’s discussion and analysis of financial condition and results of operations is based on our 

consolidated financial statements, which have been prepared in accordance with United States generally accepted 
accounting principles. The preparation of these financial statements requires us to make estimates and assumptions that 
affect the reported amounts of assets, liabilities, revenues and expenses. We base our estimates and assumptions on 
historical experience and on various assumptions that we believe are reasonable under the circumstances, and we 
evaluate them on an ongoing basis. These estimates and assumptions form the basis for making judgments about the 
carrying values of assets and liabilities and the recording of revenues and expenses that are not readily apparent from 
other sources. Actual results and experiences may differ materially from these estimates and judgments. In addition, our 
reported financial condition and results of operations could vary if new accounting standards are enacted that are 
applicable to our business. 

Our significant accounting policies are described in Note 1 to our audited consolidated financial statements for 
the year ended December 31, 2020 in this Annual Report on Form 10-K. We believe that our accounting policies relating 
to revenue recognition, research and development expenses and accounting for the liability related to sale of future 

90 

 
royalties are the most critical to understanding and evaluating our reported financial results. We have identified these 
policies as critical because they both are important to the presentation of our financial condition and results of operations 
and require us to make judgments and estimates on matters that are inherently uncertain and may change in future 
periods. For more information regarding these policies, you should refer to Note 1 of our audited consolidated financial 
statements included in this Annual Report on Form 10-K. 

Revenue Recognition 

We generate revenue primarily from the receipt of upfront or license fees, milestone payments, royalties, and 
payments for pharmaceutical ingredient or related procurement services that are made pursuant to license agreements or 
related supply agreements. Substantially all of our revenue to date has been derived from our license agreement with EA 
Pharma and a now-terminated license agreement with Ferring International Center S.A., or Ferring. 

We enter into licensing agreements which are within the scope of ASC 606, under which we may exclusively 
license rights to research, develop, manufacture and commercialize our product candidates to third parties. The terms of 
these arrangements may include payment to us of one or more of the following: non-refundable, upfront license fees; 
reimbursement of certain costs; development, regulatory and commercial milestone payments; and royalties on net sales 
of licensed products.  

In determining the appropriate amount of revenue to be recognized as we fulfill our obligations under each of our 

agreements, we perform the following steps: (i) identification of the promised goods or services in the contract; 
(ii) determination of whether the promised goods or services are performance obligations, including whether they are 
distinct in the context of the contract; (iii) measurement of the transaction price, including the constraint on variable 
consideration; (iv) allocation of the transaction price to the performance obligations; and (v) recognition of revenue 
when (or as) we satisfy each performance obligation. As part of the accounting for these arrangements, we must use 
significant judgment to determine: (a) the number of performance obligations based on the determination under step 
(ii) above and (b) the transaction price under step (iii) above. We use our judgment to determine whether milestones or 
other variable consideration, except for royalties, should be included in the transaction price as described further below. 
The transaction price is allocated to each performance obligation on a relative stand-alone selling price basis, for which 
we recognize revenue as or when the performance obligations under the contract are satisfied. 

Amounts received prior to revenue recognition are recorded as deferred revenue.  

Milestone Payments  

At the inception of each arrangement that includes development milestone payments, the Company evaluates 

whether the milestones are considered probable of being achieved and estimates the amount to be included in the 
transaction price using the most likely amount method. If it is probable that a significant revenue reversal would not 
occur, the associated milestone value is included in the transaction price. Milestone payments that are not within the 
control of the Company or the licensee, such as regulatory approvals, are not considered probable of being achieved until 
those approvals are received. The Company evaluates factors such as the scientific, clinical, regulatory, commercial, and 
other risks that must be overcome to achieve the particular milestone in making this assessment. There is considerable 
judgment involved in determining whether it is probable that a significant revenue reversal would not occur. At the end 
of each subsequent reporting period, the Company reevaluates the probability of achievement of all milestones subject to 
constraint and, if necessary, adjusts its estimate of the overall transaction price. Any such adjustments are recorded on a 
cumulative catch-up basis, which would affect revenues and earnings in the period of adjustment.  

Royalties  

For arrangements that include sales-based royalties, including milestone payments based on a level of sales, and 
the license is deemed to be the predominant item to which the royalties relate, the Company recognizes revenue at the 
later of (i) when the related sales occur, or (ii) when the performance obligation to which some or all of the royalty has 
been allocated has been satisfied (or partially satisfied). 

91 

Research and Development Expenses 

Research and development expenses consist primarily of personnel costs (including salaries, benefits and stock-
based compensation) for employees in research and development functions, costs associated with nonclinical and clinical 
development services, including clinical trials and related manufacturing costs, third-party contract research 
organizations, or CROs, and related services and other outside costs, including fees for third-party professional services 
such as consultants. Our nonclinical studies and clinical studies are performed by CROs. We expect to continue to focus 
our research and development efforts on nonclinical studies and clinical trials of our product candidates. As a result, we 
expect our research and development expenses to continue to increase for the foreseeable future. 

Our direct research and development expenses are tracked on a program-by-program basis and consist primarily of 

external costs such as fees paid to CROs and others in connection with our nonclinical and clinical development 
activities and related manufacturing. We do not allocate employee costs or facility expenses, including depreciation or 
other indirect costs, to specific product development programs because these costs are deployed across multiple product 
development programs and, as such, are not separately classified. 

Successful development of our current and potential future product candidates is highly uncertain. Completion 

dates and costs for our programs can vary significantly by product candidate and are difficult to predict. As a result, we 
cannot estimate with any degree of certainty the costs we will incur in connection with development of any of our 
product candidates. We anticipate we will make determinations as to which programs and product candidates to pursue 
and how much funding to direct to each program and product candidate on an ongoing basis in response to the results of 
ongoing and future clinical trials, our ability to enter into licensing, collaboration and similar arrangements with respect 
to current or potential future product candidates, the success of research and development programs and our assessments 
of commercial potential. 

Monetization of Future Royalties  

In December 2017, we entered into a royalty interest acquisition agreement (RIAA) with HealthCare Royalty 
Partners III, L.P. (HCR) pursuant to which it sold to HCR the right to receive all royalties from sales in Japan and sales 
milestones achieved from any covered territory potentially payable to us under the Agreement, up to a specified 
maximum “cap” amount of $78.8 million, based on the funds we received from HCR. In January 2018, we received 
$44.5 million from HCR, net of certain transaction expenses, under the RIAA. On June 8, 2020, the parties entered into 
an amendment to the RIAA pursuant to which HCR agreed to pay us an additional $14.8 million, net of certain 
transactions expenses, in exchange for the elimination of the (i) $78.8 million cap amount on HCR’s rights to receive 
royalties on sales in Japan and sales milestones for elobixibat in certain other territories that may become payable by EA 
Pharma and (ii) the $15.0 million payable to us if a specified sales milestone is achieved for elobixibat in Japan. We are 
obligated to make royalty interest payments to HCR under the RIAA only to the extent it receives future Japanese 
royalties, sales milestones or other specified payments from EA Pharma. Although we sold our rights to receive royalties 
from the sales of elobixibat in Japan, as a result of its ongoing involvement in the cash flows related to these royalties, 
we will continue to account for these royalties as revenue. Upon receipt of the payments from HCR we recorded net cash 
totaling $59.3 million as a liability related to sale of future royalties (royalty obligation). The royalty obligation will be 
amortized using the effective interest rate method. 

We record estimated royalties due for the current period in accrued other expenses until the payment is received 

from EA Pharma at which time we then remit payment to HCR. In order to determine the accretion of the royalty 
obligation, we are required to estimate the total amount of future royalty payments to be received and submitted to HCR. 
The sum of these amounts less the $59.3 million proceeds we received will be recorded as interest expense over the life 
of the royalty obligation. At December 31, 2020, our estimate of total interest expense resulted in an annual effective 
interest rate of approximately 19.0%.  

We periodically assess the estimated royalty payments to HCR and to the extent such payments are greater or less 

than our initial estimates or the timing of such payments is materially different than its original estimates, we will 
prospectively adjust the accretion of interest on the royalty obligation. There are a number of factors that could 
materially affect the amount and the timing of royalty payments, most of which are not within our control. Such factors 

92 

include, but are not limited to, the rate of elobixibat prescriptions, the number of doses administered, the introduction of 
competing products, manufacturing or other delays, patent protection, adverse events that result in governmental health 
authority imposed restrictions on the use of the drug products, significant changes in foreign exchange rates as the 
royalties remitted to HCR are in U.S. dollars while sales of elobixibat are in Japanese yen, and sales never achieving 
forecasted numbers, which would result in reduced royalty payments and reduced non-cash interest expense over the life 
of the royalty obligation. To the extent future royalties result in an amount less than the liability, we are not obligated to 
fund any such shortfall. 

Results of Operations 

Years Ended December 31, 2020 and December 31, 2019 

Result of Operations 

Revenue . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .    $ 
Operating Expenses 
   Research and development  . . . . . . . . . . . . . . . . . . . . . .     
   General and administrative . . . . . . . . . . . . . . . . . . . . . .     
   Other operating (income) expense, net . . . . . . . . . . . . .     
   Total operating expenses . . . . . . . . . . . . . . . . . . . . . . . .     
Operating loss . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .     
Interest expense, net  . . . . . . . . . . . . . . . . . . . . . . . . . . . . .     
Other non-operating income . . . . . . . . . . . . . . . . . . . . . . .     
Net loss . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .    $ 

Revenue 

Year Ended 
December 31,  

2020 

2019 
(in thousands) 

Change 
$ 

 8,308   $ 

 9,636   $ 

 (1,328)

 76,777  
 42,448  
 (14,646) 
 104,579  
 (96,271) 
 (11,362) 
 -  

 (107,633)  $ 

 45,575  
 22,963  
 2,210  
 70,748  
 (61,112) 
 (5,296) 
 3,691  
 (62,717)  $ 

 31,202 
 19,485 
 (16,856)
 33,831 
 (35,159)
 (6,066)
 (3,691)
 (44,916)

Year Ended  
December 31,  

      Change 

2020 

2019 
(in thousands) 

$ 

Revenue . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .    $ 

 8,308   $ 

 9,636   $ 

 (1,328)

Revenue was $8.3 million for the year ended December 31, 2020, compared with revenue of $9.6 million for 
the year ended December 31, 2019, a decrease of $1.3 million. The decrease in revenue primarily relates to a sales-based 
milestone achieved in 2019 offset by higher sales-based royalties earned in 2020. 

Research and development expenses  

Research and development expenses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .    $ 

 76,777   $ 

 45,575   $ 

 31,202 

Research and development expenses were $76.8 million for the year ended December 31, 2020 compared with 
$45.6 million for the year ended December 31, 2019, an increase of $31.2 million. The higher research and development 
expenses for the 2020 period were principally due to personnel expenses as we continue to increase our headcount and 
program activities. The increase in program activities related primarily to odevixibat for regulatory submissions in PFIC, 

Year Ended December 31,    

2020 

2019 
(in thousands) 

Change 
$ 

93 

 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
     
     
     
 
 
     
 
   
 
   
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
     
 
     
     
     
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
     
     
     
 
 
 
the initiation of clinical trials for additional indications for biliary atresia and Alagille syndrome, and preclinical 
programs. 

The following table summarizes our principal product development programs and the out-of-pocket third-party 

expenses incurred with respect to each clinical-stage product candidate and our preclinical programs for the years ended 
December 31, 2020 and 2019. 

  Year Ended December 31,    

2020 

2019 

Change 
$ 

(in thousands) 

Direct third-party project costs: 

Odevixibat . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .     $ 
Elobixibat  . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .    
A3384  . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .    
Preclinical . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .    

Total  . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .     $ 

 42,799   $  19,605    $  23,194   
 (1,827) 
 4,753   
 2,926  
 93  
 (425) 
 518   
 1,286   
 4,049   
 5,335  
 51,153   $  28,925    $  22,228   

Other project costs(1): 

Personnel costs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .     $ 
Other costs(2) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .    

Total  . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .     $ 
Total research and development costs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .     $ 

 22,933   $  11,484    $  11,449   
 (2,475) 
 5,166   
 2,691  
 25,624   $  16,650    $ 
 8,974   
 76,777   $  45,575    $  31,202   

(1)  Other project costs are leveraged across multiple programs. 
(2)  Other costs include facility, supply, consultant and overhead costs that support multiple programs. 

General and administrative expenses  

  Year Ended December 31,   

2020 

2019 
(in thousands) 

Change 
$ 

General and administrative expenses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .      $  42,448    $  22,963   $  19,485  

General and administrative expenses were $42.4 million for the year ended December 31, 2020 compared with 

$23.0 million for the year ended December 31, 2019, an increase of $19.5 million. The increase is attributable to 
personnel and related expenses as we continue to increase our headcount, and commercialization readiness activity. 

Other operating (income) expense, net 

Other operating (income) expense, net  . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .    $  (14,646)  $   2,210   $  (16,856) 

Other operating (income) expense, net totaled $14.6 million of income for the year ended December 31, 2020 

compared with $2.2 million of expense for the year ended December 31, 2019, a difference of $16.9 million. The 
difference primarily relates to changes in foreign currency exchange rates in the two periods. 

  Year Ended December 31,   

2020 

2019 
(in thousands) 

Change 
$ 

94 

 
 
 
 
 
 
 
 
 
 
 
 
 
 
     
     
     
     
 
 
 
 
 
 
 
  
   
 
  
  
  
  
  
  
  
  
  
 
 
 
 
  
 
 
  
  
  
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
     
     
     
     
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
     
     
     
 
 
 
 
 
 
Interest expense, net 

  Year Ended December 31,   

2020 

2019 
(in thousands) 

Change 
$ 

Interest expense, net  . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .     $  (11,362)  $   (5,296)   $  (6,066) 

Interest expense, net totaled $11.4 million of expense for the year ended December 31, 2020 compared with $5.3 
million for the year ended December 31, 2019, a difference of $6.1 million. The difference was principally attributable 
to higher non-cash interest expense recorded in connection with the sale of future royalties, related to sales of elobixibat 
in Japan in addition to interest expense associated with our note payable offset by interest income associated with our 
interest-bearing cash and cash equivalents. 

Other non-operating income 

Other non-operating income . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .     $ 

 —   $   3,691   $  (3,691) 

Other non-operating income was $0.0 million of income for the year ended December 31, 2020 compared with 

$3.7 million of income for the year ended December 31, 2019, a difference of $3.7 million. The difference is primarily 
related to the proceeds from a legal settlement in 2019. 

     Year Ended December 31,   

2020 

2019 
(in thousands) 

Change 
 $    

Liquidity and Capital Resources 

Sources of Liquidity 

We do not expect to generate significant revenue from product sales unless and until we or a potential future 

licensee or collaborator obtains marketing approval for, and commercializes, one or more of our current or potential 
future product candidates (other than elobixibat as a treatment for chronic constipation in Japan), which we do not expect 
to occur until at least the second half of 2021, if at all. We anticipate that we will continue to generate losses for the 
foreseeable future, and we expect the losses to increase as we continue the development of and seek regulatory approvals 
for our product candidates. We are subject to all of the risks applicable to the development of new pharmaceutical 
products and may encounter unforeseen expenses, difficulties, complications, delays and other unknown factors that may 
harm our business. We expect that we will need substantial additional funding to complete development of and 
potentially commercialize our product candidates. 

Our operations have historically been financed primarily through issuances of equity or convertible debt, upfront 
fees paid upon entering into license agreements, payments received upon the achievement of specified milestone events 
under license agreements, grants and venture debt borrowings and the HCR royalty monetization transactions. Our 
primary uses of capital are, and we expect will continue to be, personnel-related costs, third party expenses associated 
with our research and development programs, including the conduct of clinical trials, and manufacturing-related costs for 
our product candidates. 

As of  December 31, 2020, our cash and cash equivalents were approximately $251.3 million. 

During the first quarter of 2018, following the Japanese MHLW’s approval of elobixibat for the treatment of 

chronic constipation in January 2018, we received a $44.5 million payment, net of certain transaction expenses, from 
HCR under our RIAA. Additionally, this approval triggered a milestone payment to us from EA Pharma of $11.2 
million. In June 2020, we entered into an amendment to the RIAA with HCR pursuant to which HCR agreed to pay us 
an additional $14.8 million, net of certain transaction expenses in exchange for the elimination of the (i) $78.8 million 
cap amount on HCR’s rights to receive royalties on sales in Japan and sales milestones for elobixibat in certain other 

95 

 
 
 
 
 
 
 
 
 
 
 
 
 
 
     
     
     
    
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
  
 
     
     
     
  
 
 
 
 
 
territories that may become payable by EA Pharma and (ii) $15.0 million payable to us if a specified sales milestone is 
achieved for elobixibat in Japan. As of December 31, 2020, we have received approximately $49.9 million in upfront 
and milestone payments from EA Pharma under a license agreement for the development and commercialization of 
elobixibat in specified countries in Asia.  We are eligible to receive additional amounts of up to $5.3 million under the 
amended agreement, if a specified regulatory event is achieved for elobixibat. Pursuant to the RIAA, any additional 
royalties and sales milestone payments that we receive from EA Pharma will be payable by us to HCR under the RIAA. 

In March 2019, we entered into a sales agreement with Cowen and Company, LLC, or Cowen, with respect to an 

at-the-market offering program for us to offer and sell, from time to time at our sole discretion, shares of our common 
stock having an aggregate offering price of up to $50.0 million. Subsequently, in May 2019, we sold 637,367 shares of 
our common stock for net proceeds of approximately $20.8 million pursuant to the sales agreement. This agreement 
terminated on May 7, 2020. 

In addition, in February 2020, we completed an underwritten public offering of 2,190,750 shares of our common 

stock under our universal shelf registration statement for net proceeds of approximately $43.0 million. 

On May 7, 2020, we filed a new universal shelf registration on Form S-3, or the 2020 Form S-3, with the SEC, 
which was declared effective on May 18, 2020, pursuant to which we registered for sale up to $200.0 million of any 
combination of our common stock, preferred stock, debt securities, warrants, rights and/or units from time to time and at 
prices and on terms that we may determine. On May 7, 2020, we also entered into a new sales agreement, with respect to 
an at-the-market offering program under which we may offer and sell, from time to time at our sole discretion, shares of 
our common stock having an aggregate offering price of up to $50.0 million. This agreement terminated on September 9, 
2020.  

On September 14, 2020, we completed an underwritten public offering of 4,000,000 shares of our common stock 

under this registration statement. We received net proceeds from this offering of approximately $150.4 million, after 
deducting underwriting discounts and commissions, but before deducting offering expenses. As of December 31, 2020, 
$40.0 million of securities remain available for issuance under the 2020 Form S-3. 

On June 8, 2020, we entered into a Loan and Security Agreement with the several banks and other financial 
institutions or entities from time to time parties to the Loan and Security Agreement, as lenders, or collectively referred 
to as the Lender, and Hercules Capital, Inc., in its capacity as administrative agent and collateral agent for itself and 
Lender (in such capacity, the Agent or Hercules). The Loan and Security Agreement provides for term loans in an 
aggregate principal amount of up to $80.0 million to be delivered in multiple tranches, (the Term Loans). The tranches 
consist of (i) a term loan advance to Borrower in an aggregate principal amount of up to $15.0 million, of which (A) we 
agreed to borrow an aggregate principal amount of $10.0 million on the date on which all conditions to the funding of 
the Term Loans by the Lender were met (the Closing Date), but we did not request that the Lender make an additional 
term loan advance to us in an aggregate principal amount of $5.0 million prior to December 15, 2020 as permitted under 
the agreement, (ii) subject to the achievement of certain initial performance milestones, or Performance Milestone I, we 
have the right to request that the Lender make additional term loan advances to us in an aggregate principal amount of up 
to $20.0 million from January 1, 2021 through December 15, 2021 in minimum increments of $10.0 million, and 
(iii) subject to the Lender’s investment committee’s sole discretion, we have the right to request that the Lender make 
additional term loan advances to us in an aggregate principal amount of up to $45.0 million through March 31, 2022 in 
minimum increments of $5.0 million. As of December 31, 2020, we borrowed an aggregate principal amount of $10.0 
million and an aggregate principal amount of up to $65.0 million remains available for future borrowings. 

Under the Loan and Security Agreement, we also agreed to issue to Hercules warrants to purchase a number of 
shares of our common stock equal to 1% of the aggregate amount of the Term Loans that are funded, as such amounts 
are funded. On the Closing Date, we issued a warrant for 5,311 shares of our common stock. The warrants will be 
exercisable for a period of seven years from the date of the issuance of each warrant at a per-share exercise price equal to 
$18.83, subject to certain adjustments as specified in the warrants. The shares of common stock underlying the warrants 
were subsequently registered on Form S-3 with the SEC, which was declared effective on August 18, 2020.  

96 

On or about February 25, 2021, we expect to file a new universal shelf registration on Form S-3 with the SEC, 
pursuant to which we expect to register for sale up to 400million of any combination of our common stock, preferred 
stock, debt securities, warrants, rights and/or units from time to time and at prices and on terms that we may determine, 
which we refer to as the 2021 Form S-3. On or about February 25, 2021, we intend to enter into a new sales agreement, 
which we refer to as the 2021 Sales Agreement, with respect to an at-the-market offering program under which we may 
offer and sell, from time to time at our sole discretion, shares of our common stock having an aggregate offering price of 
up to $100 million. Our issuance and sale, if any, of shares under the 2021 Sales Agreement is subject to the 
effectiveness of the 2021 Form S-3. We make no assurances as to if or when the 2021 Form S-3 will become effective 
or, if it does become effective, as to the continued effectiveness of the 2021 Form S-3. No additional securities registered 
under the 2020 Form S-3 will be offered or sold after date of effectiveness of the 2021 Form S-3.  This report shall not 
constitute an offer to sell or the solicitation of an offer to buy any shares under the 2021 Sales Agreement that we intend 
to enter into or any securities under the 2021 Form S-3 that we intend to file with the SEC, nor shall there be any sale of 
such securities in any state in which such offer, solicitation or sale would be unlawful prior to registration or 
qualification under the securities laws of any such state. 

Cash Flows 

Years ended December 31, 2020 and December 31, 2019 

December 31,  

2020 

2019 

(in thousands) 

Net cash (used in) provided by: 

Operating activities . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .    $  (101,029)  
Investing activities  . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .   
 (78)  
Financing activities . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .   
    220,276   
Total  . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .    $   119,169   
 260   

 (52,919)
 (540)
 23,504 
 (29,955)
 (2,087)
Effect of exchange rate changes on cash and cash equivalents . . . . . . . . . . . . . . . . . . . . . . . .   
Net increase (decrease) in cash and cash equivalents. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .    $   119,429   $   (32,042)

Operating activities 

Cash used in operating activities of $101.0 million for the year ended December 31, 2020 was primarily a result of 

our $107.6 million net loss from operations and a net decrease in assets and liabilities of $4.7 million. The net decrease 
in operating assets and liabilities for the year ended December 31, 2020 was primarily driven by decreases in prepaid 
expenses and other current assets, decreases in accrued expenses, as well as decreases in other current and long-term 
liabilities. This decrease was offset by non cash items, including $14.6 million of stock-based compensation expense, 
$14.6 million of foreign currency adjustments, and $10.8 million of accretion of liability related to sale of future 
royalties. Cash used in operating activities of $52.9 million for the year ended December 31, 2019 was primarily a result 
of our $62.7 million net loss from operations and a net decrease in assets and liabilities of $10.3 million. The net 
decrease in operating assets and liabilities for the year ended December 31, 2019 was primarily driven by a decrease in 
accrued expenses and an increase to prepaid expenses and other current asset, and accounts payable. This decrease was 
offset by non cash items, including $8.4 million of accretion of liability related to sale of future royalties, $7.6 million of 
stock-based compensation expense, and $4.0 million of foreign currency adjustments. 

Investing activities 

Net cash used in investing activities for the year ended December 31, 2020 was $0.1 million compared to $0.5 

million for 2019.  The change was primarily due to higher purchases of property and equipment in 2019. 

Financing activities 

Cash provided by financing activities of $220.3 million for the year ended December 31, 2020 was primarily 
related to proceeds from the issuance of common stock, net of issuance costs of $193.3 million, proceeds from the HCR 

97 

 
 
 
 
 
 
 
 
 
 
     
     
 
 
    
    
 
  
 
  
 
 
 
  
 
 
royalty agreement of $14.8 million, proceeds from the issuance of debt, net of issuance costs of $9.5 million, and 
proceeds from exercise of options of $2.8 million. Cash provided by financing activities of $23.5 million for the year 
ended December 31, 2019 was primarily related to proceeds from the issuance of common stock, net of issuance costs of 
$20.8 million and proceeds from exercise of options of $2.7 million. 

Funding Requirements 

Cash used to fund operating expenses is affected by the timing of when we pay expenses, as reflected in the 

change in our outstanding accounts payable and accrued expenses. We believe that our existing cash and cash 
equivalents will be sufficient to meet our projected operating requirements into 2023 including for the commercial 
launch of odevixibat, if approved, for our open-label trial in PFIC for odevixibat, in addition to Phase 3 clinical 
programs for odevixibat in biliary atresia and Alagille syndrome, as well as A3907, A2342 and our preclinical programs. 
We anticipate an operating cash burn rate in the range of $120.0 to $130.0 million in 2021. However, our operating plans 
may change as a result of many factors, including those described below, and we may need additional funds sooner than 
planned to meet operational needs and capital requirements. In addition, if the conditions for raising capital are favorable 
we may seek to raise additional funds at any time. 

Our future funding requirements will depend on many factors, including the following: 

• 

• 

• 

• 

• 

• 

• 

• 

• 

• 

• 

• 

• 

any unfavorable development or delay in our odevixibat program in PFIC, including the review or approval 
of our odevixibat marketing applications in the United States and Europe for PFIC and the costs and timing 
of our pre-commercialization preparations; 

the costs, design, duration and any potential delays of the pivotal clinical trial of odevixibat in biliary atresia 
and the pivotal clinical trial of odevixibat in ALGS; 

 the scope, number, progress, initiation, duration, cost, results and timing of clinical trials and nonclinical 
studies of our current or future product candidates; 

whether and to what extent milestone events are achieved under our license agreement with EA Pharma or 
any potential future licensee or collaborator; 

the outcomes and timing of regulatory reviews, approvals or other actions; 

our ability to obtain marketing approval for our product candidates; 

our ability to establish and maintain additional licensing, collaboration or similar arrangements on favorable 
terms and whether and to what extent we retain development or commercialization responsibilities under any 
new licensing, collaboration or similar arrangement; 

the success of any other business, product or technology that we acquire or in which we invest; 

our ability to maintain, expand and defend the scope of our intellectual property portfolio; 

our ability to manufacture any approved products on commercially reasonable terms; 

our ability to establish a sales and marketing organization or suitable third-party alternatives for any 
approved product; 

the number and characteristics of product candidates and programs that we pursue; 

current and potential impacts of the COVID-19 pandemic on our business; 

98 

• 

• 

• 

• 

• 

the costs of acquiring, licensing or investing in businesses, product candidates and technologies; 

our need and ability to hire additional management and scientific and medical personnel; 

the costs to operate as a public company in the United States, including the need to implement additional 
financial and reporting systems and other internal systems and infrastructure for our business; 

market acceptance of our product candidates, to the extent any are approved for commercial sale; and 

the effect of competing technological and market developments. 

We cannot determine precisely the dates of  potential regulatory approval of odevixibat in PFIC or the dates of our 

potential commercial launch, or the completion dates and related costs of our development programs due to inherent 
uncertainties in outcomes of clinical trials and the regulatory approval process. We cannot be certain that we will be able 
to successfully complete our pre-commercialization activities or research and development programs or establish 
licensing, collaboration or similar arrangements for our product candidates. Our failure or the failure of any current or 
potential future licensee to complete research and development programs for our product candidates could have a 
material adverse effect on our financial position or results of operations. 

We expect to continue to incur losses. Our ability to achieve and maintain profitability is dependent upon the 
successful development, regulatory approval and commercialization of our product candidates and achieving a level of 
revenues adequate to support our cost structure. We may never achieve profitability. 

If the conditions for raising capital are favorable, we may seek to finance future cash needs through public or 

private equity or debt offerings or other financings. Additionally, if we need to raise additional capital to fund our 
operations, complete clinical trials, or potentially commercialize our product candidates, we may likewise seek to 
finance future cash needs through public or private equity or debt offerings or other financings. The necessary funding 
may not be available to us on acceptable terms or at all. 

We have an effective universal shelf registration statement on Form S-3 with the SEC, pursuant to which we 

registered for sale up to $200 million of any combination of our common stock, preferred stock, debt securities, 
warrants, rights and/or units from time to time and at prices and on terms that we may determine. As of December 31, 
2020, $40.0 million of securities remain available for issuance under the shelf registration statement, which we refer to 
as the 2020 Form S-3. On or about February 25, 2021, we expect to file a new universal shelf registration on Form S-3 
with the SEC, pursuant to which we expect to register for sale up to $400million of any combination of our common 
stock, preferred stock, debt securities, warrants, rights and/or units from time to time and at prices and on terms that we 
may determine, which we refer to as the 2021 Form S-3, including up to $100 million of our common stock pursuant to 
the new sales agreement with respect to an at-the-market offering program. We make no assurances as to if or when the 
2021 Form S-3 will become effective or, if it does become effective, as to the continued effectiveness of the 2021 
Form S-3. No additional securities registered under the 2020 Form S-3 will be offered or sold after the date of 
effectiveness of the 2021 Form S-3.  This report shall not constitute an offer to sell or the solicitation of an offer to buy 
any shares under the 2021 Sales Agreement that we intend to enter into or any securities under the 2021 Form S-3 that 
we intend to file with the SEC, nor shall there be any sale of such securities in any state in which such offer, solicitation 
or sale would be unlawful prior to registration or qualification under the securities laws of any such state. 

The sale of additional equity or convertible debt securities may result in significant dilution to our stockholders, 

and the terms may include liquidation or other preferences that adversely affect the rights of our stockholders. In 
addition, following the closing of our September 2020 public offering of common stock, we have a limited number of 
authorized shares of common stock available for future issuance that are not already issued or reserved for issuance. The 
incurrence of additional debt financing would result in debt service obligations and the instruments governing such debt 
may provide for operating and financing covenants that would restrict our operations. We may also seek to finance 
future cash needs through potential future licensing, collaboration or similar arrangements. These arrangements may not 
be available on acceptable terms or at all, and we may have to relinquish valuable rights to our technologies, future 
revenue streams, research programs or product candidates or to grant licenses on terms that may not be favorable to us. 

99 

If adequate funds are not available, we may be required to delay, reduce the scope of or eliminate our development 
programs or obtain funds through third-party arrangements that may require us to relinquish rights to certain product 
candidates that we might otherwise seek to develop or commercialize independently. 

Item 7A.  QUANTITATIVE AND QUALITATIVE DISCLOSURES ABOUT MARKET RISK 

Not required for smaller reporting companies. 

100 

 
 
 
Item 8. 

FINANCIAL STATEMENTS AND SUPPLEMENTARY DATA 

ALBIREO PHARMA, INC. 

Index to Consolidated Financial Statements and Financial Statement Schedules 
Report of Independent Registered Public Accounting Firm  . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  
Consolidated Balance Sheets as of December 31, 2020 and 2019 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  
Consolidated Statements of Operations for the Years Ended December 31, 2020 and 2019  . . . . . . . . . . .  
Consolidated Statements of Comprehensive Loss for the Years Ended December 31, 2020 and 2019 . . .  
Consolidated Statements of Stockholders’ Equity for the Years Ended December 31, 2020 and 2019 . . .  
Consolidated Statements of Cash Flows for the Years Ended December 31, 2020 and 2019 . . . . . . . . . . .  
Notes to Consolidated Financial Statements . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  

Number 

F-1
F-3
F-4
F-5
F-6
F-7
F-8

101 

 
 
 
     
 
 
 
Item 9. 

CHANGES IN AND DISAGREEMENTS WITH ACCOUNTANTS ON ACCOUNTING AND 
FINANCIAL DISCLOSURE 

Not applicable. 

Item 9A.  CONTROLS AND PROCEDURES 

Evaluation of Disclosure Controls and Procedures 

We have performed an evaluation under the supervision and with the participation of our management, including 

our Chief Executive Officer (CEO) and Chief Financial Officer (CFO), of the effectiveness of our disclosure controls 
and procedures, as defined in Rule 13a-15(e) under the Securities Exchange Act of 1934 (the Exchange Act). Based on 
that evaluation, our management, including our CEO and CFO, concluded that our disclosure controls and procedures 
were effective as of December 31, 2020 to provide reasonable assurance that information required to be disclosed by us 
in the reports filed or submitted by us under the Exchange Act is recorded, processed, summarized and reported within 
the time periods specified in the SEC’s rules and forms. Such disclosure controls and procedures include controls and 
procedures designed to ensure that information required to be disclosed is accumulated and communicated to our 
management, including our CEO and CFO, to allow timely decisions regarding required disclosures. 

Management’s Report on Internal Control over Financial Reporting 

The Company’s management is responsible for establishing and maintaining adequate internal control over 
financial reporting, as such term is defined in Rule 13a-15(f) of the Exchange Act. Internal control over financial 
reporting is a process designed to provide reasonable assurance regarding the reliability of financial reporting and the 
preparation of financial statements for external purposes in accordance with generally accepted accounting principles. 

The Company’s internal control over financial reporting includes those policies and procedures that: (1) pertain to 
the maintenance of records that, in reasonable detail, accurately and fairly reflect the transactions and dispositions of the 
assets of the Company; (2) provide reasonable assurance that transactions are recorded as necessary to permit 
preparation of financial statements in accordance with generally accepted accounting principles, and that receipts and 
expenditures of the Company are being made only in accordance with authorizations of the Company’s management and 
directors; and (3) provide reasonable assurance regarding prevention or timely detection of unauthorized acquisition, use 
or disposition of assets of the Company that could have a material effect on the financial statements. 

Because of its inherent limitations, internal control over financial reporting may not prevent or detect 

misstatements. Also, projections of any evaluation of effectiveness to future periods are subject to the risk that controls 
may become inadequate because of changes in conditions, or that the degree of compliance with the policies or 
procedures may deteriorate. 

Under the supervision, and with the participation, of our management, including the CEO and CFO, we conducted 

an evaluation of the effectiveness of our internal control over financial reporting as of December 31, 2020. In making 
this assessment, management used the criteria established in Internal Control-Integrated Framework (2013) issued by the 
Committee of Sponsoring Organizations of the Treadway Commission (“COSO”). Based on this assessment, 
management, including our CEO and CFO, has concluded that our internal control over financial reporting was effective 
as of December 31, 2020. 

Changes in Internal Control over Financial Reporting 

There were no changes in our internal control over financial reporting identified in connection with the evaluation 

of such internal control that occurred during the quarter ended December 31, 2020, that has materially affected, or is 
reasonably likely to materially affect, our internal control over financial reporting. 

102 

 
Item 9B.  OTHER INFORMATION 

Not applicable. 

PART III 

Item 10. 

DIRECTORS, EXECUTIVE OFFICERS AND CORPORATE GOVERNANCE 

The response to this item is incorporated by reference from the discussion responsive thereto under the captions 
“Management and Corporate Governance” and “Corporate Code of Conduct and Ethics” in our proxy statement for the 
2021 annual meeting of stockholders. 

Item 11. 

EXECUTIVE COMPENSATION 

The response to this item is incorporated by reference from the discussion responsive thereto under the caption 
“Executive Officer and Director Compensation” in our proxy statement for the 2021 annual meeting of stockholders. 

Item 12. 

SECURITY OWNERSHIP OF CERTAIN BENEFICIAL OWNERS AND MANAGEMENT AND 
RELATED STOCKHOLDER MATTERS 

The response to this item is incorporated by reference from the discussion responsive thereto under the captions 
“Security Ownership of Certain Beneficial Owners and Management” and “Equity Compensation Plan Information” in 
our proxy statement for the 2021 annual meeting of stockholders. 

Item 13. 

CERTAIN RELATIONSHIPS AND RELATED TRANSACTIONS, AND DIRECTOR 
INDEPENDENCE 

The response to this item is incorporated by reference from the discussion responsive thereto under the captions 
“Certain Relationships and Related Person Transactions” and “Management and Corporate Governance” in our proxy 
statement for the 2021 annual meeting of stockholders. 

Item 14. 

PRINCIPAL ACCOUNTING FEES AND SERVICES 

The response to this item is incorporated by reference from the discussion responsive thereto under the caption 

“Ratification of Appointment of Independent Registered Public Accounting Firm” in our proxy statement for the 2021 
annual meeting of stockholders. 

103 

 
 
 
 
 
 
 
PART IV 

Item 15. 

EXHIBITS AND FINANCIAL STATEMENT SCHEDULES 

Item 15(a)                      The following documents are filed as part of this Annual Report on Form 10-K: 

Item 15(a)(1) and (2)    See “Index to Consolidated Financial Statements and Financial Statement Schedules” at Item 8 

to this Annual Report on Form 10-K. Other financial statement schedules have not been 
included because they are not applicable or the information is included in the financial 
statements or notes thereto. 

Item 15(a)(3)                 Exhibits 

The following is a list of exhibits filed as part of this Annual Report on Form 10-K.  

Exhibit 
Number    

Exhibit Description 

2.1 

  Amended and Restated Share 

Exchange Agreement, dated as of 
July 13, 2016, by and among the 
Registrant (formerly Biodel Inc.), 
Albireo Limited and the Sellers 
listed on Schedule I thereto. 

3.1 

  Restated Certificate of 

Incorporation, as amended, of the 
Registrant. 

3.2 

  Amended and Restated Bylaws of 

the Registrant. 

4.1 

  Form of common stock certificate.   

4.2 

  Description of the Registrant’s 

Securities. 

4.3 

  Form of Warrant issued by the 

Registrant to Hercules Capital, Inc. 

10.1* 

  Amended and Restated 

Employment Agreement, dated as 
of March 6, 2019, by and between 
the Registrant and Ronald H.W. 
Cooper. 

10.2* 

  Employment Agreement, dated as 

of October 4, 2018, by and 
between the Registrant and Simon 
N. R. Harford. 

Incorporated by 
Reference 
herein from 
Form or Schedule    

Filed 
Herewith    

Filing Date 

SEC File/ 
Reg. 
Number 

8-K 
(Exhibit 2.1) 

7/13/2016 

  001-33451 

S-3 
(Exhibit 4.1.1) 

S-8 
(Exhibit 4.2) 

10-K 
(Exhibit 4.1) 
10-K 
(Exhibit 4.2) 

8-K 
(Exhibit 10.2) 

10-K 
(Exhibit 10.1) 

10/13/2017 

  333-220958 

7/6/2007 

  333-144407 

12/22/2016 

  001-33451 

3/2/2020 

  001-33451 

6/9/2020 

  001-33451 

3/6/2019 

  001-33451 

10-Q 
(Exhibit 10.3) 

11/8/2018 

  001-33451 

104 

 
   
   
   
   
   
   
   
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
   
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
Exhibit 
Number    

Exhibit Description 

10.3* 

  Amended and Restated 

Employment Agreement, dated as 
of March 6, 2019, by and between 
Albireo AB and Jan P. Mattsson, 
Ph.D. 

10.4* 

  Amended and Restated 

Employment Agreement, dated as 
of March 6, 2019, by and between 
the Registrant and Martha J. 
Carter. 

10.5* 

  Amended and Restated 

Employment Agreement, dated as 
of March 6, 2019, by and between 
the Registrant and Patrick Horn. 

10.6* 

  Amended and Restated 

Employment Agreement, dated as 
of March 6, 2019, by and between 
the Registrant and Jason G. 
Duncan. 

10.7* 

  Employment Agreement, dated as 

of March 25, 2019, by and between 
the Registrant and Pamela 
Stephenson. 

10.8* 

  Employment Agreement, dated as 

of October 31, 2019, by and 
between the Registrant and 
Michelle Graham. 

10.9* 

  Albireo Pharma, Inc. 2018 Equity 

Incentive Plan, as amended. 

10.10*    Form of Stock Option Agreement 

under the Albireo Pharma, Inc. 
2018 Equity Incentive Plan. 

10.11*    Form of Restricted Stock Unit 
Agreement under the Albireo 
Pharma, Inc. 2018 Equity Incentive 
Plan. 

10.12*    Albireo Pharma, Inc. 2018 

Employee Stock Purchase Plan. 

Incorporated by 
Reference 
herein from 
Form or Schedule    

Filed 
Herewith    

Filing Date 

SEC File/ 
Reg. 
Number 

10-K 
(Exhibit 10.3) 

10-K 
(Exhibit 10.4) 

3/6/2019 

  001-33451 

3/6/2019 

  001-33451 

10-K 
(Exhibit 10.5) 

3/6/2019 

  001-33451 

10-K 
(Exhibit 10.6) 

3/6/2019 

  001-33451 

10-Q 
(Exhibit 10.1) 

10-Q 
(Exhibit 10.1) 

8-K 
(Exhibit 10.1) 

10-Q 
(Exhibit 10.2) 

10-Q 
(Exhibit 10.5) 

10-Q 
(Exhibit 10.3) 

5/9/2019 

  001-33451 

11/6/2019 

  001-33451 

6/18/2019 

  001-33451 

8/9/2018 

  001-33451 

8/9/2018 

  001-33451 

8/9/2018 

  001-33451 

105 

 
   
   
   
   
   
   
   
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
Exhibit 
Number    

Exhibit Description 

10.13*    Inducement Stock Option 

Agreement, dated as of October 10, 
2018, by and between the 
Registrant and Simon N.R. 
Harford. 

10.14*    Inducement Restricted Stock Unit 

Agreement, dated as of October 10, 
2018, by and between the 
Registrant and Simon N.R. 
Harford. 

Incorporated by 
Reference 
herein from 
Form or Schedule    

Filed 
Herewith    

Filing Date 

SEC File/ 
Reg. 
Number 

10-Q 
(Exhibit 10.5) 

11/8/2018 

  001-33451 

10-Q 
(Exhibit 10.6) 

11/8/2018 

  001-33451 

10.15*    Albireo Pharma, Inc. 2017 

Inducement Equity Incentive Plan. 

10-Q 
(Exhibit 10.1) 

11/14/2017 

  001-33451 

10.16*    Form of Stock Option Agreement 

under the Albireo Pharma, Inc. 
2017 Inducement Equity Incentive 
Plan. 

10.17*    Albireo Pharma, Inc. 2016 Equity 

Incentive Plan. 

10.18*    Form of Stock Option Agreement 

under the Albireo Pharma, Inc. 
2016 Equity Incentive Plan. 

10.19*    Replacement Stock Options 

granted to Ronald H.W. Cooper in 
connection with the closing of the 
Biodel Transaction. 

10.20*    2010 Stock Incentive Plan, as 

amended. 

10.21*    2010 Incentive Stock Option 

Agreement. 

10.22*    2010 Non Statutory Stock Option 

Agreement. 

10.23*    2010 Restricted Stock Unit 

Agreement. 

10.24*    Amended and Restated 2004 Stock 

Incentive Plan. 

10-K 
(Exhibit 10.11) 

8-K 
(Exhibit 10.9) 

10-K 
(Exhibit 10.13) 

10-K 
(Exhibit 10.14) 

Schedule 14A 
(Exhibit A) 

10-Q 
(Exhibit 10.2) 

10-Q 
(Exhibit 10.3) 

10-Q 
(Exhibit 10.4) 

S-1/A 
(Exhibit 10.3) 

3/27/18 

  001-33451 

11/4/2016 

  001-33451 

12/22/2016 

  001-33451 

12/22/2016 

  001-33451 

1/26/2012 

  001-33451 

5/7/2010 

  001-33451 

5/7/2010 

  001-33451 

5/7/2010 

  001-33451 

3/27/2007 

  333-140504 

106 

 
   
   
   
   
   
   
   
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
Exhibit 
Number    

Exhibit Description 

10.25*    Form of Incentive Stock Option 

Agreement for 2004 Amended and 
Restated Stock Incentive Plan. 

10.26*    2005 Non-Employee Directors’ 

Stock Option Plan. 

10.27*    Form of Option Agreement for 
2005 Non-Employee Directors’ 
Stock Option Plan. 

10.28*    Form of Indemnification 

Agreement, by and between the 
Registrant and each of its directors 
and executive officers. 

10.29*    Nonemployee Director 

Compensation Policy. 

10.30 

  Asset Purchase and License 

Agreement, dated as of 
September 2, 2016, by and among 
Unilife Corporation, Unilife 
Medical Solution, Inc. and 
Biodel Inc. 

10.30.1**   License  Agreement,  dated  as  of
April 2,  2012,  by  and  between
Elobix  AB,  as  assignee  of  Albireo
AB,  and  EA  Pharma  Co., Ltd. 
(formerly  known  as  Ajinomoto
Pharmaceuticals Co., Ltd.). 

10.30.2**   First Amendment to the License 

Agreement, dated as of January 30, 
2015, by and between Elobix AB, 
as assignee of Albireo AB, and EA 
Pharma Co., Ltd. (formerly known 
as Ajinomoto Pharmaceuticals 
Co., Ltd.). 

10.30.3**   Second Amendment to the License 

Agreement, dated as of April 6, 
2016, by and between Elobix AB 
and EA Pharma Co., Ltd. (formerly 
known as Ajinomoto 
Pharmaceuticals Co., Ltd.). 

Incorporated by 
Reference 
herein from 
Form or Schedule    

Filed 
Herewith    

Filing Date 

SEC File/ 
Reg. 
Number 

10-K 
(Exhibit 10.19) 

S-1/A 
(Exhibit 10.5) 

10-K 
(Exhibit 10.20) 

8-K 
(Exhibit 10.8) 

10-Q 
(Exhibit 10.1) 

12/21/2007 

  001-33451 

3/27/2007 

  333-140504 

12/21/2007 

  001-33451 

11/4/2016 

  001-33451 

5/7/2020 

  001-33451 

8-K 
(Exhibit 10.1) 

9/9/2016 

  001-33451 

10-K 
(Exhibit 10.28.1) 

3/27/2017 

001-33451 

10-K 
(Exhibit 10.28.2) 

3/27/2017 

  001-33451 

10-K 
(Exhibit 10.28.3) 

3/27/2017 

  001-33451 

107 

 
   
   
   
   
   
   
   
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
Exhibit 
Number    

Exhibit Description 

10.30.4**   Third Amendment to the License 
Agreement, dated as of 
December 7, 2017, by and between 
Elobix AB and EA Pharma 
Co., Ltd. (formerly known as 
Ajinomoto Pharmaceuticals 
Co., Ltd.). 

10.31.1**   Royalty Interest Acquisition 

Agreement, dated as of 
December 28, 2017, by and among 
Elobix AB, HealthCare Royalty 
Partners III, L.P. and, solely for the 
purposes specified therein, the 
Registrant. 

10.31.2** * Amendment to Royalty Interest 

Acquisition Agreement, dated as of 
June 8, 2020, by and among Elobix 
AB, HealthCare Royalty Partners 
III, L.P. and, solely for the 
purposes specified therein, the 
Registrant. 

10.32.1    Office Lease Agreement, dated as 

of February 7, 2017, by and 
between the Registrant and SHIGO 
10 PO Owner LLC. 

10.32.2    Amendment No. 1 to Office Lease 
Agreement, dated as of March 28, 
2019, by and between the 
Registrant and POSIG Investors, 
LLC. 

10.32.3    Amendment No. 2 to Office Lease 

Agreement, dated as of May 4, 
2020, by and between NS Boston 
III PO Owner LLC and the 
Registrant. 

Incorporated by 
Reference 
herein from 
Form or Schedule    

Filed 
Herewith    

Filing Date 

SEC File/ 
Reg. 
Number 

10-K 
(Exhibit 10.27.4) 

3/27/2018 

  001-33451 

10-K 
(Exhibit 10.28) 

3/27/2018 

  001-33451 

8-K 
(Exhibit 10.3) 

6/9/2020 

  001-33451 

8-K 
(Exhibit 10.1) 

2/10/2017 

  001-33451 

8-K 
(Exhibit 10.1) 

4/3/2019 

  001-33451 

10-Q 
(Exhibit 10.2) 

5/7/2020 

  001-33451 

108 

 
   
   
   
   
   
   
   
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
Exhibit 
Number    

Exhibit Description 

10.33 

  Loan and Security Agreement, 

dated as of June 8, 2020, by and 
among the Registrant, Albireo AB, 
and Hercules Capital, Inc. 

Incorporated by 
Reference 
herein from 
Form or Schedule    

Filed 
Herewith    

Filing Date 

SEC File/ 
Reg. 
Number 

8-K 
(Exhibit 10.1) 

6/9/2020 

  001-33451 

10-Q 
(Exhibit 10.1) 

10-Q 
(Exhibit 10.2) 

10-Q 
(Exhibit 10.3) 

11/5/2020 

  001-33451 

11/5/2020 

  001-33451 

11/5/2020 

  001-33451 

10.34*    Albireo Pharma, Inc. 2020 

Inducement Equity Incentive Plan. 

10.35*    Form of Stock Option Agreement 

under the Albireo Pharma, Inc. 
2020 Inducement Equity Incentive 
Plan. 

10.36*    Form of Restricted Stock Unit 

Agreement under the 2020 
Inducement Equity Incentive Plan. 

14.1 

  Albireo Pharma, Inc. Corporate 
Code of Conduct and Ethics and 
Whistleblower Policy. 

21.1 

  Subsidiaries.   

23.1 

  Consent of Ernst & Young LLP. 

31.1 

  Certification of the Chief 

Executive Officer. 

31.2 

  Certification of the Chief Financial 

Officer. 

32.1 

  Certification pursuant to 

Section 906 of the Sarbanes-Oxley 
Act of 2002. 

101 

  Inline XBRL Instance Document 
(the instance document does not 
appear in the Interactive Data File 
because its XBRL tags are 
embedded within the Inline XBRL 
document). 

  SCH XBRL Taxonomy Extension 

Schema Document. 

X 

X 

X 

X 

X 

X 

X 

X 

109 

 
   
   
   
   
   
   
   
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
Exhibit 
Number    

Exhibit Description 

  CAL XBRL Taxonomy Extension 
Calculation Linkbase Document. 

  DEF XBRL Taxonomy Extension 

Definition. 

  LAB XBRL Taxonomy Extension 

Label Linkbase Document. 

104 

  PRE XBRL Taxonomy 

Presentation Linkbase Document. 
  Cover Page Interactive Date File 
(formatted as Inline XBRL and 
contained in Exhibit 101). 

Incorporated by 
Reference 
herein from 
Form or Schedule    

Filed 
Herewith    

Filing Date 

SEC File/ 
Reg. 
Number 

X 

X 

X 

X 

X 

*       Management contract or compensatory plan or arrangement. 

**     Confidential treatment has been granted with respect to certain portions of this Exhibit, which portions have been 

omitted and filed separately with the Securities and Exchange Commission as part of an application for confidential 
treatment pursuant to the Securities Exchange Act of 1934, as amended. 

***  Certain confidential portions of this Exhibit were omitted by means of marking such portions with brackets 

(“[***]”) because the identified confidential portions (i) are not material and (ii) would be competitively harmful if 
publicly disclosed. 

Item 16. 

FORM 10-K SUMMARY. 

None. 

110 

 
   
   
   
   
   
   
   
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Pursuant to the requirements of Section 13 or 15(d) of the Securities Exchange Act of 1934, the registrant has duly 

caused this report to be signed on its behalf by the undersigned, thereunto duly authorized. 

SIGNATURES 

Date: February 25, 2021 

ALBIREO PHARMA, INC. 

By:  /s/ Ronald H.W. Cooper 
Ronald H.W. Cooper 
President and Chief Executive Officer 

Pursuant to the requirements of the Securities Exchange Act of 1934, this report has been signed below by the 

following persons on behalf of the registrant and in the capacities and on the dates indicated. 

Signatures 

Title 

/s/ Ronald H.W. Cooper 
Ronald H.W. Cooper 

President, Chief Executive Officer and Director   
(principal executive officer) 

Date 

February 25, 2021 

February 25, 2021 

Chief Financial Officer and Treasurer 
(principal financial officer and 
principal accounting officer) 

/s/ Simon N.R. Harford 
Simon N.R. Harford 

/s/ David Chiswell, Ph.D. 
David Chiswell, Ph.D. 

/s/ Anne Klibanski, M.D. 
Anne Klibanski, M.D. 

/s/ Michael Gutch, Ph.D. 
Michael Gutch, Ph.D. 

/s/ Roger A. Jeffs, Ph.D. 
Roger A. Jeffs, Ph.D. 

/s/ Stephanie S. Okey, M.S. 
Stephanie S. Okey, M.S. 

/s/ Davey S. Scoon 
Davey S. Scoon 

Chairman of the Board 

February 25, 2021 

February 25, 2021 

February 25, 2021 

February 25, 2021 

February 25, 2021 

February 25, 2021 

Director 

Director 

Director 

Director 

Director 

111 

 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
     
     
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Report of Independent Registered Public Accounting Firm 

To the Stockholders and Board of Directors of Albireo Pharma, Inc. 

Opinion on the Financial Statements  

We  have  audited  the  accompanying  consolidated  balance  sheets  of  Albireo  Pharma,  Inc.  (the  Company)  as  of 
December 31, 2020 and 2019, the related consolidated statements of operations, comprehensive loss, stockholders’ equity 
and cash flows for each of the two years in the period ended December 31, 2020, and the related notes (collectively referred 
to as the “consolidated financial statements”). In our opinion, the consolidated financial statements present fairly, in all 
material respects, the financial position of the Company at December 31, 2020 and 2019, and the results of its operations 
and its cash flows for each of the two years in the period ended December 31, 2020, in conformity with U.S. generally 
accepted accounting principles.  

Basis for Opinion 

These financial statements are the responsibility of the Company’s management. Our responsibility is to express an opinion 
on the Company’s financial statements based on our audits. We are a public accounting firm registered with the Public 
Company Accounting Oversight Board (United States) (PCAOB) and are required to be independent with respect to the 
Company in accordance with the U.S. federal securities laws and the applicable rules and regulations of the Securities and 
Exchange Commission and the PCAOB.  

We  conducted  our  audits  in  accordance  with  the  standards  of  the  PCAOB.  Those  standards  require  that  we  plan  and 
perform the audit to obtain reasonable assurance about whether the financial statements are free of material misstatement, 
whether due to error or fraud. The Company is not required to have, nor were we engaged to perform, an audit of its 
internal control over financial reporting. As part of our audits we are required to obtain an understanding of internal control 
over financial reporting but not for the purpose of expressing an opinion on the effectiveness of the Company's internal 
control over financial reporting. Accordingly, we express no such opinion. 

Our audits included performing procedures to assess the risks of material misstatement of the financial statements, whether 
due to error or fraud, and performing procedures that respond to those risks. Such procedures included examining, on a 
test basis, evidence regarding the amounts and disclosures in the financial statements. Our audits also included evaluating 
the  accounting  principles  used  and  significant  estimates  made  by  management,  as  well  as  evaluating  the  overall 
presentation of the financial statements. We believe that our audits provide a reasonable basis for our opinion.  

Critical Audit Matter 

The critical audit matter communicated below is a matter arising from the current period audit of the financial statements 
that  was  communicated  or  required  to  be  communicated  to  the  audit  committee  and  that:  (1) relates  to  accounts  or 
disclosures that are material to the financial statements and (2) involved our especially challenging, subjective or complex 
judgments.  The  communication  of  the  critical  audit  matter  does  not  alter  in  any  way  our  opinion  on  the  consolidated 
financial statements, taken as a whole, and we are not, by communicating the critical audit matter below, providing a 
separate opinion on the critical audit matter or on the accounts or disclosures to which it relates. 

  Accounting for Clinical Trial Expense 

Description of the 
Matter 

  As  of  December 31,  2020,  the  Company’s  accrued  clinical  trial  development  costs  totaled  $4.9 
million.  Additionally,  the  Company’s  prepaid  expenses  and  other  current  assets  totaled  $10.6 
million, which includes amounts paid in advance of services being provided by contracted third 
parties. As discussed in Note 1 to the consolidated financial statements, the significant factors used 
in estimating clinical trial expenses include the number of patients enrolled, the percentage of work 
completed to date and contract milestones achieved. The Company’s estimates are highly dependent 

F-1 

 
 
 
 
 
upon  the  timeliness  and  accuracy  of  the  data  provided  by  contracted  third  parties  regarding  the 
status of their contracted activity.  

Auditing the Company’s clinical trial expenses is especially challenging due to the application of 
significant management judgment over the estimate of services provided. Specifically, the amount 
of accrued and prepaid clinical trial development costs recognized is sensitive to the availability of 
information to make the estimates, including the estimate of the period over which services will be 
performed, the level of effort expended as of the balance sheet date and the associated cost of such 
services. Additionally, due to the long duration of clinical trials and contractual terms for invoicing, 
the actual amounts incurred are required to be estimated as of the report date. 

  To  test  clinical  trial  expenses,  we  performed  audit  procedures  that  included,  among  others, 
reviewing material contracts and related amendments for key financial and contractual terms.  In 
addition,  for  a  sample  of  transactions,  we  compared  the  clinical  trial  expenses  incurred  against 
related year-to-date invoices and contracts to test that the expense was recognized in the appropriate 
period  and  that  amounts  were  properly  accrued  or  capitalized  depending  upon  the  timing  of 
payments. We also evaluated management’s estimates of progress for a sample of clinical trials and 
manufacturing  efforts  by  making  direct  inquiries  of  the  Company’s  research  and  development 
personnel who oversee the clinical trials.  Further, we confirmed with contracted third parties the 
costs  incurred  to  date,  the  terms  and  conditions  of  the  related  contract,  including  any  executed 
amendments. To evaluate the completeness of the accrued clinical trial development costs we also 
examined  invoices  received  from  contracted  third  parties  as  well  as  tested  material  cash 
disbursements made subsequent to December 31, 2020. 

How We 
Addressed the 
Matter in Our 
Audit 

/s/ Ernst & Young LLP 

We have served as the Company’s auditor since 2016. 

Boston, Massachusetts 
February 25, 2021 

F-2 

 
 
 
Albireo Pharma, Inc. 

Consolidated Balance Sheets 
(in thousands, except share and per share data) 

    December 31,      December 31, 

2020 

2019 

Assets 
Current assets: 

Cash and cash equivalents . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .     $   251,272   $  131,843 
Prepaid expenses and other current assets  . . . . . . . . . . . . . . . . . . . .    
 9,956 
    141,799 
Total current assets  . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .    
 597 
Property and equipment, net . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .    
 17,260 
Goodwill  . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .    
Other assets . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .    
 5,413 
Total assets  . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .     $   285,607   $  165,069 
Liabilities and Stockholders' Equity 
Current liabilities: 

 10,593  
 261,865  
 478  
 17,260  
 6,004  

Accounts payable . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .     $ 
Accrued expenses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .    
Other current liabilities. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .    
Total current liabilities . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .    
Liability related to sale of future royalties  . . . . . . . . . . . . . . . . . . . . .    
Note payable, net of discount . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .    
Other long-term liabilities  . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .    
Total liabilities . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .    
Stockholders’ Equity: 

Preferred stock, $0.01 par value per share — 50,000,000 

authorized at December 31, 2020 and December 31, 2019; 0  
and 0 issued and outstanding at December 31, 2020 and 
December 31, 2019, respectively . . . . . . . . . . . . . . . . . . . . . . . . . .    

Common stock, $0.01 par value per share — 30,000,000 

 5,283   $
 19,051  
 948  
 25,282  
 65,894  
 9,621  
 3,579  
 104,376  

 4,785 
 13,486 
 653 
 18,924 
 48,714 
 — 
 4,270 
 71,908 

 —  

 — 

authorized at December 31, 2020 and December 31, 2019; 
19,107,040 and 12,749,443 issued and outstanding at 
December 31, 2020 and December 31, 2019, respectively . . . . . .    
Additional paid-in capital . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .    
Accumulated other comprehensive (loss) income . . . . . . . . . . . . . .    
Accumulated deficit . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .    
Total stockholders’ equity . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .    

 127 
    245,769 
 6,452 
   (159,187)
 93,161 
Total liabilities and stockholders’ equity . . . . . . . . . . . . . . . . . . . . . . .     $   285,607   $  165,069 

 191  
 456,472  
 (8,612) 
    (266,820) 
 181,231  

See accompanying Notes to Consolidated Financial Statements. 

F-3 

 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
  
  
  
 
 
  
  
  
  
 
 
 
 
 
 
 
 
  
  
  
  
  
  
 
 
 
 
 
 
  
  
 
 
 
 
 
 
  
  
  
  
  
  
  
 
 
 
Albireo Pharma, Inc. 

Consolidated Statements of Operations 
(in thousands, except share and per share data) 

Revenue . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .    $ 
Operating expenses: 

Research and development. . . . . . . . . . . . . . . . . . . . . . . . . . .   
General and administrative  . . . . . . . . . . . . . . . . . . . . . . . . . .   
Other operating (income) expense, net . . . . . . . . . . . . . . . . .   
Total operating expenses . . . . . . . . . . . . . . . . . . . . . . . . . . .   
Operating loss . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .   
Interest expense, net . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .   
Other non-operating income . . . . . . . . . . . . . . . . . . . . . . . . . . .   
Net loss  . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .    $ 
Net loss per common share - basic and diluted  . . . . . . . . . .    $ 
Weighted-average common shares used to compute basic 

Year Ended December 31,  
2019 
2020 

 8,308    $ 

 9,636 

 76,777   
 42,448   
 (14,646) 
 104,579   
 (96,271) 
 (11,362) 
 —   
 (107,633)  $ 
 (6.73)  $ 

 45,575 
 22,963 
 2,210 
 70,748 
 (61,112)
 (5,296)
 3,691 
 (62,717)
 (5.04)

and diluted net loss per common share  . . . . . . . . . . . . . . .   

 15,983,058   

 12,437,742 

See accompanying Notes to Consolidated Financial Statements. 

F-4 

 
 
 
 
 
 
 
 
 
     
 
     
     
 
 
 
 
 
  
  
  
  
  
  
  
  
  
  
  
  
 
 
  
  
 
 
 
Albireo Pharma, Inc. 

Consolidated Statements of Comprehensive Loss 
(in thousands) 

Net loss  . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .    $ (107,633)  $  (62,717)
Other comprehensive (loss) income: 

      Year Ended December 31, 

2020 

2019 

Foreign currency translation adjustment . . . . . . . . . . . . . . . . . . . . . . . .   
Total other comprehensive (loss) income . . . . . . . . . . . . . . . . . . . . . .   

 2,159 
 2,159 
Total comprehensive loss . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .    $ (122,697)  $  (60,558)

    (15,064) 
    (15,064) 

See accompanying Notes to Consolidated Financial Statements. 

F-5 

 
 
 
 
 
 
 
 
 
 
     
    
 
 
 
 
  
  
 
 
 
Albireo Pharma, Inc. 

Consolidated Statements of Stockholders’ Equity 
(in thousands, except share amounts) 

Common Stock 
Shares 

    Additional      
Paid-In 
     Amount      Capital 

  Comprehensive   Accumulated   Stockholders’ 
     Income (loss)        Deficit 

Equity 

Total 

  Accumulated   
Other 

Balance--December 31, 2018 . . . . . . . . .       11,969,928   $  120   $ 214,694    $ 
Share based compensation expense . . . .    
Exercise of options and vesting of  

 7,578   

 —    

 —  

RSUs and ESPP . . . . . . . . . . . . . . . . . .     
Issuance of common stock, net of costs .     
Other comprehensive income . . . . . . . . .     
Net loss . . . . . . . . . . . . . . . . . . . . . . . . . . .    
Balance--December 31, 2019 . . . . . . . . .       12,749,443   $  127   $ 245,769    $ 
Share based compensation expense . . . .     
Exercise of options and vesting of  

 1     
 2,728   
 6       20,769   
 —   
 —   

 142,148  
 637,367  
 —  
 —  

 —       14,615   

 —     
 —    

 —  

RSUs and ESPP . . . . . . . . . . . . . . . . . .     
Issuance of warrants . . . . . . . . . . . . . . . .    
Issuance of common stock, net of costs .    
Other comprehensive loss . . . . . . . . . . . .     
Net loss . . . . . . . . . . . . . . . . . . . . . . . . . . .     
Balance--December 31, 2020 . . . . . . . . .    

 166,847  

 6,190,750  
 —  
 —  

 2     

 2,772   
 113   
 62      193,203   
 —   
 —     
 —   
 —     

 19,107,040   $  191   $ 456,472    $ 

 4,293   $  (96,470)  $   122,637 
 7,578 

 —  

 —  
 —  
 2,159  
 —  

 —  
 —  
 —  
 (62,717) 

 6,452   $ (159,187)  $ 

 —  

 —  

 2,729 
 20,775 
 2,159 
 (62,717)
 93,161 
 14,615 

 —  

 —  

 2,774 
 113 
 193,265 
 (15,064)
   (107,633)
 (8,612)  $ (266,820)  $   181,231 

 —  
   (107,633) 

 (15,064) 
 —  

See accompanying Notes to Consolidated Financial Statements. 

F-6 

 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
   
 
 
 
    
 
      
 
      
 
    
 
 
 
 
    
    
 
 
 
 
 
  
  
 
 
  
  
  
 
  
  
  
 
 
 
 
 
  
  
 
 
  
  
 
 
 
 
   
 
 
 
 
 
 
 
 
 
 
 
  
  
 
 
  
  
 
 
 
Albireo Pharma, Inc. 

Consolidated Statements of Cash Flows 
(in thousands) 

  Year Ended December 31,  

2020 

2019 

Cash flows from operating activities: 

Net loss . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .     $  (107,633)  $  (62,717)
Adjustments to reconcile net loss to net cash used in operating 

activities: 
Accretion of liability related to sale of future royalties . . . . . . . . . .    
Accretion of debt discount and amortization of issuance costs  . . .    
Depreciation and amortization  . . . . . . . . . . . . . . . . . . . . . . . . . . . . .    
Share based compensation expense  . . . . . . . . . . . . . . . . . . . . . . . . .    
Foreign currency adjustments . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .    
Changes in operating assets and liabilities: 

 10,778  
 248  
 192  
 14,615  
 (14,573) 

 8,360 
 — 
 127 
 7,578 
 4,006 

Prepaid expenses and other current assets . . . . . . . . . . . . . . . . . . .    
Other assets  . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .    
Accounts payable . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .    
Accrued expenses  . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .    
Other current and long-term liabilities . . . . . . . . . . . . . . . . . . . . . .    
Net cash used in operating activities . . . . . . . . . . . . . . . . . . . . . .    

 (508) 
 575  
 275  
 (4,774) 
 (224) 
   (101,029) 

 (6,331)
 (360)
 577 
 (4,095)
 (64)
    (52,919)

Cash flows from investing activities: 

Purchase of property and equipment . . . . . . . . . . . . . . . . . . . . . . . . . .    
Net cash used in investing activities  . . . . . . . . . . . . . . . . . . . . . .    

 (78) 
 (78) 

 (540)
 (540)

Cash flows from financing activities: 

 20,775 
Proceeds from issuance of common stock, net of issuance costs . . .    
Proceeds from royalty agreement, net of issuance costs . . . . . . . . . .    
 — 
 — 
Proceeds from issuance of debt, net of issuance costs . . . . . . . . . . . .    
Proceeds from exercise of options and vesting of RSUs . . . . . . . . . .    
 2,729 
 23,504 
Net cash provided by financing activities . . . . . . . . . . . . . . . . . .    
 (2,087)
Effect of exchange rate changes on cash and cash equivalents . . . . . .    
    (32,042)
Net increase (decrease) in cash and cash equivalents . . . . . . . . . . . . . .    
Cash and cash equivalents—beginning of period . . . . . . . . . . . . . . . . .    
   163,885 
Cash and cash equivalents—end of period . . . . . . . . . . . . . . . . . . . . . .     $  251,272   $ 131,843 
Supplemental disclosures of cash and non-cash activities: 

    193,265  
 14,750  
 9,487  
 2,774  
    220,276  
 260  
    119,429  
    131,843  

Warrants issued with long-term debt. . . . . . . . . . . . . . . . . . . . . . . . . .     $ 
Cash paid for income taxes  . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .     $ 
Right of use assets and lease obligations recorded upon amended 

 113   $
 —   $

 — 
 20 

lease agreements  . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .     $ 

—   $

 4,747 

See accompanying Notes to Consolidated Financial Statements. 

F-7 

 
 
 
 
 
 
 
 
 
 
    
    
 
 
 
 
 
  
 
   
  
  
  
  
  
  
  
  
 
 
 
  
 
  
  
  
  
  
  
  
  
  
  
  
 
  
 
  
  
  
  
  
 
  
 
  
  
  
  
 
 
 
 
  
  
  
 
  
   
  
  
 
 
 
Albireo Pharma, Inc. 

Notes to Consolidated Financial Statements 

1. Summary of significant accounting policies and basis of presentation 

Organization  

Albireo Pharma, Inc. (the Company), is a clinical-stage biopharmaceutical company focused on the development 

and commercialization of novel bile acid modulators to treat orphan pediatric liver diseases and other liver and 
gastrointestinal diseases and disorders. The Company’s clinical pipeline includes a Phase 3 product candidate, a Phase 2 
product candidate, and elobixibat, which is approved in Japan for the treatment of chronic constipation. Odevixibat, the 
Company’s Phase 3 lead product candidate, is in development initially for the treatment of patients with progressive 
familial intrahepatic cholestasis (PFIC), a rare, life-threatening genetic disorder affecting young children. 

Basis of presentation 

These Consolidated Financial Statements have been prepared in conformity with generally accepted accounting 

principles in the United States (U.S. GAAP). Any reference in these notes to applicable guidance is meant to refer to the 
authoritative U.S. GAAP as found in the Accounting Standards Codification (ASC) and Accounting Standards Update 
(ASU) of the Financial Accounting Standards Board (FASB).  

Principles of consolidation 

The accompanying Consolidated Financial Statements include the accounts of the Company including its direct or 
indirect wholly owned subsidiaries. All intercompany balances and transactions have been eliminated in consolidation.  

Foreign currency translation 

Functional and presentation currency 

Items included in the financial statements of each entity comprising the Company are measured using the currency 

of the primary economic environment in which the entity operates (the functional currency).  

Transactions and balances 

Foreign currency transactions in each entity comprising the Company are remeasured into the functional currency 

of the entity using the exchange rates prevailing at the respective transaction dates. Foreign exchange gains and losses 
resulting from the settlement of such transactions and from the remeasurement at year-end exchange rates of monetary 
assets and liabilities denominated in foreign currencies are recognized within Other operating (income) expense, net 
except for changes in the liability related to the sale of future royalties which are recorded in Other non-operating 
(income) expense, net in the Consolidated Statements of Operations. 

The results and financial position of the Company’s subsidiaries’ that have a functional currency different from the 

USD are translated as follows: 

a. 

b. 

assets and liabilities presented are translated at the closing exchange rate as of December 31, 2020 and 2019; 

income and expenses for the statements of operations and comprehensive loss are translated at average 
exchange rates that are relevant for the respective periods for which the income and expenses occurred; and 

c. 

significant transactions use the exchange rate on the date of the transaction; 

F-8 

All resulting exchange differences arising from such translations are recognized directly in other comprehensive 

income (loss) and presented as a separate component of equity. 

Use of estimates 

The preparation of financial statements in conformity with U.S. GAAP requires management to make estimates 

and assumptions that affect the amounts of assets, liabilities, revenues and expenses reported in the financial statements 
and accompanying notes. Management must apply significant judgment in this process. On an ongoing basis, the 
Company evaluates its estimates and assumptions, including but not limited to accruals, realizability of deferred tax 
assets and the accretion of interest on the monetization liability. Actual results could materially differ from these 
estimates. 

Segment information 

The Company’s entire business is managed by a single management team, which reports to the chief executive 

officer. The chief executive officer is the chief operating decision maker. The Company has determined it has one 
operating segment as its chief operating decision maker allocates resources and assesses the performance of the business 
at this level. Accordingly, the Company has one reporting segment, which is the research and development of novel 
treatments for liver and gastrointestinal diseases and disorders. 

Cash and cash equivalents 

The Company considers all short-term highly liquid investments with an original maturity at the date of purchase 

of three months or less to be cash equivalents. 

Concentration of risk 

Credit risk 

Financial instruments that potentially subject the Company to significant concentration of credit risk consist 
primarily of cash and cash equivalents. For banks and financial institutions, only independent financial institutions with 
a high credit rating are utilized. The Company’s current license agreement is with an established and reputable 
pharmaceutical company and, historically, the Company has not had any material collection risk related to its accounts 
receivable. 

Concentration of revenue  

The Company generally does not require collateral or other security in support of accounts receivable.  All revenue 

and receivables relate to one licensee. 

Property and equipment, net 

Property and equipment, including leasehold improvements, are recorded at cost and depreciated when placed into 

service using the straight-line method, based on their estimated useful lives as follows:  

Asset Classification 
IT Equipment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .   
Vehicles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .   
Leaseholds improvements  . . . . . . . . . . . . . . . . . . . . . . . . . . . .    Lesser of useful life or lease term
Furniture and fixtures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .   

5-7 

Estimated Useful Life 
(in years) 
3-5 
5 

F-9 

 
 
 
 
 
     
 
Property and equipment purchased for less than $10,000 are expensed immediately. Costs for repairs and 

maintenance are expensed as incurred. 

Gains and losses on disposals of equipment are determined by comparing the proceeds with the carrying amount 

and are recognized within Other operating (income) expense, net in the Consolidated Statements of Operations. 

Impairment of long-lived assets 

Long-lived assets are reviewed for impairment whenever events or changes in circumstances indicate that the 
carrying amount of such assets may not be recoverable. In such instances, the recoverability of assets to be held and used 
is measured first by a comparison of the carrying amount of an asset group to future undiscounted net cash flows 
expected to be generated by the assets. If such assets are considered to be impaired, an impairment loss would be 
recognized if the carrying amount of the asset exceeds the fair value of the asset. There were no impairments recorded 
for the years ended December 31, 2020 and 2019. 

Research and development expenses 

Research and development costs are expensed as incurred and include primarily salaries, benefits and other staff-

related costs; clinical trial and related clinical manufacturing costs; contract services and other outside costs. 

The Company’s nonclinical studies and clinical trials are performed by third-party contract research organizations 

(CROs). Some of these expenses are billed monthly for services performed, while others are billed based upon 
milestones achieved. For nonclinical studies, the significant factors used in estimating accruals include the percentage of 
work completed to date and contract milestones achieved. For clinical trial expenses, the significant factors used in 
estimating accruals include the number of patients enrolled and percentage of work completed to date and contract 
milestones achieved. The Company’s estimates are highly dependent upon the timeliness and accuracy of the data 
provided by the respective CROs regarding the status of the contracted activity, with adjustments made when deemed 
necessary. 

Revenue recognition 

Milestone Payments  

At the inception of each arrangement that includes development milestone payments, the Company evaluates 

whether the milestones are considered probable of being achieved and estimates the amount to be included in the 
transaction price using the most likely amount method. If it is probable that a significant revenue reversal would not 
occur, the associated milestone value is included in the transaction price. Milestone payments that are not within the 
control of the Company or the licensee, such as regulatory approvals, are not considered probable of being achieved until 
those approvals are received. The Company evaluates factors such as the scientific, clinical, regulatory, commercial, and 
other risks that must be overcome to achieve the particular milestone in making this assessment. There is considerable 
judgment involved in determining whether it is probable that a significant revenue reversal would not occur. At the end 
of each subsequent reporting period, the Company reevaluates the probability of achievement of all milestones subject to 
constraint and, if necessary, adjusts its estimate of the overall transaction price. Any such adjustments are recorded on a 
cumulative catch-up basis, which would affect revenues and earnings in the period of adjustment.  

Royalties  

For arrangements that include sales-based royalties, including milestone payments based on a level of sales, and 
the license is deemed to be the predominant item to which the royalties relate, the Company recognizes revenue at the 
later of (i) when the related sales occur, or (ii) when the performance obligation to which some or all of the royalty has 
been allocated has been satisfied (or partially satisfied). 

F-10 

In 2012, the Company entered into a license agreement (the Agreement) with EA Pharma Co., Ltd. (EA Pharma) 

to develop a select product candidate (elobixibat) for registration and subsequent commercialization in select markets. In 
conjunction with the Agreement, the Company granted EA Pharma an exclusive license to its intellectual property for 
development and commercialization activities in the designated field and territories. The Company has completed all of 
its performance obligations.  

As of December 31, 2020, the Company is eligible to receive an additional regulatory-based milestone payment 
under the Agreement of $5.3 million if a specified regulatory event is achieved for elobixibat. The cash payments and 
any other payments for milestones and royalties from EA Pharma are non-refundable, non-creditable and not subject to 
set-off. 

The Agreement will continue until the last royalty period for any product in the territory, which is defined as the 
period when there are no remaining patent rights or regulatory exclusivity in place for any products subject to royalties. 
EA Pharma may terminate the Agreement at will upon 180 days’ prior written notice to the Company. Either party may 
terminate the Agreement for the other party’s uncured material breach or insolvency and in certain other circumstances 
agreed to by the parties. 

Monetization of Future Royalties  

In December 2017, the Company entered into a royalty interest acquisition agreement (RIAA) with HealthCare 
Royalty Partners III, L.P. (HCR) pursuant to which it sold to HCR the right to receive all royalties from sales in Japan 
and sales milestones achieved from any covered territory potentially payable to the Company under the Agreement, up 
to a specified maximum “cap” amount of $78.8 million, based on the funds the Company received from HCR. In 
January 2018, the Company received $44.5 million from HCR, net of certain transaction expenses, under the RIAA. On 
June 8, 2020, the parties entered into an amendment to the RIAA pursuant to which HCR agreed to pay the Company an 
additional $14.8 million, net of certain transactions expenses, in exchange for the elimination of the (i) $78.8 million cap 
amount on HCR’s rights to receive royalties on sales in Japan and sales milestones for elobixibat in certain other 
territories that may become payable by EA Pharma and (ii) the $15.0 million payable to the Company if a specified sales 
milestone is achieved for elobixibat in Japan. The Company is obligated to make royalty interest payments to HCR 
under the RIAA only to the extent it receives future Japanese royalties, sales milestones or other specified payments 
from EA Pharma. Although the Company sold its rights to receive royalties from the sales of elobixibat in Japan, as a 
result of its ongoing involvement in the cash flows related to these royalties, the Company will continue to account for 
these royalties as revenue. Upon receipt of the payments from HCR the Company recorded net cash totaling $59.3 
million as a liability related to sale of future royalties (royalty obligation). The royalty obligation will be amortized using 
the effective interest rate method. 

The following table shows the activity within the liability account for the year ended December 31, 2020: 

Liability related to sale of future royalties—beginning balance  . . . . . . . . . . . . . . . . . . . . . . .      $ 
Accretion of interest expense on liability related to royalty monetization . . . . . . . . . . . . . . .    
Proceeds from sale of future royalties, net  . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .    
Repayment of the liability  . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .    
Liability related to sale of future royalties—ending balance . . . . . . . . . . . . . . . . . . . . . . . . . .      $ 
Less current portion classified within accrued expenses  . . . . . . . . . . . . . . . . . . . . . . . . . . . . .    
Net ending liability related to sale of future royalties . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .      $ 

December 31, 2020 
(in thousands) 

 55,144 
10,778 
14,750 
 (12,078)
 68,594 
 (2,700)
 65,894 

The Company records estimated royalties due for the current period in accrued other expenses until the payment is 
received from EA Pharma at which time the Company then remits payment to HCR. In order to determine the accretion 
of the royalty obligation, the Company is required to estimate the total amount of future royalty payments to be received 
and submitted to HCR. The sum of these amounts less the $59.3 million proceeds the Company received will be 
recorded as interest expense over the life of the royalty obligation. At December 31, 2020, the Company’s estimate of its 
total interest expense resulted in an annual effective interest rate of approximately 19.0%.  

F-11 

 
 
 
 
 
    
 
    
 
 
 
 
 
The Company periodically assesses the estimated royalty payments to HCR and to the extent such payments are 
greater or less than its initial estimates or the timing of such payments is materially different than its original estimates, 
the Company will prospectively adjust the accretion of interest on the royalty obligation. There are a number of factors 
that could materially affect the amount and the timing of royalty payments, most of which are not within the Company’s 
control. Such factors include, but are not limited to, the rate of elobixibat prescriptions, the number of doses 
administered, the introduction of competing products, manufacturing or other delays, patent protection, adverse events 
that result in governmental health authority imposed restrictions on the use of the drug products, significant changes in 
foreign exchange rates as the royalties remitted to HCR are in U.S. dollars while sales of elobixibat are in Japanese yen, 
and sales never achieving forecasted numbers, which would result in reduced royalty payments and reduced non-cash 
interest expense over the life of the royalty obligation. To the extent future royalties result in an amount less than the 
liability, the Company is not obligated to fund any such shortfall. 

Stock-based compensation 

The Company accounts for stock-based compensation awards in accordance with FASB ASC Topic 718, 
Compensation—Stock Compensation (ASC 718). ASC 718 requires all stock-based payments, including grants of stock 
options, to be recognized in the consolidated statements of operations based on their respective fair values whether 
granted to employees or non-employees. 

The fair value of the Company’s stock options has been determined using the Black-Scholes option pricing model, 

which requires the input of subjective assumptions, including (i) the expected stock price volatility, (ii) the expected 
term of the award, (iii) the risk-free interest rate and (iv) expected dividends. For the years ended December 31, 2020 
and December 31, 2019, due to the lack of historical and implied volatility data of the Company’s common stock and 
equivalents, the expected volatility has been estimated based on the historical volatilities of peer companies in the 
Company’s industry that are publicly traded.  The Company selected companies that it considers to have comparable 
characteristics to the Company, including enterprise value, risk profiles and position within the industry and with 
historical share price information sufficient to meet the expected term of the stock options. The historical volatility data 
has been computed using the daily closing prices for the selected companies. 

Due to the lack of sufficient historical data, the Company used the “simplified” method, whereby the expected 
term equals the arithmetic average of the vesting term and the original contractual term of the award, to determine the 
expected term of stock options.  

The Company records compensation expense for service-based awards over the vesting period of the award on a 

straight-line basis. For awards with service and performance based conditions, compensation related to the performance-
based vesting conditions is recognized when achievement of the performance condition is considered probable and the 
compensation expense related to the service condition is recorded using the accelerated method. 

Modifications to stock-based awards are treated as an exchange of the original award for a new award with total 

compensation equal to the grant-date fair value of the original award plus any incremental value of the modification. The 
incremental value is based on the excess of the fair value of the modified award over the fair value of the original award 
immediately before the modification. 

Employee benefits 

Pension obligations 

The Company has a defined contribution pension plan for its Sweden-based employees whereby the Company 

pays contributions to employee benefit or insurance plans on a mandatory, contractual or voluntary basis.  

The Company paid $0.7 million and $0.4 million to the plans for the years ended December 31, 2020 and 2019, 

respectively.  

F-12 

401(k) 

The Company has a 401(k) retirement plan in which all U.S.-based employees are eligible to participate. The 

Company contributed $0.5 million and $0.2 million to the plan for the years ended December 31, 2020 and 2019, 
respectively. The Company matches employee contributions to the plan, on a per employee basis, up to 4% of each 
employee’s wages for the years ended December 31, 2020 and 2019. 

Loss contingencies 

Loss contingencies are recorded as liabilities when it is probable that a liability has occurred and the amount of 
loss is reasonably estimable. Disclosure is required when there is a reasonable possibility that an ultimate loss will be 
material. Contingent liabilities are often resolved over long periods of time. Estimating probable losses requires analysis 
that often depends on judgments about potential actions by third parties, such as regulators.  

Income taxes 

The Company accounts for income taxes in accordance with ASC 740, Income Taxes (ASC 740). Deferred income 

taxes are recorded for the expected tax consequences of temporary differences between the tax basis of assets and 
liabilities for financial reporting purposes and amounts recognized for income tax purposes. The Company records a 
valuation allowance to reduce its deferred tax assets to the amount of future tax benefit that is more likely than not to be 
realized. 

Income tax expense consists of taxes currently payable and changes in deferred tax assets and liabilities calculated 

according to local tax rules. Deferred tax assets and liabilities are based on temporary differences that arise between 
carrying values used for financial reporting purposes and amounts used for taxation purposes of assets and liabilities and 
the future tax benefits of tax loss carry forwards. A deferred tax asset is recognized only to the extent that it is more 
likely than not that future taxable profits will be available against which the asset can be utilized. 

Significant judgment is required in determining any valuation allowance recorded against deferred tax assets. In 

assessing the need for a valuation allowance, the Company considers all available evidence for each jurisdiction 
including past operating results, estimates of future taxable income and the feasibility of ongoing tax planning strategies. 
In the event that the Company changes its determination as to the amount of deferred tax assets that can be realized, the 
Company will adjust its valuation allowance with a corresponding impact to income tax expense in the period in which 
such determination is made. 

The amount of deferred tax provided is calculated using tax rates in effect at the balance sheet date. The impact of 

tax law changes is recognized in periods when the change is enacted. 

A two-step approach is applied pursuant to ASC 740 in the recognition and measurement of uncertain tax positions 

taken or expected to be taken in a tax return. The first step is to determine if the weight of available evidence indicates 
that it is more likely than not that the tax position will be sustained in an audit, including resolution of any related 
appeals or litigation processes. The second step is to measure the tax benefit as the largest amount that is more than 50% 
likely to be realized upon ultimate settlement. 

The Company’s policy is to recognize interest and penalty expenses associated with uncertain tax positions as a 
component of income tax expense in the Consolidated Statements of Operations. As of the years ended December 31, 
2020 and 2019, the Company had no accrued interest or penalties related to uncertain tax positions and no amounts have 
been recognized in the Consolidated Statements of Operations. 

Net loss per share 

Basic net loss per share is calculated by dividing the net loss attributable to holders of common stock by the 
weighted average number of shares of common stock outstanding. Diluted net loss per share is calculated by dividing the 

F-13 

net loss attributable to holders of common stock by the weighted-average number of shares of common stock 
outstanding. If the Company were in a net income position, diluted net income per share would be calculated by dividing 
the net income attributable to holders of common stock by the weighted-average number of shares of common stock plus 
dilutive common stock equivalents outstanding. 

Goodwill  

Goodwill is the excess of the purchase price in a business combination over the fair value of identifiable net assets 

acquired. Goodwill has an indefinite life and is subject to periodic testing for impairment. 

Goodwill is assessed at least annually, but also whenever events or changes in circumstances indicate the carrying 
value may not be recoverable. Factors that could trigger an impairment review, include: (a) significant underperformance 
relative to historical or projected future operating results; (b) significant changes in the manner of or use of the acquired 
assets or the strategy for the Company’s overall business; (c) significant negative industry or economic trends; 
(d) significant decline in the Company’s stock price for a sustained period; and (e) a decline in the Company’s market 
capitalization below net book value. 

The Company conducts an impairment assessment on October 1 each year taking a quantitative test which 
compares the fair value to the net carrying value, and records an impairment of goodwill to the extent that the net 
carrying value exceeds the fair value. During the fourth quarter of 2020 and 2019, we completed our annual impairment 
assessment and concluded that goodwill was not impaired in any of those years. 

Recently adopted accounting pronouncements 

In August 2018, the FASB issued ASU 2018-15, “Intangibles – Goodwill and Other – Internal-Use Software 
(Subtopic 350-40): Customer’s Accounting for Implementation Costs Incurred in a Cloud Computing Arrangement That 
Is a Service Contract.” (ASU 2018-15). This standard aligns the requirements for capitalizing implementation costs 
incurred in a hosting arrangement that is a service contract with the requirements for capitalizing implementation costs 
incurred to develop or obtain internal-use software (and hosting arrangements that include an internal-use software 
license). The guidance also requires the entity to expense the capitalized implementation costs of a hosting arrangement 
that is a service contract over the term of the hosting arrangement, which includes reasonably certain renewals. The 
Company adopted this guidance in the first quarter of 2020 on a prospective basis and there was no material impact on 
its consolidated financial statements. 

2. Fair value of financial instruments 

In measuring fair value, the Company evaluates valuation techniques such as the market approach, the income 

approach and the cost approach. A three-level valuation hierarchy, which prioritizes the inputs to valuation techniques 
that are used to measure fair value, is based upon whether such inputs are observable or unobservable. 

Observable inputs reflect market data obtained from independent sources, while unobservable inputs reflect 

market assumptions made by the reporting entity. The three-level hierarchy for the inputs to valuation techniques is 
briefly summarized as follows: 

Level 1—Observable inputs such as quoted prices (unadjusted) for identical instruments in active markets; 

Level 2—Observable inputs such as quoted prices for similar instruments in active markets, quoted prices for 
identical or similar instruments in markets that are not active, or model-derived valuations whose significant inputs 
are observable for substantially the full term of the assets or liabilities; and 

Level 3—Unobservable inputs that reflect the reporting entity’s estimate of assumptions that market participants 
would use in pricing the asset or liability. 

F-14 

 
 
 
3. Property and equipment, net 

Property and equipment, net consisted of the following (in thousands): 

Year Ended 
December 31,  

2020 

2019 

IT equipment   . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .     $ 
Vehicles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .       
Leasehold improvements . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .       
Furniture and fixtures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .       
Total property and equipment, gross . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .       
Less: Accumulated depreciation  . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .       
Total property and equipment, net  . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .     $ 

318 
  $ 
 —       
58       
453       
 829       
 (351)  
 478 

  $ 

 306 
 81 
 58 
 378 
 823 
 (226)
 597 

Depreciation expense for the years ended December 31, 2020 and 2019 was $0.2 million and $0.1 million, 

respectively. 

4. Accrued expenses 

Accrued expenses consisted of the following (in thousands): 

Accrued payroll and benefits . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .      $  8,492    $  2,990 
Accrued professional fees . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .        
 359 
Accrued development costs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .          5,968         2,887 
Accrued liability related to monetization of future royalties . . . . . . . . . . .          2,700         6,430 
Accrued other  . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .          1,242       
 820 
Total accrued expenses. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .      $ 19,051    $ 13,486 

 649       

Year Ended 
December 31,  

2020 

2019 

5. Commitments and contingencies 

Operating lease commitments 

The Company’s portfolio of commercial real estate leases consists of office space for its corporate headquarters in 
Boston, Massachusetts and for administrative space in Göteborg, Sweden, both of which are accounted for as operating 
leases. These leases include renewal rights and, as for the corporate headquarters lease, escalating payments. On 
March 28, 2019, the Company entered into an amendment to the Boston, Massachusetts lease to (i) replace the 
Company’s prior office space with a new office space that is being leased from the same landlord and (ii) extend the 
term of the lease through October 31, 2026. The new leased space contains monthly lease payments subject to annual 
escalations of $1.00 per square foot for the remaining term of the lease with the Company obligated to make 
approximately $4.2 million of aggregate lease payments over the term of the lease, or approximately $900,000 annually. 

The Company’s lease in Göteborg, Sweden includes the rental of office space. This lease includes annual rent 
escalations based on the changes in the Swedish Consumer Price Index.  This lease renews automatically for consecutive 
three year terms unless notice of non-renewal is given by either party at least nine months prior to the end of the current 
term of February 2022.  

As of December 31, 2020 the net balance of ROU assets totaled $4.1 million and was classified within other non-

current assets. Operating lease expense was $1.2 million and $0.7 million for the years ended December 31, 2020 and 
2019, respectively.  There were no short-term lease or variable lease costs incurred for the either of the years ended 

F-15 

 
 
 
 
 
 
 
 
 
 
 
 
     
     
  
 
 
 
 
 
 
 
 
 
 
 
 
 
    
    
 
 
 
 
December 31, 2020 and 2019.  As of December 31, 2020, the weighted average remaining lease term for the Company’s 
operating leases was 5.68 years. As of December 31, 2020, the weighted- average discount rate was 9.95%.  

The following table summarizes the Company’s significant contractual obligations under operating leases as of 

payment due date by period at December 31, 2020: 

  Total Minimum Lease Payments

(in thousands) 

2021 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .      
2022 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .      
2023 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .      
2024 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .      
2025 and beyond . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .      
Total minimum lease payments . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .     $ 
Less imputed interest  . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .      
Total lease liability . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .     $ 

Reported as: 
Other current liabilities . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .     $ 
Other long-term liabilities . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .      
Total lease liabilities . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .     $ 

 1,028 
 906 
 921 
 936 
 1,756 
 5,547 
 (1,344)
 4,203 

 (640)
 (3,563)
 (4,203)

As of December 31, 2020, future minimum commitments under facility operating leases were $4.2 million. For the 

years ended December 31, 2020 and 2019, the Company paid $1.0 million and $0.4 million in cash related to operating 
leases, respectively. 

Agreements with CROs 

As of December 31, 2020, the Company had various agreements with CROs for the conduct of specified research 
and development activities and, based on the terms of the respective agreements, the Company is contractually obligated 
to make future payments of up to $7.2 million upon the completion of contracted work. 

Legal Contingency   

On November 18, 2019 we entered into a settlement agreement with Ferring resolving to the parties’ mutual 
satisfaction a litigation we brought in New York State Supreme Court concerning the License Agreement, dated July 2, 
2012, between us and Ferring. The parties have exchanged mutual releases and filed a stipulation voluntarily 
discontinuing the case with prejudice. Albireo received net proceeds of $3.7 million included in other non-operating 
income in the consolidated statements of operations. 

6. Note Payable 

2020 Loan and Security Agreement 

On June 8, 2020, the Company entered into a Loan and Security Agreement (the Loan and Security Agreement) 

with the several banks and other financial institutions or entities from time to time parties to the Loan and Security 
Agreement, as lenders (collectively, referred to as the “Lender”), and Hercules Capital, Inc., in its capacity as 
administrative agent and collateral agent for itself and Lender (in such capacity, the “Agent” or “Hercules”) pursuant to 
which term loans of up to an aggregate principal amount of up to $80.0 million (the “Term Loans”) are available to the 
Company. The Loan Agreement provides for (i) an initial term loan advance of $10.0 million, which closed on June 8, 
2020, (ii) subject to the achievement of certain initial performance milestones (“Performance Milestone I”), a right of the 
Company to request that the Lender make additional term loan advances to the Company in an aggregate principal 

F-16 

 
 
 
 
 
 
 
    
 
   
 
    
 
 
 
amount of up to $20.0 million from January 1, 2021 through December 15, 2021 in minimum increments of $10.0 
million, and (iii) subject to the Lender’s investment committee’s sole discretion, a right of the Company to request that 
the Lender make additional term loan advances in an aggregate principal amount of up to $45.0 million through 
March 31, 2022 in minimum increments of $5.0 million. The Company is required to pay an end of term fee (“End of 
Term Charge”) equal to 6.95% of the aggregate principal amount of the Term Loans advances upon repayment.  

The Term Loans mature on January 1, 2024, which is extendable to June 1, 2024 upon achievement of 

Performance Milestone I (the “Maturity Date”). 

The Term Loan bears interest at an annual rate equal to the greater of 9.15% and 9.15% plus the prime rate of 

interest minus 3.25%. Borrowings under the Loan and Security Agreement are repayable in monthly interest-only 
payments through January 1, 2022 and extendable to (i) July 1, 2022 upon achievement of Performance Milestone I and 
(ii) July 1, 2023 upon achievement of certain additional performance milestones. After the interest-only payment period, 
borrowings under the Loan and Security Agreement are repayable in equal monthly payments of principal and accrued 
interest until the Maturity Date. At the Company’s option, the Company may elect to prepay all, but not less than all, of 
the outstanding term loan by paying the entire principal balance and all accrued and unpaid interest thereon plus a 
prepayment charge equal to the following percentage of the principal amount being prepaid: 2.0% of the principal 
amount outstanding if the prepayment occurs after the first nine months following the Closing Date, but on or prior to 
24 months following the Closing Date, and 1.0% of the principal amount outstanding at any time thereafter but prior to 
the Maturity Date. 

In connection with the Loan Agreement, the Company granted Agent a security interest senior to any current and 

future debts and to any security interest, in all of the Company’s right, title, and interest in, to and under all of 
Company’s property and other assets, and certain equity interests and accounts of Albireo AB, subject to limited 
exceptions including the Borrower’s intellectual property. The Loan Agreement also contains certain events of default, 
representations, warranties and non-financial covenants of the Company. 

The debt discount and issuance costs are being accreted to the principal amount of debt and being amortized from 
the date of issuance through the Maturity Date to interest expense using the effective-interest rate method.  The effective 
interest rate of the outstanding debt under the Loan Agreement is approximately 15.3%. 

As of December 31, 2020 the carrying value of the note payable consists of the following: 

December 31, 2020 
(in thousands) 

Note payable, including End of Term Charge . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .   
Debt discount, net of accretion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .   
Note payable net of discount, long-term . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .   

$ 

 10,695 
 (1,074)
 9,621 

During the year ended December 31, 2020, the Company recognized $0.8 million, of interest expense related to 
the Loan Agreement. No interest expense was associated with the Loan Agreement for the year ended December 31, 
2019. 

Through December 31, 2020, the Company borrowed $10.0 million under the Loan Agreement and incurred $1.3 

million of debt discount and issuance costs inclusive of facility fees, legal fees, End of Term Charge and fair value of the 
warrant.   

F-17 

 
 
 
 
 
 
 
 
 
 
 
 
Estimated future principal payments due under the Loan Agreement, including the contractual End of Term 

Charge are as follows as of December 31, 2020: 

2021 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .    $ 
2022 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .   
2023 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .   
2024 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .   

 — 
 4,553 
 4,994 
 1,148 

As of December 31, 2020, based on Level 3 inputs and the borrowing rates available to the Company for loans 

with similar terms and consideration of the Company’s credit risk, the carrying value of the Company’s variable interest 
rate debt, excluding unamortized debt issuance costs, approximates fair value. 

  Note Principal Payments 

(in thousands) 

Warrants 

Under the Loan and Security Agreement, the Company agreed to issue to Hercules warrants (the “Warrants”) to 

purchase a number of shares of the Company’s common stock, par value $0.01 per share (the “Common Stock”) equal to 
1% of the aggregate amount of the Term Loans that are funded, as such amounts are funded. On the Closing Date, the 
Company issued a Warrant for 5,311 shares of Common Stock. The Warrants will be exercisable for a period of seven 
years from the date of the issuance of each Warrant at a per-share exercise price equal to $18.83, subject to certain 
adjustments as specified in the Warrants. In addition, the Company has granted to the holders of the Warrants certain 
registration rights. Specifically, the Company has agreed to use its commercially reasonable efforts to (i) file registration 
statements with the U.S. Securities and Exchange Commission within 60 days following the date of the issuance of each 
Warrant for purposes of registering the shares of Common Stock issuable upon exercise of the Warrants for resale by 
Hercules, and (ii) cause the registration statement to be declared effective as soon as practicable after filing, and in any 
event no later than 180 days after the date of the issuance of each Warrant. 

The Company accounted for the Warrants as equity instruments since they were indexed to the Company’s 

common stock and met the criteria for classification in stockholders’ equity. The relative fair value of the Warrants 
related to the first tranche funding was approximately $0.1 million, and was treated as a discount to the Term Loans. 
This amount is being amortized to interest expense using the effective interest method over the life of the Term Loans. 
The Company estimated the fair value of the Warrants using the Black-Scholes option-pricing model. 

7. Goodwill 

There was no change in goodwill during 2020 or 2019. 

8. Net loss per share 

The following table sets forth the computation of Basic loss per share and Diluted loss per share (in thousands, 

except for share and per share data): 

Basic and Diluted loss per share: 

Numerator 

Net loss . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .      $ 

 (107,633)   $ 

 (62,717)

Denominator 

Year Ended  

2020 

2019 

Weighted average number of shares outstanding  . . . . . . . . .          15,983,058        12,437,742 
 (5.04)

Basic and Diluted loss per share: . . . . . . . . . . . . . . . . . . . . . . . .      $ 

 (6.73)   $ 

F-18 

 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
     
     
 
 
    
    
    
     
     
 
The following outstanding common stock equivalents were excluded from the computation of Diluted loss per 

share for the periods presented because including them would have been anti-dilutive: 

Options to purchase common stock, RSUs and warrants . . . . . . . . . . .      2,413,037 

2020 

2019 
 1,833,920 

Year Ended  

9. Income taxes  

Effects of the Tax Cuts and Job Act 

The Company has had an overall net operating loss position since its inception. There was no current or deferred 

tax provision for the years ended December 31, 2020 and 2019, respectively. 

For the years ended December 31, 2020 and 2019, the components of loss before income taxes were as follows (in 

thousands): 

U.S.  . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .     $  (86,041)   $  (29,337)
Foreign . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .         (21,592)      (33,380)
Total . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .     $ (107,633)   $  (62,717)

A reconciliation of the U.S. statutory income tax rate to the consolidated effective income tax rate was as follows: 

Year Ended 
December 31, 

2020 

2019 

Year Ended 
December 31, 

      2020 

2019 

U.S. statutory income tax rate  . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .    
Stock compensation  . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .    
State taxes, net of federal tax effect . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .    
Change in valuation allowance  . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .    
Change in deferred tax rate . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .    
Other items  . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .   
Effective income tax rate . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .    

 21  %  
 —   
 5   
 (26) 
 —   
 —   
 0.0  %  

 21 % 
 (1) 
 3  
 (20) 
 (2) 
 (1) 
 0.0 % 

F-19 

 
 
 
 
 
  
 
 
    
    
 
 
 
 
 
 
 
 
 
    
 
 
 
    
     
    
 
 
 
 
 
 
 
    
 
 
 
 
 
    
     
 
 
Deferred taxes are recognized for temporary differences between the bases of assets and liabilities for financial 
statement and income tax purposes. The tax effect of temporary differences that give rise to significant portions of the 
deferred tax assets are as follows (in thousands): 

Year Ended 
December 31,  

2020 

2019 

Deferred tax assets: 

Tax loss carryforwards . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .     $  59,790     $  35,120 
 130 
Research and development credits  . . . . . . . . . . . . . . . . . . . . . . . . . . . . .       
 530 
Accrued expenses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .       
 2,090 
Stock compensation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .       
Interest carryforwards  . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .   
 1,910 
Other . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .       
 1,410 
Total gross deferred tax assets  . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .         72,180        41,190 
Valuation allowance . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .        (71,040)      (39,910)
Total deferred tax assets . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .     $ 
 1,140    $  1,280 
Deferred tax liabilities: 

 130      
 1,870      
 4,750      
 4,420  
 1,220      

Intangible assets . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .     $ 
Right of use asset . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .       
Total deferred tax liabilities  . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .       
Net deferred tax assets  . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .     $ 

 (20)    $
 (1,120)     
 (1,140)     
 —     $

 (30)
 (1,250)
 (1,280)
 — 

A valuation allowance is recorded against deferred tax assets if it is more likely than not that some or all of the 

deferred tax assets will not be realized. Due to the uncertainty surrounding the realization of the favorable tax attributes 
in future returns, the Company has recorded a valuation allowance of $71.0 million and $39.9 million at December 31, 
2020 and 2019, respectively. The change in valuation allowance for the years ended December 31, 2020 and 2019, were 
$31.1 million and $12.6 million, respectively.  

As of December 31, 2020, deferred tax assets related to net operating loss (NOL) carryforwards were $59.8 

million, which may be used subject to certain limitations to offset future taxable income, if any. The NOL includes 
approximately $1.5 million for U.S. federal tax purposes that are subject to expire in 2036 and approximately $94.2 
million of federal net operating losses which have no expiration date and can be carried forward indefinitely. The 
Company has state NOLs of approximately $244.4 million which may be available to offset future income tax liabilities 
and that expire at various dates through 2039. Additional NOLs of approximately $120.5 million were generated in 
various non-U.S. jurisdictions and will not expire. A valuation allowance has been established on the NOL 
carryforwards as it is uncertain as to whether future taxable income will be generated to utilize such NOLs.  

Utilization of the NOL and credit carryforwards may be subject to a substantial annual limitation due to ownership 
change limitations that have occurred previously or that could occur in the future as provided by Section 382 of the Code 
and similar state and foreign provisions. These ownership changes may limit the amount of NOL and credit 
carryforwards that can be utilized annually to offset future taxable income and tax, respectively. In general, an 
ownership change, as defined by Code Section 382, results from transactions increasing the ownership of certain 
shareholders or public groups in the stock of a corporation by more than 50 percentage points over a three-year period. 
During 2016, the Company completed an analysis to assess and concluded that an ownership change within the meaning 
of Code Section 382 occurred. The analysis has not yet been updated beyond 2016. 

The Company’s policy is for any earnings of non-U.S. subsidiaries to be indefinitely invested outside the United 

States on the basis of estimates that future domestic cash generation will be sufficient to meet future domestic cash needs 
and the Company’s specific plans for reinvestment of those subsidiary earnings, if any. 

F-20 

 
 
 
 
 
 
 
 
 
 
 
 
    
     
       
       
 
 
     
     
 
Uncertain tax positions 

The Company accounts for uncertain tax positions under the recognition and measurement criteria of ASC 740-10. 

For those tax positions for which it is more likely than not that a tax benefit will be sustained, the Company records the 
largest amount of tax benefit with a greater than 50% likelihood of being realized upon settlement with a taxing authority 
that has full knowledge of all relevant information. If the Company does not believe that it is not more likely than not 
that a tax benefit will be sustained, no tax benefit is recognized. As of December 31, 2020 and 2019, no uncertain tax 
positions have been recorded. Interest and penalties related to the settlement of uncertain tax positions, if any, will be 
reflected in income tax expense. The Company did not recognize any interest or penalties associated with unrecognized 
tax benefits in the accompanying consolidated financial statements. The Company does not expect any material changes 
to the unrecognized benefits within 12 months of the reporting date. Due to existence of the valuation allowance, future 
changes in the Company’s unrecognized tax benefits will not impact its effective tax rate. 

The Company files U.S. federal and state tax returns and has determined that its major tax jurisdictions are the 

United States and Massachusetts, as well as the United Kingdom and Sweden. The Company’s tax returns may be 
examined for certain tax jurisdictions back to December 31, 2016. 

The Company is subject to a territorial tax system under the Act, in which the Company is required to provide for 

tax on Global Intangible Low-Taxed Income (“GILTI”) earned by certain foreign subsidiaries. The Company has 
adopted an accounting policy to provide for the tax expense related to GILTI in the year the tax is incurred as a period 
expense. 

10. Stockholders’ equity 

Preferred Stock 

As of December 31, 2020, the Company has 50,000,000 shares of preferred stock authorized. There are no shares 

of preferred stock issued or outstanding. 

Financing 

2019 Sales Agreement 

In May 2019, we sold 637,367 shares of our common stock for net proceeds of approximately $20.8 million 

pursuant to an at the market offering.  

In February 2020, we completed an underwritten public offering of 2,190,750 shares of our common stock under a 

universal shelf registration statement for net proceeds of approximately $43.0 million. 

In May 2020, we filed a new universal shelf registration on Form S-3 with the SEC, pursuant to which we 

registered for sale up to $200.0 million of any combination of our common stock, preferred stock, debt securities, 
warrants, rights and/or units from time to time and at prices and on terms that we may determine. As of December 31, 
2020, $40.0 million of securities remain available for issuance under the shelf registration statement. 

In September 2020, the Company completed an underwritten public offering of 4,000,000 shares of its common 

stock. The Company received net proceeds from this offering of approximately $150.4 million, after deducting 
underwriting discounts and, commissions but before deducting offering expenses. 

F-21 

11. Stock-based Compensation 

The Company recognized stock-based compensation expense in the accompanying Consolidated Statements of 

Operations as follows (in thousands): 

General and administrative . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .     $   8,646     $  4,600 
 5,969        2,978 
Research and development . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .       
Total stock-based compensation  . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .     $  14,615     $  7,578 

A summary of the outstanding stock options as of December 31, 2020 is as follows: 

Year Ended  
December 31,  

2020 

2019 

Stock Options Outstanding 
  Weighted-   

  Number of    Price Per  

Shares 

  Weighted-  
Average 
  Average    Remaining   Aggregate 
Intrinsic 
  Exercise    Contractual 
  Value (in 
    thousands) 
 8.06   $  8,421 

Term 
(Years) 

     Share 

Outstanding—December 31, 2019 . . . . . . . . . . .    
Granted . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .     
Expirations/forfeitures . . . . . . . . . . . . . . . . . . .     
Exercises . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .     

 1,768,170    $  22.34  
 864,725     $  26.18   
 (133,992)   $  23.88   
 (127,080)   $  19.86   
Outstanding—December 31, 2020 . . . . . . . . . . .       2,371,823     $  23.89   
Exercisable—December 31, 2020 . . . . . . . . . . .       1,024,923     $  20.97  
Vested or expected to vest at—

 7.75    $ 32,764 
 6.39    $ 17,109 

December 31, 2020  . . . . . . . . . . . . . . . . . . . . .       2,371,823     $  23.89  

 7.75    $ 32,764 

Aggregate intrinsic value represents the difference between the fair value of the underlying common stock and the 

exercise price of outstanding, in-the-money options. 

As of December 31, 2020, the total unrecognized compensation expense related to unvested options was $17.4 

million, which the Company expects to recognize over a weighted average vesting period of 2.57 years. 

In determining the estimated fair value of the stock-based awards, the Company uses the Black-Scholes option 

pricing model and assumptions discussed below.  

The fair value of share option awards was estimated with the following assumptions: 

As of December 31,  
2020 
Price per share of common stock . . . . . . . . . . . .    $  15.62  -   
-   
Expected term (in years) . . . . . . . . . . . . . . . . . . .   
 6.1 
Risk-free interest rate . . . . . . . . . . . . . . . . . . . . .   
-   
 0.35 
Expected volatility  . . . . . . . . . . . . . . . . . . . . . . .   
 79.41  -   
0% 
Dividend rate . . . . . . . . . . . . . . . . . . . . . . . . . . . .  

  As of December 31,  

2019 

   $  16.92 -    37.86  
 5.6  -   
 1.37  -   

 43.65 
 6.1 
 6.1 
 2.6  % 
 1.7  %   
 92.9  %     81.04 -    144.9 % 

0%  

Restricted Stock Units 

Each Restricted Stock Units “RSU” award represents one share of common stock and each award vests 25% on 

the first anniversary and in equal quarterly installments thereafter. The costs of the awards, determined as the fair market 
value of the shares on the grant date, are expensed on a straight line basis over the length of the award.   

F-22 

 
 
 
 
 
 
 
 
 
 
 
 
 
     
     
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
    
     
    
    
    
 
 
 
 
 
     
  
     
 
  
  
 
 
 
 
 
A summary of outstanding RSU as of December 31, 2020 is as follows: 

  Weighted 
Average 

Non-vested and outstanding RSU balance at December 31, 2019  . . . . .    

Changes during the period: 
Granted . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .    
Expirations/forfeitures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .    
Vested . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .    
Non-vested and outstanding RSU balance at December 31, 2020 . . . .    

Employee Stock Purchase Plan 

      Shares 

 65,750    $ 

  Grant-Date 
      Fair Value 
 24.67 

 —      
 (7,188)     
 (25,497)    
  $ 
 33,065 

 — 
 20.93 
 25.36 
 24.95 

In June of 2018, the Company’s Board of Directors adopted the 2018 Employee Stock Purchase Plan (the Plan) 
that allows eligible employees to purchase shares of its common stock at a discount through payroll deductions.  The 
Plan was subsequently approved by shareholders, with 300,000 shares being available to be issued under the Plan.  

The Employee Stock Purchase Plan “ESPP” terms state implementation will be by a series of six-month offering 

periods, with a new offering period commencing on June 1 and December 1 of each year or the first business day 
thereafter. The initial Offering Period under the Plan began on December 1, 2018.  During the years ended December 31, 
2020 and 2019, 14,270 and 8,354 shares were purchased under the Plan, respectively. As of December 31, 2020, 
277,376 shares are available to be issued. The Plan is intended to qualify under the Internal Revenue Code of 1986, 
Section 423. ESPP expense for the years ended December 31, 2020 and 2019 was $0.1 million and $0.1 million, 
respectively. The expense is disclosed in the stock-based compensation expense table within this footnote. 

F-23 

 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
    
 
 
Directors

Stockholders and Stock Listing

David Chiswell, Ph.D.
Chairman of the Board of Directors, Albireo Pharma
Non­Executive Director, Avillion LLP

Ronald H.W. Cooper
President and Chief Executive Officer, Albireo Pharma

Michael Gutch, Ph.D.
Chief Business Officer and Chief Financial Officer, Entasis
Therapeutics

Roger A. Jeffs, Ph.D.
Co-Founder and Vice Chairman of Kriya Therapeutics

Anne Klibanski, M.D.
President and Chief Executive Officer,
Partners Healthcare

Stephanie S. Okey, M.S.
Former Senior Vice President, Head of North America, 
Rare Diseases, Genzyme, a Sanofi company

Davey S. Scoon
Non­Executive Director of the Board of Trustees,
Allianz Global Investors

Executive Officers

Ronald H.W. Cooper
President and Chief Executive Officer

Jan P. Mattsson, Ph.D.
Chief Scientific Officer

Simon N.R. Harford
Chief Financial Officer and Treasurer

Patrick T. Horn, M.D., Ph.D.
Chief Medical Officer

Martha J. Carter
Chief Regulatory Officer

Pamela Stephenson
Chief Commercial Officer

Jason G. Duncan
Chief Legal Officer, General Counsel and Secretary

Michelle Graham
Chief Human Resources Officer

Our common stock is traded on The Nasdaq Capital
Market under the symbol ALBO. On March 30, 2021, the
closing price of our common stock was $33.82 per share
and our common stock was held by 24 stockholders of
record.

Investor Information

You may obtain a copy of any of the exhibits to our Annual 
Report on Form 10­K free of charge. These documents are 
available on our website at www.albireopharma.com or by 
contacting Investor Relations at Albireo Pharma, Inc.

Requests for information about Albireo Pharma, Inc. should 
be directed to:

Investor Relations
Albireo Pharma, Inc.
10 Post Office Square, Suite 1000
Boston, Massachusetts 02109
Telephone: (857) 254­5555

Annual Meeting

The annual meeting of stockholders will be held virtually
via live webcast on Thursday, 

June 17, 2021 at 8:30 a.m. ET.

You will be able to attend our annual meeting, vote and
submit your questions during the meeting by visiting
www.virtualshareholdermeeting.com/ALBO2021.

Internet Website

www.albireopharma.com

Legal Counsel

Mintz, Levin, Cohn, Ferris, Glovsky and Popeo, P.C. 
Boston, Massachusetts

Independent Registered Public Accounting Firm

Ernst & Young LLP
Boston, Massachusetts

Transfer Agent and Registrar

Continental Stock Transfer & Trust Company
1 State Street, 30th Floor
New York, NY 10004­1561

Albireo Pharma, Inc.

10 Post Office Square, Suite 1000
Boston, Massachusetts 02109
(857) 254-5555 
www.albireopharma.com