Eiger BioPharmaceuticals and AnGes Announce Exclusive Partnership for Regulatory Approval and Commercialization of Zokinvy® (lonafarnib) in Japan

PALO ALTO, Calif. and OSAKA, Japan, May 10, 2022 /PRNewswire/ -- Eiger Biopharmaceuticals Inc. ("Eiger", Nasdaq: EIGR) and AnGes Inc. ("AnGes", TYO: 4563) today announced that the companies entered into an agreement for the regulatory approval, marketing, and distribution of Zokinvy^® (lonafarnib) for the treatment of Hutchinson-Gilford progeria syndrome (HGPS or progeria) and processing-deficient progeroid laminopathies (PL) in Japan.

Under the terms of the agreement, AnGes is responsible for obtaining and maintaining regulatory approval for Zokinvy in Japan and will be the exclusive partner for distribution and commercialization. Eiger will receive upfront and milestone payments up to $1.5M as well as earn revenue from the sale of Zokinvy to AnGes.

"We are thrilled to build a new partnership with Eiger for Zokinvy in Japan. We are committed to obtaining regulatory approval in an expeditious manner to deliver Zokinvy to the HGPS and PL patients in Japan," said Ei Yamada, President and CEO, AnGes. "In parallel with the regulatory approval process, we will persistently advance our preparations to initiate the diagnostic test for HGPS as part of developing the genetic disease diagnosis testing of new-born babies at AnGes Clinical Research Laboratories (ACRL) established last year."

"We are pleased to enter into this partnership with AnGes to seek regulatory approval and commercialization of Zokinvy in Japan to help patients suffering from progeria and progeroid laminopathies," said David Cory, President and CEO, Eiger. "This collaboration is representative of Eiger's strategic approach to fully leverage our innovative therapies to enhance shareholder value and support underserved patients around the world."

About progeria and progeroid laminopathies

Progeria, also known as Hutchinson-Gilford progeria syndrome, and progeroid laminopathies are separate and distinct ultra-rare, fatal, genetic premature aging diseases that accelerate mortality in young patients. It is estimated that there are 400 children worldwide with progeria and 200 children with progeroid laminopathies.

Progeria is caused by a point mutation in the LMNA gene, yielding the farnesylated aberrant protein, progerin. Progeroid laminopathies are genetic conditions of accelerated aging caused by a constellation of mutations in the LMNA and/or ZMPSTE24 genes yielding farnesylated proteins that are distinct from progerin. While non–progerin producing, these genetic mutations result in disease manifestations with phenotypes that have overlap with, but are distinct from, progeria.

Without Zokinvy therapy, children with progeria commonly die of the same heart disease that affects millions of normally aging adults (arteriosclerosis), by an average age of 14.5 years. Disease manifestations include severe failure to thrive, scleroderma–like skin, global lipodystrophy, alopecia, joint contractures, skeletal dysplasia, global accelerated atherosclerosis with cardiovascular decline, and debilitating strokes. 

About Zokinvy^® (lonafarnib) 

Zokinvy was approved in the U.S. in November 2020 to reduce the risk of death with HGPS (progeria), and to treat processing-deficient progeroid laminopathies. It is indicated for adults and children over 12 months of age.

Zokinvy blocks the accumulation of defective, farnesylated proteins which form tight associations with the nuclear envelope, leading to cellular instability and premature aging in children and young adults with progeria and processing-deficient progeroid laminopathies.

Zokinvy is a first-in-class disease-modifying agent that has demonstrated a statistically significant survival benefit in children and young adults with progeria. In patients with progeria, Zokinvy reduced the incidence of mortality by 60% (p=0.0064) and increased average survival time by at least 2.5 years. The most commonly reported adverse reactions were gastrointestinal (vomiting, diarrhea, nausea), and most were mild or moderate (Grade 1 or 2) in severity. Many progeria patients have received continuous Zokinvy therapy for more than 10 years.

Eiger licensed exclusive worldwide rights to lonafarnib from Merck, known as MSD outside of the United States and Canada. Merck will not receive any milestone payments for the development of lonafarnib for the treatment of progeria and has waived royalty obligations from Eiger for a specified quantity of lonafarnib.

For more information including prescribing information for Zokinvy in the U.S. please go to www.zokinvy.com. Please click here for the full U.S. important safety information. Eiger has filed a marketing authorization application with the European Medicines Agency and expects a CHMP opinion in Q2 2022. Zokinvy is not approved for any indication in Japan.

About Eiger
Eiger is a commercial-stage biopharmaceutical company focused on the development of innovative therapies to treat and cure hepatitis delta virus (HDV) and other serious diseases. The Eiger HDV platform includes two first-in-class therapies in Phase 3 that target critical host processes involved in viral replication. Eiger is also developing peginterferon lambda as a therapeutic for COVID-19 and is planning to submit an Emergency Use Authorization application to FDA based on positive results from the investigator sponsored Phase 3 TOGETHER study.

All five Eiger rare disease programs have been granted FDA Breakthrough Therapy designation: lonafarnib and peginterferon lambda for HDV, Zokinvy for progeria, and avexitide for both congenital hyperinsulinism and post-bariatric hypoglycemia.

For additional information about Eiger and its clinical programs, please visit www.eigerbio.com.

About AnGes Inc.
AnGes, Inc., a biopharmaceutical company focused on the development of gene-based medicines. In September 2019, AnGes commenced the commercialization in Japan of Collategene^® (Hepatocyte Growth Factor, HGF, plasmid gene therapy) for the treatment of Chronic arterial occlusive disease with lower limb ulcers. Collategene^® is the world's first marketed drug using Plasmid DNA. AnGes is currently focusing on the development of DNA vaccines for COVID-19 and Hypertension, Tie2 tyrosine kinase receptor agonist for COVID-19 treatment, and NF-κB decoy oligonucleotide and Chimera decoy oligonucleotide as next generation product for Chronic Discogenic Lumbar Back Pain. Furthermore, AnGes acquired EmendoBio to expand its capabilities in Genome Editing Technologies in December 2020. For more information, visit https://www.anges.co.jp/en

Eiger Note Regarding Forward-Looking Statements
This press release contains forward-looking statements within the meaning of the safe harbor provisions of the U.S. Private Securities Litigation Reform Act of 1995. These forward-looking statements include words such as "believe," "will," "may," "estimate," "continue," "anticipate," "contemplate," "intend," "target," "project," "should," "plan," "expect," "predict," "could," "potentially," other words of similar meaning and the negative of these terms. All statements other than statements of historical facts, including statements regarding our future financial condition, timing for and outcomes of clinical results, prospective products, preclinical and clinical pipelines, regulatory objectives, business strategy and plans and objectives for future operations, are forward looking statements. Forward-looking statements are our current statements regarding our intentions, beliefs, projections, outlook, analyses or current expectations concerning, among other things, our anticipated significant milestones in 2022; the timing of our ongoing and planned clinical development across our pipeline; the approval of Zokinvy in jurisdictions outside of the U.S.; our ability to obtain an Emergency Use Authorization from FDA for Peginterferon Lambda for COVID-19; our capability to provide sufficient quantities of any of our product candidates, our ability to finance the continued advancement of our development pipeline products; and the potential for success of any of our product candidates. Various important factors could cause actual results or events to differ materially from the forward-looking statements that Eiger makes, including additional applicable risks and uncertainties described in the "Risk Factors" sections in the Annual Report on Form 10-K for the year ended December 31, 2021 and Eiger's subsequent filings with the SEC. The forward-looking statements contained in this press release are based on information currently available to Eiger and speak only as of the date on which they are made. Eiger does not undertake and specifically disclaims any obligation to update any forward-looking statements, whether as a result of any new information, future events, changed circumstances or otherwise.

Media Contacts:

EIGER Investors:
Sylvia Wheeler
Wheelhouse Life Science Advisors 
swheeler@wheelhouselsa.com 

EIGER Media:
Sarah Mathieson
SVP, Corporate Affairs
smathieson@eigerbio.com

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SOURCE Eiger BioPharmaceuticals, Inc.