Sentynl, a Zydus Group company, receives worldwide proprietary rights to Zokinvy, adding to portfolio of biopharmaceuticals for rare and ultra-rare diseases

Zydus集团公司Sentynl获得了Zokinvy的全球专有权，增加了罕见和超罕见疾病的生物制药组合

Zokinvy is approved in the U.S. (2020), European Union and Great Britain (2022), and Japan (2024) for the treatment of progeria, a collection of ultra-rare, fatal, genetic premature aging diseases that accelerate mortality in young patients

佐金维在美国获得批准。S、 （2020年），欧盟和英国（2022年）以及日本（2024年）用于治疗早衰症，早衰症是一系列超罕见，致命的遗传性早衰疾病，可加速年轻患者的死亡率

SOLANA BEACH, Calif. and AHMEDABAD, India and PALO ALTO, Calif., May 3, 2024 /PRNewswire/ -- Sentynl Therapeutics, Inc. (Sentynl), a U.S.-based biopharmaceutical company wholly-owned by Zydus Lifesciences, Ltd. (Zydus Group), and Eiger BioPharmaceuticals, Inc. (OTC: EIGRQ), a commercial-stage biopharmaceutical company focused on the development of innovative therapies for rare metabolic diseases, today announced the closing of the sale of Eiger's Zokinvy® (lonafarnib) program to Sentynl..

加利福尼亚州索拉纳海滩（SOLANA BEACH）、印度艾哈迈达巴德（AHMEDABAD）和加利福尼亚州帕洛阿尔托（PALO ALTO），2024年5月3日/PRNewswire/--Sentynl Therapeutics，Inc.（Sentynl），一家总部位于美国的生物制药公司，由Zydus Lifesciences，Ltd.（Zydus Group）全资拥有，Eiger BioPharmaceuticals，Inc.（OTC:EIGRQ），一家专注于开发罕见代谢疾病创新疗法的商业阶段生物制药公司，今天宣布结束向Sentynl出售Eiger的Zokinvy®（lonafarnib）计划。

Zokinvy is the first and only treatment approved by the U.S. Food and Drug Administration (FDA) to target the cause and symptoms of progeria, also known as Hutchinson-Gilford progeria syndrome (HGPS) and processing-deficient progeroid laminopathies (PDPL), in young people 12 months of age and older.

Zokinvy是美国食品和药物管理局（FDA）批准的第一种也是唯一一种针对12个月及以上年轻人早衰（也称为Hutchinson-Gilford早衰综合征（HGPS）和加工缺陷型早衰性椎板病（PDPL））病因和症状的治疗方法。

Collectively known as progeria, HGPS and PDPL are ultra-rare, fatal, genetic premature aging diseases that accelerate mortality in young patients. Following its U.S. approval in 2020, Zokinvy secured approval in the European Union and Great Britain (2022) and in Japan (January 2024)..

HGPS和PDPL统称为早衰，是一种极为罕见的致命遗传性早衰疾病，可加速年轻患者的死亡率。在2020年获得美国批准后，佐金维（Zokinvy）获得了欧盟和英国（2022年）以及日本（2024年1月）的批准。

Speaking on the acquisition, Dr. Sharvil Patel, Managing Director of Zydus Lifesciences, said, 'This acquisition marks an important milestone in growing our portfolio of medicines for rare and orphan diseases, which can have devastating consequences if left untreated. We are focused on supporting patients to live healthier and more fulfilled lives.

在谈到此次收购时，Zydus Lifesciences董事总经理夏维尔·帕特尔（Sharvil Patel）博士表示，“此次收购标志着我们扩大罕见病和孤儿病药物组合的一个重要里程碑，如果不及时治疗，可能会产生毁灭性的后果。我们专注于支持患者过上更健康、更充实的生活。

The acquisition of Zokinvy directly furthers this mission, as it has a demonstrated meaningful impact on young patients and their families.'.

收购Zokinvy直接推动了这一使命，因为它对年轻患者及其家人产生了重大影响。”

'It is an honor to add Zokinvy to our portfolio of products that have a tangible impact on the lives of rare disease patients, whose needs are too often unmet or overlooked,' said Matt Heck, President & Chief Executive Officer of Sentynl. 'We are firmly committed to provide best-in-class global access to Zokinvy and are eager to serve the patients and their families affected by progeria.

Sentynl总裁兼首席执行官马特·赫克（MattHeck）说，很荣幸能将佐金维（Zokinvy）加入我们的产品组合中，这些产品对罕见病患者的生活产生了切实的影响，他们的需求往往得不到满足或被忽视我们坚定地致力于为Zokinvy提供全球一流的服务，并渴望为受早衰影响的患者及其家人提供服务。

We are grateful to Eiger and The Progeria Research Foundation for their dedicated effort to develop and secure availability of this life-changing product.'.

我们感谢Eiger和Progeria研究基金会为开发和确保这种改变生命的产品的可用性所做的不懈努力。”

'We are pleased to complete this agreement with Sentynl, given our shared commitment to supporting patients of life-threatening, rare conditions with high unmet medical needs,' said David Apelian, MD, PhD, MBA, CEO of Eiger. 'We thank The Progeria Research Foundation for their continued support of Zokinvy.'.

艾格首席执行官、医学博士、工商管理硕士大卫·阿佩利安（DavidApelian）说：“鉴于我们共同致力于支持危及生命、罕见疾病且医疗需求未得到满足的患者，我们很高兴与Sentynl达成这项协议。”我们感谢Progeria研究基金会对Zokinvy的持续支持。”

Audrey Gordon, President and Executive Director of The Progeria Research Foundation (PRF), added, 'Without Zokinvy therapy, children with progeria die of the same heart disease that affects millions of normally aging adults, but by an average age of 14.5 years old. Zokinvy gives these beautiful children longer, healthier lives.

早衰研究基金会（PRF）总裁兼执行董事奥黛丽·戈登（AudreyGordon）补充道，“如果没有佐金维（Zokinvy）治疗，早衰儿童会死于影响数百万正常衰老成年人的心脏病，但平均年龄为14.5岁。佐金维让这些美丽的孩子拥有更长、更健康的生命。

Since we first launched PRF in 1999, we have achieved tremendous progress in global awareness, breakthrough research, and treatment of progeria. We are thankful for our successful partnership with Eiger, and are excited to now join forces with Sentynl in our journey to continue advancing the research and treatment of this syndrome, with the ultimate goal to find the cure.'.

自1999年我们首次推出PRF以来，我们在全球意识，突破性研究和早衰治疗方面取得了巨大进展。我们感谢与Eiger的成功合作，现在很高兴与Sentynl携手合作，继续推进这种综合征的研究和治疗，最终目标是找到治愈方法。”

As previously disclosed by Eiger, on April 1, 2024, Eiger and its direct subsidiaries filed voluntary petitions for relief under chapter 11 of Title 11 of the United States Code (Chapter 11 Cases) in the United States Bankruptcy Court for the Northern District of Texas (Bankruptcy Court). On April 17, 2024, following the completion of the auction held as part of the Eiger's court-supervised sale process, Sentynl was designated the winning bidder with a final bid during the auction of a base price in the amount of $46.1 million less a credit in the amount of $0.9 million for the termination fee resulting in a net base price in the amount of $45.2 million, subject to certain purchase price adjustments, including a reduction of $100,000 per diem if the sale closed after April 24, 2024.

正如Eiger之前披露的那样，2024年4月1日，Eiger及其直接子公司根据《美国法典》第11编第11章（第11章案例）向德克萨斯州北区美国破产法院（破产法院）提交了自愿救济申请。2024年4月17日，作为埃格法院监督的拍卖过程的一部分，拍卖完成后，Sentynl被指定为中标人，在拍卖过程中最终出价为4610万美元，减去90万美元的终止费信贷，导致净基价为4520万美元，但需进行某些购买价格调整，包括如果2024年4月24日后拍卖结束，则每日减少100000美元。

At a hearing held on April 23, 2024, the Bankruptcy Court approved the sale to Sentynl, with the sale closing on May 3, 2024. Under the terms of the acquisition, Sentynl acquired global rights to Zokinvy and will be responsible for its manufacture and commercialization..

在2024年4月23日举行的听证会上，破产法院批准出售给Sentynl，出售于2024年5月3日结束。根据收购条款，Sentynl收购了Zokinvy的全球权利，并将负责其制造和商业化。

For questions on continued access to Zokinvy, please contact the Sentynl Cares support team at 1-888-251-2800 Monday-Friday, 8 am-8 pm ET. For inquiries after hours, follow the recorded instructions.

有关继续访问Zokinvy的问题，请联系Sentynl Cares支持团队，电话：1-888-251-2800，周一至周五，美国东部时间上午8点至下午8点。如需在下班后查询，请按照记录的说明进行。

About Progeria

关于早衰

Collectively known as progeria, Hutchinson-Gilford progeria syndrome and progeroid laminopathies are ultra-rare, fatal, genetic premature aging diseases that accelerate mortality in young patients.

Hutchinson-Gilford早衰综合征和早衰性椎板病统称为早衰，是一种极为罕见的致命遗传性早衰疾病，可加速年轻患者的死亡率。

HGPS 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.4,5.

HGPS是由LMNA基因中的点突变引起的，产生法尼基化异常蛋白progerin。早衰性椎板病是由LMNA和/或ZMPSTE24基因中的一系列突变引起的加速衰老的遗传条件，产生法尼基化蛋白。不同于progerin.4,5。

Without Zokinvy therapy, children with HGPS 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.3.

如果没有Zokinvy治疗，患有HGPS的儿童通常会死于影响数百万正常衰老成年人（动脉硬化）的心脏病，平均年龄为14.5岁。疾病表现包括严重failure壮成长，硬皮病样皮肤，全身脂肪营养不良，脱发，关节挛缩，骨骼发育不良，全身加速动脉粥样硬化伴心血管功能下降，以及使人衰弱的中风。

About Zokinvy (lonafarnib)

关于Zokinvy（lonafarnib）

Zokinvy is a first-in-class disease-modifying agent that blocks the accumulation of defective progerin and progerin-like proteins which leads to cellular instability and premature aging in children and young adults with progeria. Zokinvy has demonstrated a statistically significant survival benefit in children and young adults with HGPS.1,4.

Zokinvy是一种一流的疾病调节剂，可阻止有缺陷的progerin和progerin样蛋白的积累，从而导致患有早衰的儿童和年轻人的细胞不稳定和过早衰老。Zokinvy已证明对患有HGPS的儿童和年轻人具有统计学上显着的生存益处。

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.1,2

最常报告的不良反应是胃肠道（呕吐，腹泻，恶心），大多数严重程度为轻度或中度（1级或2级）。许多早衰患者接受了连续的Zokinvy治疗超过10年。1,2

Zokinvy is FDA approved for the treatment of patients 12 months of age and older with a genetically confirmed diagnosis of Hutchinson-Gilford progeria syndrome or a processing-deficient progeroid laminopathy associated with either a heterozygous LMNA mutation with progerin-like protein accumulation or a homozygous or compound heterozygous ZMPSTE24 mutation..

Zokinvy被FDA批准用于治疗12个月及以上经遗传学确诊为Hutchinson-Gilford早衰综合征或加工缺陷型早衰性椎板病的患者，这些患者与早衰蛋白样蛋白积累的杂合LMNA突变或纯合或复合杂合ZMPSTE24突变有关。

For Important Safety Information and prescribing information for Zokinvy in the U.S., please visit www.zokinvy.com.

有关美国佐金维的重要安全信息和处方信息。S、 ，请访问www.zokinvy.com。

Eiger licensed exclusive worldwide rights to lonafarnib for the treatment of H-G progeria from MSD, the tradename of Merck & Co., Inc, Rahway, N.J., USA. MSD provided lonafarnib free of charge for clinical studies supported by the PRF and waived royalty and milestone obligations on lonafarnib from Sentynl for people living with the condition..

Eiger从MSD（美国新泽西州拉赫韦的默克公司的商标名）向洛那法尼授予了治疗H-G早衰的全球独家权利。MSD免费为PRF支持的临床研究提供了洛那法尼，并免除了Sentynl对患有这种疾病的人对洛那法尼的版税和里程碑义务。

Eiger and AnGes entered into an exclusive distribution agreement for the treatment of HGPS and PDPL indications, Zokinvy (Lonafarnib), in Japan on May 10, 2022. In March 2023, the Ministry of Health, Labour and Welfare designated Zokinvy as an orphan drug.

2022年5月10日，Eiger和AnGes在日本签订了治疗HGPS和PDPL适应症Zokinvy（Lonafarnib）的独家经销协议。2023年3月，卫生、劳动和福利部将佐金维定为孤儿药。

About Sentynl Therapeutics

关于Sentynl Therapeutics

Sentynl Therapeutics is a U.S.-based biopharmaceutical company focused on bringing innovative therapies to patients living with rare diseases. The company was acquired by the Zydus Group in 2017. Sentynl's experienced management team has previously built multiple successful pharmaceutical companies.

Sentynl Therapeutics是一家总部位于美国的生物制药公司，专注于为患有罕见疾病的患者带来创新疗法。该公司于2017年被Zydus集团收购。Sentynl经验丰富的管理团队此前已经建立了多家成功的制药公司。

With a focus on commercialization, Sentynl looks to source effective and well-differentiated products across a broad spectrum of therapeutic areas to address unmet needs. Sentynl is committed to the highest ethical standards and compliance with all applicable laws, regulations and industry guidelines.

Sentynl专注于商业化，希望在广泛的治疗领域寻找有效且差异化程度高的产品，以满足未满足的需求。Sentynl致力于最高的道德标准，并遵守所有适用的法律、法规和行业准则。

For more information, visit https://sentynl.com..

有关更多信息，请访问https://sentynl.com..

About Zydus Group

关于Zydus Group

Zydus Lifesciences Ltd. with an overarching purpose of empowering people with freedom to live healthier and more fulfilled lives, is an innovative, global lifesciences company that discovers, develops, manufactures, and markets a broad range of healthcare therapies. The group has a significant presence in cancer related therapies and offers a wide range of solutions with cytotoxic, supportive & targeted drugs.

Zydus Lifesciences Ltd.是一家创新的全球生命科学公司，致力于发现、开发、制造和销售各种医疗保健疗法，其总体目标是赋予人们自由地过上更健康、更充实的生活。该集团在癌症相关疗法中占有重要地位，并提供多种细胞毒性，支持性和靶向性药物的解决方案。

The group employs over 26,000 people worldwide, including 1,400 scientists engaged in R & D, and is driven by its mission to unlock new possibilities in lifesciences through quality healthcare solutions that impact lives. The group aspires to transform lives through path-breaking discoveries. For more information, visit https://www.zyduslife.com/zyduslife/..

该集团在全球拥有26000多名员工，其中包括1400名从事研发的科学家，其使命是通过影响生命的优质医疗保健解决方案，挖掘生命科学的新可能性。该组织渴望通过突破性的发现改变生活。有关更多信息，请访问https://www.zyduslife.com/zyduslife/.

About Eiger Biopharmaceuticals

关于Eiger Biopharmaceuticals

Eiger is a commercial-stage biopharmaceutical company focused on the development of innovative therapies for rare metabolic diseases. Eiger's lead product candidate, avexitide, is a well characterized, first-in-class GLP-1 antagonist for the treatment of post-bariatric hypoglycemia (PBH) and congenital hyperinsulinism (HI).

Eiger是一家商业阶段的生物制药公司，专注于开发罕见代谢疾病的创新疗法。Eiger的主要候选产品avexitide是一种特征明确的一流GLP-1拮抗剂，用于治疗减肥后低血糖症（PBH）和先天性高胰岛素血症（HI）。

Avexitide is the only drug in development for PBH with Breakthrough Therapy designation from the FDA. On April 1, 2024, Eiger and its direct subsidiaries filed the Chapter 11 Cases in the Bankruptcy Court..

Avexitide是唯一一种正在开发用于PBH的药物，具有FDA的突破性治疗指定。2024年4月1日，Eiger及其直接子公司向破产法院提交了第11章的案件。

Eiger's securityholders are cautioned that trading in Eiger's securities during the pendency of the Chapter 11 Cases is highly speculative and poses substantial risks. Trading prices for Eiger's securities may bear little or no relationship to the actual recovery, if any, by holders thereof in the Chapter 11 Cases.

Eiger的证券持有人被警告，在第11章案件悬而未决期间，Eiger的证券交易具有高度投机性，并构成重大风险。在第11章的案例中，埃格尔证券的交易价格可能与持有人的实际回收（如果有的话）几乎没有关系。

Accordingly, Eiger urges extreme caution with respect to existing and future investments in its securities. In particular, Eiger expects that its securityholders could experience a significant or complete loss on their investment, depending on the outcome of the Chapter 11 Cases..

因此，艾格敦促对其证券的现有和未来投资极其谨慎。特别是，根据第11章案例的结果，Eiger预计其证券持有人的投资可能会遭受重大或完全损失。

About The Progeria Research Foundation

关于早衰研究基金会

The Progeria Research Foundation (PRF) was established in 1999 by the family of Sam Berns, a child with Progeria. Within four years of its founding, the PRF Genetics Consortium discovered the Progeria gene, a collaboration led by Dr. Francis Collins, Acting Science Advisor to the President of the United States and former Director of the National Institutes of Health (NIH).

早衰研究基金会（PRF）由早衰儿童萨姆·伯恩斯（SamBerns）的家人于1999年成立。在成立后的四年内，PRF遗传学协会发现了早衰基因，这是由美国总统代理科学顾问、美国国立卫生研究院（NIH）前院长弗朗西斯·柯林斯博士领导的合作。

PRF has funded and co-coordinated all Zokinvy associated clinical trials for Progeria and Progeroid Laminopathies, conducted at Boston Children's Hospital, and supports scientists who conduct Progeria research worldwide. PRF's International Patient Registry includes over 393 children with Progeria in 72 countries.

PRF资助并共同协调了在波士顿儿童医院进行的所有与Zokinvy相关的早衰和早衰性椎板病临床试验，并支持在全球范围内进行早衰研究的科学家。PRF的国际患者登记处包括72个国家的393多名早衰儿童。

PRF is the only non-profit organization solely dedicated to finding treatments and the cure for Progeria and its aging-related conditions, including heart disease. The organization fills a void, putting these children and Progeria at the forefront of scientific efforts. For more information and to support PRF's mission, please visit www.progeriaresearch.org..

PRF是唯一一家专门致力于寻找治疗和治愈早衰及其衰老相关疾病（包括心脏病）的非营利性组织。该组织填补了一个空白，将这些儿童和早衰患者置于科学研究的前沿。欲了解更多信息并支持PRF的使命，请访问www.progeriaresearch.org。

Note Regarding Forward-Looking Statements of Eiger

关于Eiger前瞻性声明的注释

This press release contains forward-looking statements within the meaning of the safe harbor provisions of the Private Securities Litigation Reform Act of 1995. All statements other than statements of historical facts, including statements regarding the continued commercialization and manufacturing of Zokinvy, the outcome of the Chapter 11 Cases, business strategy and plans and objectives for future operations, are forward-looking statements.

本新闻稿包含1995年《私人证券诉讼改革法案》安全港条款所指的前瞻性声明。除历史事实陈述外，所有陈述均为前瞻性陈述，包括关于佐金维继续商业化和制造的陈述、第11章案例的结果、商业战略以及未来运营的计划和目标。

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' section in Eiger's Quarterly Report on Form 10-K for the year ended December 31, 2023, and Eiger's subsequent filings with the SEC.

各种重要因素可能导致实际结果或事件与Eiger所作的前瞻性陈述存在重大差异，包括Eiger截至2023年12月31日的10-K表季度报告中“风险因素”部分所述的其他适用风险和不确定性，以及Eiger随后向SEC提交的文件。

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..

本新闻稿中包含的前瞻性声明基于Eiger目前可获得的信息，仅在发布之日发表。Eiger不承担并明确否认任何更新任何前瞻性声明的义务，无论是由于任何新信息、未来事件、情况变化或其他原因。

References:

参考文献：

1. Data on file, Eiger BioPharmaceuticals.

1.Eiger BioPharmaceuticals存档数据。

2. Summary of Product Characteristics, July 2022.

2、《产品特性总结》，2022年7月。

3. Gordon LB, Brown WT, Collins FS. Hutchinson-Gilford Progeria Syndrome. 2003 Dec 12

3.Gordon LB，Brown WT，Collins FS。哈钦森-吉尔福德早衰综合征。2003年12月12日

[Updated 2019 Jan 17]. In: Adam MP, Ardinger HH, Pagon RA, et al., editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2020.

[2019年1月17日更新]。在：Adam MP，Ardinger HH，Pagon RA等，编辑。GeneReviews®[互联网]。西雅图（华盛顿州）：西雅图华盛顿大学；1993-2020年。

4. Gordon LB, Shappell H, Massaro J, et al. Association of lonafarnib treatment vs no treatment with mortality rate in patients with Hutchinson-Gilford progeria syndrome. JAMA. 2018;319(16):1687-1695. doi:10.1001/jama.2018.3264.

4.Gordon LB，Shappell H，Massaro J等人，lonafarnib治疗与不治疗与Hutchinson-Gilford早衰综合征患者死亡率的关系。杰玛。2018年；319（16）：1687-1695年。doi:10.1001/jama.2018.3264。

5. Marcelot A, Worman HJ, and Zinn-Justin S. Protein structural and mechanistic basis of progeroid laminopathies. FEBS Journal. 2021:288:2757-2772. Doi:10.111/febs.15526.

5.Marcelot A，Worman HJ和Zinn-Justin S.早衰性椎板病的蛋白质结构和机制基础。FEBS期刊。2021:288:2757-2772。Doi:10.111/febs.15526。

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SOURCE Sentynl Therapeutics

来源Sentynl Therapeutics

Company Codes: OTC-BB:EIGRQ, OTC-PINK:EIGRQ, OtherOTC:EIGRQ

公司代码：OTC-BB：EIGRQ，OTC-PINK：EIGRQ，其他OTC：EIGRQ