Business Wire

OH-FORGE-BIOLOGICS

19.3.2024 13:01:30 CET | Business Wire | Press release

Share
Forge Biologics’ Novel AAV Gene Therapy FBX-101 for Patients with Krabbe Disease is Granted UK’s Innovation Passport Designation

Forge Biologics, a member of Ajinomoto Bio-Pharma Services and a leading manufacturer of genetic medicines, today announced that the Medicines and Healthcare Products Regulatory Agency (MHRA), the healthcare regulatory body of the United Kingdom (UK), granted Innovation Passport designation to the Company’s novel AAV gene therapy program, FBX-101, to enter the Innovative Licensing and Access Pathway (ILAP). FBX-101 was created for the treatment of patients with Krabbe disease, a rare neurodegenerative disease that is usually fatal in untreated patients by age two. The ILAP is intended to accelerate regulatory and market access interactions in the UK.

Maria Escolar, M.D., Forge’s Chief Medical Officer, will discuss the significance of achieving this regulatory designation and present a poster on updated data from REKLAIM, an early phase clinical trial for FBX-101, during the Advanced Therapies 2024 conference being held in London, March 19-20, 2024.

“Considering the rapid progression of Krabbe disease in young patients and the positive results we’ve observed in the REKLAIM trial, we are pleased to receive the Innovation Passport designation which will enable Forge to further accelerate the clinical development of FBX-101,” stated Dr. Escolar. “ILAP was created to provide access to tools to decrease the time to market and facilitate patient access to medicines in the UK for life-threatening or seriously debilitating conditions.”

The REKLAIM clinical trial is evaluating the safety and efficacy of FBX-101 in patients with infantile and late infantile Krabbe disease. Children assessed in REKLAIM have received intravenous FBX-101 in addition to the current standard of care, hematopoietic stem cell transplantation (HSCT). Clinical data demonstrate that FBX 101 has been well tolerated and has an excellent safety profile after the treatment of five patients. Results show improvements in motor function in all patients, the most meaningful clinical outcome for this disease.

FBX-101 has been granted Orphan Drug Designation and Priority Medicines (PRIME) designation by the European Medicines Agency (EMA), allowing Forge to advance and expedite the development of FBX-101 as the leading worldwide gene therapy for patients with Krabbe disease. In the US, the FDA has granted FBX-101 Fast Track Designation, Orphan Drug Designation, and Rare Pediatric Disease Designation.

ILAP is intended to accelerate regulatory and market access interactions in the UK for patient populations with significant need, leading to accelerated patient access to novel therapies. It also provides access to a range of UK development services and tools, including frequent MHRA interactions, accelerated Marketing Authorization Application (MAA) assessment, a Target Development Profile, expanded licensing routes, and more, creating a roadmap for advancement of therapies through regulatory approval.

Regulatory review by the MHRA through ILAP provides opportunities for further global acceptance of Forge’s manufacturing process and facility though additional reviews and inspections, including Compliance Readiness Inspections that provide custom and pragmatic supervisory and licensing inspections specific to the GMP guidelines. Through this designation Forge has expanded global regulatory experience to include interactions with the MHRA in the UK. This access to global health authority resources will enable an added level of assurance to clients utilizing Forge’s CDMO services with integrated regulatory support.

About Krabbe Disease

Krabbe disease is a rare neurodegenerative disease affecting about 1-2.5 in 100,000 people in the US. Krabbe disease is caused by autosomal recessive mutations in the galactocerebrosidase (GALC) gene, an enzyme responsible for the breakdown of certain types of sphingolipids, such as psychosine. Without functional GALC, psychosine accumulates to toxic levels in cells, specifically in cells insulating the nerves in the brain and peripheral nervous system, causing rapid demyelination. Krabbe disease initially manifests in young patients as irritability, developmental delay, and progressive muscle weakness. Symptoms rapidly advance to difficulty swallowing, breathing, and regression of neurodevelopment followed by seizures, vision and hearing loss. Infantile Krabbe disease (0-12 months of age at onset) usually leads to death in untreated patients by two years of age. Late Infantile patients (12-36 months of age at onset) usually die by the age of six. The current standard of care, hematopoietic stem cell transplantation (HSCT), has been shown to stabilize cognitive decline and significantly improve long-term neurological outcomes. However, HSCT does not correct the peripheral neuropathy that is progressive as the patient grows, leading to loss of gross motor skills and eventually death.

About FBX-101

FBX-101 was developed to treat children with Krabbe disease. FBX-101 is an adeno-associated viral serotype rh10 (AAVrh10) gene therapy that is delivered intravenously after HSCT infusion. The vector delivers a functional copy of the GALC gene to cells in both the central and peripheral nervous system and has shown to functionally correct the central and peripheral neuropathy, improve myelination and gross motor function, and significantly prolong lifespan in animal models. This approach also has the potential to overcome some of the immunological safety challenges observed in traditional AAV gene therapies and extend the duration of gene transfer.

About the REKLAIM Trial

REKLAIM is a nonblinded, non-randomized, early phase dose escalation clinical trial currently enrolling children with asymptomatic infantile and symptomatic late infantile Krabbe disease to investigate the safety and efficacy of a single intravenous infusion of FBX-101 administered more than 21 days after HSCT, the current standard of care. Data from extensive natural history subjects will be used to compare as the control group. More information on the REKLAIM trial can be found online at https://www.clinicaltrials.gov/ct2/show/NCT05739643.

About Forge Biologics

Forge Biologics, a member of Ajinomoto Bio-Pharma Services, is a hybrid gene therapy contract manufacturing and clinical-stage therapeutics development company, enabling access to life-changing gene therapies by bringing them from concept to reality. Forge’s 200,000 square foot facility, the Hearth, is headquartered in Columbus, Ohio, and houses 20 custom-designed cGMP suites with 200,000L of manufacturing capacity. Forge’s end-to-end, scalable plasmid and AAV manufacturing services include research-grade manufacturing, process and analytical development, cGMP manufacturing, fill and finish, and integrated regulatory support to help accelerate the timelines of transformative medicines for patients with genetic diseases. To learn more, visit www.forgebiologics.com.

To view this piece of content from cts.businesswire.com, please give your consent at the top of this page.

View source version on businesswire.com: https://www.businesswire.com/news/home/20240319976189/en/

About Business Wire

Business Wire
Business Wire
101 California Street, 20th Floor
CA 94111 San Francisco

http://businesswire.com
DK

Subscribe to releases from Business Wire

Subscribe to all the latest releases from Business Wire by registering your e-mail address below. You can unsubscribe at any time.

Latest releases from Business Wire

Clearlake Capital Closes on $14.8 Billion to Capitalize on AI-Driven Transformation and Continue Sector-Focused Investment Strategy16.6.2026 15:00:00 CEST | Press release

Fund VIII Reflects Strong Global Investor Support for Clearlake’s Integrated Platform, Operational Value-Creation Model, and AI-Enabled Investment Approach Clearlake Capital Group, L.P. (“Clearlake” or the “Firm”), a global investment firm managing integrated platforms spanning private equity, liquid and private credit, and other related strategies, today announced the successful close of its eighth flagship fund, Clearlake Capital Partners VIII (“Fund VIII”), alongside related co-investment vehicles and separately managed accounts. Together, these vehicles represent $14.8 billion of capital commitments and position Clearlake to continue investing behind the secular trends reshaping industries, including artificial intelligence, software modernization, digital transformation, and operational efficiency. The successful fundraise comes amid a highly competitive and selective fundraising environment for private markets and underscores continued investor confidence in Clearlake's different

Gurobi's Research Leadership Recognized Across Leading Scientific Organizations16.6.2026 15:00:00 CEST | Press release

Awards recognize foundational research that helped shape the fields of mixed-integer and nonlinear optimization—and reflect the company’s deep scientific roots. Gurobi Optimization, LLC, the leader in decision intelligence technology, today announced recognitions received by Dr. Edward Rothberg and Dr. Andreas Wächter from leading academic and industry organizations for research that has had lasting impact on the field of optimization. “Gurobi was founded on a commitment to rigorous, applied optimization research,” said Dr. Oliver Bastert, CTO, Gurobi. “And these recognitions are a reflection of that legacy. The foundational work that Ed and Andreas produced continues to shape the field, and that same spirit of scientific innovation drives everything we do today.” Dr. Edward Rothberg, co-founder and Chairman of the Board at Gurobi, was recently honored with the INFORMS Test of Time Paper Award for his paper, “An Evolutionary Algorithm for Polishing Mixed Integer Programming Solutions.”

Airship Helps DIE ZEIT Turn Silent Advocates into 4.9-Star Rating16.6.2026 15:00:00 CEST | Press release

Leveraging Airship’s Customer Experience Platform, Germany’s leading digital news publisher transforms reader engagement and captures 822 active responses in a single day. Airship, the mobile-first customer experience company, today announced that DIE ZEIT, one of Germany’s largest and most prestigious publishers, has successfully transformed its mobile app presence, lifting the Google Play app store rating from 2.7 stars in early 2026 to a near-perfect 4.9 stars. At a time when many traditional publishers are seeing readership numbers plummet, DIE ZEIT is growing — reaching an all-time high circulation in 2025 and building a digital ecosystem around the brand. The majority of the publication’s readers are now digital, but the publication’s app store rating for DIE ZEIT didn’t match this momentum. DIE ZEIT was up against a universal challenge faced by digital product teams and customer experience executives globally: the "silent advocate" problem. While highly satisfied customers rarel

New CSC Report Illustrates How AI Is Affecting Enterprise Security Leaders16.6.2026 15:00:00 CEST | Press release

CISO Outlook 2026 report indicates 73% of security leaders view AI as an opportunity rather than a risk, however traditional and AI-driven cyber threats persist CSC, an enterprise-class domain registrar and world leader in mitigating brand, fraud, domain, and domain name system (DNS) threats, today released new research on how chief information security officers (CISOs) are adapting to an evolving artificial intelligence (AI) ecosystem while managing traditional cyber threats, such as DNS outages. According to CSC’s CISO Outlook 2026 report, 73% of respondents say AI presents more of an opportunity than a risk for cybersecurity. However, these security leaders continue to face challenges in addressing attacks, including AI-powered domain generation algorithms (DGAs), which 86% of respondents cite as a threat. To thoroughly understand the CISO perspective on today’s cybersecurity landscape, CSC surveyed 300 senior executives—including CISOs, chief technology officers (CTOs), chief infor

TestMu AI Introduces DevTools Assertions in Kane CLI, Enabling Browser-Level Validation Through Natural Language16.6.2026 14:30:00 CEST | Press release

New capability allows developers and AI agents to validate network activity, console logs, browser storage, and performance metrics without writing custom scripts TestMu AI (formerly LambdaTest), the world's first Agentic AI-powered Quality Engineering platform, today announced DevTools Assertions for Kane CLI, enabling developers and AI agents to validate browser internals using natural language. Traditional browser automation focuses on what users can see: buttons, forms, text, and visual states. However, modern web applications depend heavily on browser activity that remains invisible to users, including network requests, API responses, console logs, cookies, local storage, and performance metrics. With DevTools Assertions, Kane CLI extends browser validation beyond the UI, allowing teams to verify these underlying signals using plain-English objectives. Developers can now write objectives such as: "Click Add to Cart and assert the request to /api/cart returned status 200." "Run the

In our pressroom you can read all our latest releases, find our press contacts, images, documents and other relevant information about us.

Visit our pressroom
World GlobeA line styled icon from Orion Icon Library.HiddenA line styled icon from Orion Icon Library.Eye