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

emnify Appoints Sacha Herrmann as Chief Financial Officer and Christian Friis as Chief Revenue Officer2.10.2026 17:12:00 CEST | Press release

Appointments expand emnify executive team to accelerate global growth emnify, a global leader in IoT connectivity, today announced the appointments of Sacha Herrmann as Chief Financial Officer and Christian Friis as Chief Revenue Officer, effective immediately. This press release features multimedia. View the full release here: https://www.businesswire.com/news/home/20261001902751/en/ emnify Appoints Sacha Herrmann as Chief Financial Officer and Christian Friis as Chief Revenue Officer The appointments follow the recent naming of Kenneth De Spiegeleire as Chief Executive Officer and Frank Stoecker as Executive Chairman of the Board. Together, these leadership changes strengthen emnify financial, operational, and commercial capabilities as the company enters its next phase of international growth. “We have proven momentum. Our ambition is to now turn it into global category leadership,” said Kenneth De Spiegeleire, CEO of emnify. “That requires sharper focus, disciplined execution, and

Tacton Appoints Former IFS Executive Merlin Knott as Chief Commercial Officer to Accelerate Global Growth and Scale Beyond CPQ2.10.2026 15:00:00 CEST | Press release

Enterprise software veteran to lead Tacton’s united commercial organization and partner expansion worldwide. Tacton, a global leader in Configure, Price, Quote (CPQ) software and configuration solutions for manufacturers of complex products, today announced the appointment of Merlin Knott as Chief Commercial Officer (CCO). In this role, Knott will lead Tacton’s global commercial organization, including marketing, sales, and pre-sales, aligning the teams responsible for bringing Tacton’s solutions to market, and driving value to its customers and partners globally. This press release features multimedia. View the full release here: https://www.businesswire.com/news/home/20261002794882/en/ Merlin Knott, Chief Commercial Officer, Tacton Knott joins Tacton with decades of experience building and scaling commercial organizations across the enterprise software industry. Most recently, he held senior leadership roles at IFS, the world’s leading provider of Industrial AI software, where he led

EDW Presents 2026 Data Quality Award to Repeat Winner Domivest2.10.2026 15:00:00 CEST | Press release

European DataWarehouse (EDW) presented its fifth annual Data Quality Award at the 2026 TSI Congress in Berlin. This year, EDW's Best Data Quality – Deal of the Year (2026) award was presented to Domivest B.V. for its transaction Domi 2023-1 B.V., recognising the firm's outstanding commitment to accurate, complete, and consistent reporting. The award is based on EDW's proprietary data quality scoring methodology, which evaluates transactions against thousands of validation rules and checks. Data quality excellence is not a one-off achievement. The fact that the same issuers continue to rank among the top performers year after year demonstrates that superior data quality is the result of sustained commitment, robust processes, and ongoing investment. "Receiving the Data Quality Award for a second time means a great deal to us. Transparent, accurate loan-level data is key to investor trust in our transactions, and we are grateful to European DataWarehouse for recognising the work our trea

ILiAD Biotechnologies Expands BPZE1 Public Health and Commercial Potential with Publication of Pediatric Phase 2b Study in The Lancet Infectious Diseases2.10.2026 14:00:00 CEST | Press release

Intranasal pertussis vaccine candidate BPZE1 demonstrates robust nasal mucosal and systemic immune responses with a favorable safety profile in children ages 6 through 17 yearsFirst pediatric clinical trial builds on 5 prior adult BPZE1 clinical studiesDemonstrates safety and immunogenicity of BPZE1 in acellular pertussis vaccine primed individualsNo interference observed when BPZE1 co-administered with diphtheria and tetanus antigens ILiAD Biotechnologies, Inc. (ILiAD), an advanced clinical stage biotech company focused on the prevention and treatment of disease caused by Bordetella pertussis, today announced the publication of results from its Phase 2b SUPER (Stand Up to Pertussis) clinical trial in The Lancet Infectious Diseases. This randomized, double blind, placebo- and active-comparator-controlled trial demonstrated that BPZE1, a live attenuated intranasal pertussis vaccine, induced robust nasal mucosal immune responses in healthy children aged 6 through 17 years (LINK). The stu

NIQ Brings New AI and Automation Capabilities to Retail Space Planning and Merchandising2.10.2026 12:45:00 CEST | Press release

New AI and automation capabilities help retailers close execution gaps and scale merchandising decisions across every store. NielsenIQ (NYSE: NIQ), a leading consumer intelligence company, today announced new AI and automation capabilities for NIQ Spaceman®, its retail space planning and merchandising platform trusted by more than 600 retailers across 65+ countries. The enhancements help retailers create store-specific planograms up to 50 times faster, identify execution and compliance gaps, and scale merchandising decisions across their store networks through expanded cloud-based access. "Retailers are under increasing pressure to localize assortments, execute consistently across stores, and respond more quickly to changing shopper behavior," said Natalie Williams, SVP, Product at NIQ. "These enhancements help simplify that process by embedding agentic AI directly into merchandising workflows and automating traditionally manual planning tasks. That means teams can spend less time buil

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