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

Donaldson Introduces Alpha-Pore™ Technology Platform for Extended Sterile Filtration Performance2.11.2026 15:35:00 CET | Press release

Donaldson Company, Inc. (NYSE: DCI), a leading worldwide manufacturer of innovative filtration products and solutions, today announced Alpha‑Pore™ technology, a next generation sterile filtration platform engineered to extend filter service life while maintaining critical process performance. Built for critical applications, Alpha‑Pore technology enables longer filter run time compared with competitive single- and dual-layer polyethersulfone (PES) membrane filters, while maintaining flow characteristics, sterility assurance, and process integrity.* The platform is designed to help manufacturers improve operational efficiency through fewer changeouts and more consistent performance over time. Engineered for Extended Performance Sterile filtration processes often require balancing run time with key performance parameters such as flow, differential pressure, and contaminant retention. Alpha‑Pore technology is designed to achieve all three. By optimizing pore structure and filter architect

SBC Medical Forms Capital and Business Alliance with AtomicSoftware, Provider of the medicalforce Clinic Management Platform Used by 800+ Clinics in Japan2.11.2026 15:00:00 CET | Press release

Companies to co-develop AI features, starting with physician documentation support, to enhance medicalforce and SBC Medical’s in-house systems and drive per-clinic productivity and profitability SBC Medical Group Holdings Incorporated (Nasdaq: SBC) (“The Company” or “SBC Medical”), a Medical Services Organization (MSO) providing management support across a wide range of healthcare fields, today announced that it entered into a capital and business alliance agreement on October 26, 2026, with AtomicSoftware Inc. (“AtomicSoftware”), the Tokyo-based provider of medicalforce, an all-in-one software-as-a-service (SaaS) platform for clinics. In connection with the alliance, SBC Medical has invested ¥600 million (approximately US$3.8 million) in AtomicSoftware’s Series C funding round. The two companies will co-develop AI features, beginning with a feature that supports physicians in preparing medical records. Key HighlightsProven platform: medicalforce is used by more than 800 clinics*1 in J

Smartly Launches Intelligence Pro to Connect Campaign Planning, Performance and Incrementality2.11.2026 15:00:00 CET | Press release

The new offering brings together planning, performance benchmarks, headroom analysis and incrementality signals to help marketers make more informed decisions across the campaign lifecycle Smartly, the AI-powered advertising technology company, today launched Intelligence Pro, a premium offering that brings together four capabilities to help marketers plan investment, understand true incrementality and determine where to spend their next dollar. Intelligence Pro connects causal inference models with planning tools to help marketers prove business impact and make faster decisions from increasingly complex data. “Marketing teams are inundated with data, but the challenge is identifying what signals are useful and turning them into a decision they can act on,” said Maxwell Tang, Chief Product Officer at Smartly. “Intelligence Pro brings forecasting, market context and state-of-the-art incrementality signals together to help marketers assess where to invest and what their media is contribu

NetApp Completes Acquisition of PEAK:AIO to Accelerate Zettabyte-scale AI Deployments2.11.2026 14:00:00 CET | Press release

Acquisition strengthens NetApp Novus, high-performance storage, with differentiated metadata technology and a parallel file architecture purpose-built for AI NetApp® (NASDAQ: NTAP), the Intelligent Data Infrastructure company, today announced that it completed the acquisition of PEAK:AIO, a pioneer in next-generation metadata architecture and high-performance parallel file systems. With this acquisition, NetApp can leverage metadata services and parallel namespace innovations to power the fastest storage on the planet, NetApp Novus, to support zettabyte-scale file systems for the most demanding workloads of AI clouds and GPU-as-a-service providers. For the best AI infrastructure economics, organizations need to keep GPU resources continuously supplied with data without bottlenecks. The acquisition advances NetApp’s strategy for the next phase of AI infrastructure by bringing PEAK:AIO’s metadata and parallel file system expertise together with NetApp ONTAP, giving customers a path to AI

Bentley Systems Announces Seequent’s Acquisition of Seismic Interpretation Software Company Eliis2.11.2026 14:00:00 CET | Press release

Strengthens Seequent’s subsurface software portfolio, energy and geophysics capabilities, and expands team of specialists Bentley Systems, Incorporated (Nasdaq: BSY), the infrastructure engineering software company, today announced that Seequent, The Bentley Subsurface Company, has acquired seismic interpretation software company Eliis. The acquisition will enrich Seequent’s capabilities in geophysics with seismic data interpretation and further advance subsurface understanding to help solve complex global challenges. Eliis’ global team of more than 100 geoscientists, technical specialists, and researchers will join Seequent. This press release features multimedia. View the full release here: https://www.businesswire.com/news/home/20261029964674/en/ 3D view from PaleoScan showcasing recent advancements in seismic interpretation workflows that support prospect evaluation and exploration decision-making Founded in 2007 and with headquarters in Montpellier, France, Eliis develops advanced

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