Business Wire

OH-FORGE-BIOLOGICS

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

Lone Star Announces Sale of SPX FLOW to ITT Inc.5.12.2025 13:30:00 CET | Press release

Lone Star Funds (“Lone Star”) today announced the signing by an affiliate of Lone Star Fund XI, LP of a definitive agreement to sell SPX FLOW, Inc. (“SPX FLOW”), a leading provider of highly engineered equipment and process technologies for attractive end markets including industrial, health and nutrition, to ITT Inc. (NYSE: ITT) for $4.775 billion in cash and shares of common stock. Based in Charlotte, N.C., SPX FLOW focuses on process technologies delivering mixing, blending, fluid handling, separation, thermal heat transfer and other solutions integral to industrial, health and nutrition markets. The company has operations in more than 25 countries and sales in more than 140 countries. In partnership with Lone Star, SPX FLOW has focused on improving its sales execution and operating platform, while ensuring high quality and innovative product development. The management team has improved the company’s commercial organization and executed growth initiatives to build its presence in e

AmTrust Financial Services and Blackstone Credit & Insurance Close Strategic Transaction and Launch Newly Formed Multinational MGACompany Named ANV Group Holdings Ltd.5.12.2025 13:00:00 CET | Press release

Strategic Transaction Unlocks Value for AmTrust and Positions ANV Group Holdings for Accelerated GrowthFormer AmTrust President Adam Karkowsky Leading ANV Group Holdings as Chairman and CEO AmTrust Financial Services, Inc. (“AmTrust” or the “Company”), a global specialty property casualty insurer, and Blackstone Credit & Insurance (“BXCI”), today announced the closing of a strategic transaction under which AmTrust and funds managed by BXCI have partnered to spin-off certain of AmTrust’s Managing General Agencies (“MGAs”) and fee-based businesses in the U.S., United Kingdom, and Continental Europe, into ANV Group Holdings Ltd. (“ANV”), a newly formed independent company, following receipt of regulatory approvals. AmTrust and ANV have entered into a ten-year capacity agreement through which AmTrust will remain the underwriter for the existing books of business offered through the MGAs. As previously announced on September 15, 2025, the agreement includes seven AmTrust subsidiaries: ANV S

Galderma Opens up New Chapter for Sculptra® with MDR Certification and New Expanded Indication for Body5.12.2025 07:00:00 CET | Press release

Sculptra®, the first proven regenerative biostimulator, has received European Union (EU) Medical Device Regulation (MDR) certification, expanding its current clinical application for the face to include gluteal area, posterior thighs, décolletage, and upper arms1 Studies investigating the efficacy of Sculptra on areas beyond the face show patients experienced progressive improvements in skin quality (including the improvement in cellulite appearance), firmness, lift, projection, and contour Sculptra’s versatility and new indication allow practitioners to shift patient treatment goals beyond rejuvenation toward enhancement, meeting evolving patient needs The certification validates Sculptra’s safety performance, efficacy, and quality, reinforcing Galderma’s commitment to innovation and meeting evolving patient needs Galderma (SIX: GALD), the pure-play dermatology category leader, today announced the certification of Sculptra for body indications in the European Union (EU) following its

Northern Escape Heli-Skiing Takes the Title of “World’s Best Heli-Ski-Operator” for the 4th Year Running5.12.2025 02:40:00 CET | Press release

4x World’s Best Heli-Skiing Operation near Terrace, BC Northern Escape Heli-Skiing (NEH) has been named World’s Best Heli-Ski Operator 2025 by the World Ski Awards, marking its fourth straight victory following years 2022, 2023 and 2024. This recognition places NEH amongst an elite group of ski tourism operators consistently voted best in the world by industry leaders and enthusiasts alike. This press release features multimedia. View the full release here: https://www.businesswire.com/news/home/20251204074550/en/ A four-year run built on reliability NEH operates in British Columbia’s Skeena Mountains, where deep northern snow and vast terrain come together with small-group heli-skiing and heli snowboarding. When helicopters can’t safely fly, NEH has cat skiing readily available as a backup — an exceedingly rare offering in this industry. The operation spans over 833,571 wild acres of alpine bowls, glaciers and old-growth tree skiing. The NEH experience Guests choose from three lodge f

IDE034, a Bispecific ADC Licensed by Biocytogen to IDEAYA, Receives FDA IND Clearance5.12.2025 01:00:00 CET | Press release

Biocytogen Pharmaceuticals (Beijing) Co., Ltd. (Biocytogen, HKEX: 02315), announced that its partner IDEAYA Biosciences, Inc. (Nasdaq: IDYA), a precision oncology company, has received the clearance of an investigational new drug (IND) application with the U.S. Food and Drug Administration (FDA) for the initiation of a Phase 1 clinical trial of IDE034, a potential first-in-class B7H3/PTK7 bispecific antibody-drug conjugate (ADC). IDEAYA expects to begin patient enrollment in Q1 2026, initially evaluating patients with solid tumors known to express B7H3 and PTK7, including lung, colorectal, head and neck, and ovarian/gynecological cancers. IDE034 is a bispecific B7H3/PTK7 TOP1 ADC, independently developed by Biocytogen and licensed to IDEAYA in July 2024. The IND clearance marks an important milestone for this licensed program, supporting subsequent clinical development of IDE034, while highlighting Biocytogen’s technical capabilities in bispecific ADC discovery and development. Dr. Yue

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