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

REPLY: The Board of Directors Approved the Draft Financial Statements for the Year 202512.3.2026 15:38:00 CET | Press release

All economic indicators are positive.Consolidated turnover of €2,483.6 million (€2,300.5 million in 2024);EBITDA at €467.6 million (€410.6 million in 2024);EBIT at €391.7 million (€330.4 million in 2024)Group net profit at €250.9 million (€211.1 million in 2024)Approval of the proposed dividend distribution of €1.35 per share. Today the Board of Directors of Reply S.p.A. [MTA, STAR: REY] approved the draft financial statement for the year 2025, which will be submitted for approval to the Shareholders’ Meeting to be held in first call in Turin on 23 April 2026. The Reply Group closed 2025 with a consolidated turnover of €2,483.6 million, an increase of 8.0% compared to €2,300.5 million in 2024. All indicators are positive for the period. Consolidated EBITDA was €467.6 million, an increase of 13.9% compared to €410.6 million at December 2024. EBIT, from January to December, was at €391.7 million, which is an increase of 18.5% compared to €330.4 million at December 2024. The Group net pro

LZE GmbH Introduces Fraunhofer’s RFicient® Technology to the Market12.3.2026 14:51:00 CET | Press release

LZE GmbH is expanding its technology transfer portfolio and making the RFicient® ultra-low-power wake-up receiver technology from the Fraunhofer Institute for Integrated Circuits IIS available for the first time as a standard chip for close-to-production industrial applications. The solution enables energy-efficient IoT designs that remain continuously reachable while consuming only microamps – a key step for long-lasting, low-maintenance IoT products. LZE GmbH drives technology transfer to market: standard chip availability for close-to-production applications As a bridge between research and industry, LZE GmbH is making it easier for companies to access innovative technologies and helping them to quickly and reliably transform new developments into market-ready solutions. With RFicient®-IC (FH101RF), LZE is providing another high-tech product that comes directly from Fraunhofer research and can now be ordered in volume and integrated into close-to-production product development for t

Owkin Creates New Spin out Waiv, Formerly Owkin Dx, With $33M Financing12.3.2026 14:30:00 CET | Press release

Investment lead by OTB Ventures and Alpha Intelligence CapitalWaiv develops AI-powered precision testing to better identify and stratify patients in the clinic and in clinical trials, transforming patient careWaiv extends Owkin’s strategy of real-world validation for its AI Owkin, the AI company on a mission to solve the complexity of biology, today announced the spin out of Waiv, formerly known as Owkin Dx. The move follows significant investor interest and positions Waiv to bring AI-powered precision testing for better identification of patients in the clinic and in clinical trials, to transform patient care. This follows on from the successful launch of Bioptimus, an Owkin incubated company, in February 2024. Waiv translates AI innovation into real-world clinical impact, developing tests that predict biomarkers and patient outcomes, including RlapsRisk BC for prognostic risk profiling. With multiple tests already in use in clinical settings, its deployment platform Destra, and colla

RQM+ Launches SMART Solutions Life Cycle Partnership Model12.3.2026 14:30:00 CET | Press release

Helps MedTech Companies Navigate MDR, IVDR, and AI-Enabled Device Development RQM+, a leading MedTech CRO offering regulatory consulting, clinical trial, laboratory, and reimbursement services, today announced the launch of SMART Solutions, a life cycle partnership model designed to help medical device and diagnostics companies manage growing regulatory and development complexity. SMART Solutions introduces a strategy-led operating framework that unifies regulatory, quality, clinical, reimbursement, and laboratory expertise to support MedTech companies across the entire product life cycle to help reduce risk from early development through post-market. “MedTech companies are navigating unprecedented complexity as regulatory expectations evolve, product innovation accelerates, and post-market expectations are expanding,” said John Potthoff, Ph.D., chief executive officer of RQM+. “SMART Solutions moves beyond traditional consulting by providing an integrated life cycle partnership that h

Cryptio Raises $45m Series B as Digital Assets Move Into Regulated Financial Markets12.3.2026 14:06:00 CET | Press release

The system of record for tokenized finance – ERP infrastructure for institutions operating in digital assets Cryptio, a leader in financial data transformation and enterprise resource planning (ERP) applications for regulated digital assets, announced today a $45 million Series B funding round co-led by BlackFin Capital Partners and Sentinel Global, with participation from 1kx, Alven, BlueYard Capital and Ledger Cathay Capital. Banks, exchanges, asset managers, including Société Générale’s SG Forge, Circle, Gemini, and Securitize rely on Cryptio to ensure financial integrity across their digital assets businesses. Existing ERP systems fall short for digital assets Traditional ERP and accounting systems were not designed for blockchain-native assets, real-time reporting, or modern custody frameworks. As regulated financial institutions expand into stablecoins, tokenized securities and other on-chain instruments, these limitations create material operational and reporting challenges. Cry

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