FDA grants orphan drug status to novel MPS IIIB treatment

Published 07/05/2025, 16:42
FDA grants orphan drug status to novel MPS IIIB treatment

TOKYO & HYOGO, Japan – MEDIPAL HOLDINGS CORPORATION (TSE:7459) and JCR Pharmaceuticals Co., Ltd. (TSE:4552) have announced the U.S. Food and Drug Administration’s orphan drug designation for their investigational drug JR-446, aimed at treating mucopolysaccharidosis type IIIB (MPS IIIB), also known as Sanfilippo syndrome type B.

MPS IIIB is a rare genetic disorder affecting an estimated 500 to 1,000 individuals globally, characterized by severe central nervous system symptoms. As of now, no treatments have been approved for this debilitating condition. JR-446, which utilizes JCR’s proprietary J-Brain Cargo® technology, is currently undergoing a Phase I/II trial in Japan.

The orphan drug designation is a critical milestone in the clinical development of JR-446, as it provides incentives such as market exclusivity for seven years upon FDA approval, tax credits for clinical trials, and waived application fees. This status is granted to drugs targeting diseases affecting fewer than 200,000 people in the U.S.

MEDIPAL’s President and CEO Shuichi Watanabe expressed the company’s commitment to advancing JR-446’s development in collaboration with JCR, highlighting the urgent need for a treatment among patients and their families. Similarly, JCR’s President, Chairman, and CEO Shin Ashida emphasized the designation’s support for the therapy’s potential to meet the significant unmet medical need of MPS IIIB patients.

In September 2023, MEDIPAL and JCR entered a licensing agreement granting MEDIPAL the rights to commercialize JR-446 outside Japan. MEDIPAL will also assist JCR in clinical development efforts within Japan.

This partnership aligns with MEDIPAL’s role in managing the operations of companies across various sectors, including pharmaceutical wholesales, while JCR Pharmaceuticals continues to specialize in treatments for rare and genetic diseases, expanding its reach globally. According to InvestingPro, JCR has maintained dividend payments for 33 consecutive years, currently offering a 4.14% yield, despite challenging market conditions.

The orphan drug designation by the FDA marks a hopeful advancement for individuals affected by MPS IIIB, as JR-446 continues through the clinical trial process. This announcement is based on a press release statement from the companies involved. While JCR’s stock has shown strong returns recently, InvestingPro analysis suggests the company is currently fairly valued, with additional financial metrics and insights available to subscribers.

This article was generated with the support of AI and reviewed by an editor. For more information see our T&C.

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