Nippon Shinyaku Co., Ltd.

AB2 Bio has received a $30 million payment from Nippon Shinyaku after the Japanese company exercised its exclusive U.S. commercialization option for Tadekinig alfa, an investigational therapy for Primary Monogenic IL-18-Driven Hyperinflammatory Syndrome in patients with NLRC4 and XIAP mutations. The option exercise triggers a payment under the companies' 2025 agreement, with AB2 Bio also eligible for up to $100 million in development milestones and up to $500 million in commercial milestone and royalty payments, bringing the total potential milestone and royalty value to $600 million. AB2 Bio retains worldwide rights to Tadekinig alfa for all other indications and all ex-U.S. rights for the lead indication, and will continue to lead Biologics License Application preparation and U.S. regulatory activities. The therapy has received Orphan Drug Designation in the U.S. and Europe, as well as Breakthrough Therapy and Rare Pediatric Disease Designations from the FDA.

Nippon Shinyaku has entered into an option agreement with Elixirgen Therapeutics under which Nippon Shinyaku may obtain exclusive worldwide rights to develop and commercialize EXG-7001 for the treatment of Duchenne muscular dystrophy. EXG-7001 is an investigational mRNA-based therapeutic that expresses full-length human dystrophin protein, intended to suppress local muscle dysfunction regardless of genetic mutation. Elixirgen is currently preparing for clinical trials in the United States, and following option exercise and regulatory approval, NS Pharma, a wholly owned subsidiary of Nippon Shinyaku, intends to commercialize the therapy. The agreement was facilitated by the NS Pharma Innovation Research Partnering team.

Nippon Shinyaku, the parent company of NS Pharma, has entered into a Sponsored Research Agreement with Boston Children's Hospital to develop innovative neurologic therapies. The agreement was facilitated by the NS Pharma Innovation Research Partnering team and builds on a strategic collaboration announced in July 2025. Under the deal, Nippon Shinyaku will integrate Boston Children's Hospital's research capabilities with its own drug discovery expertise to accelerate new treatments for patients with unmet medical needs.