Shionogi & Co. has agreed to acquire biopharmaceutical company IntraBio for $2 billion, strengthening its presence in rare diseases and adding an approved treatment for serious neurological conditions to its portfolio.
Under the agreement announced on October 5, 2026, Shionogi will acquire all outstanding shares of IntraBio through its U.S. subsidiary, Shionogi Inc. The transaction is expected to close between November and December 2026, subject to customary closing conditions.
The acquisition will give Shionogi global rights to AQNEURSA (levacetylleucine), a medicine developed by IntraBio for rare neurological diseases. AQNEURSA was approved by the U.S. Food and Drug Administration (FDA) in 2024 for neurological manifestations of Niemann-Pick disease type C (NPC) in adults and children. It received European approval in 2026.
More recently, the FDA approved AQNEURSA for the treatment of ataxia in adults and children with Ataxia-Telangiectasia (A-T), making it the first and only approved treatment for ataxia associated with the rare genetic disorder, according to Shionogi. The medicine is currently under review by the European Medicines Agency for A-T.
Niemann-Pick disease type C is a rare genetic disorder that affects the movement of cholesterol and other lipids inside cells. It can cause progressive neurological and systemic symptoms and may affect patients from childhood through adulthood.
Ataxia-Telangiectasia is another rare inherited disorder that typically begins in early childhood. It can cause problems with movement, coordination and speech, while also affecting the immune system and increasing the risk of certain cancers.
Shionogi said the acquisition will also bring IntraBio’s expertise in rare neurodegenerative and genetic neurological diseases. The company has clinical programs targeting conditions including Pompe disease, Fragile X syndrome and Jordan’s syndrome, along with additional early-stage programs.
The deal is part of Shionogi’s broader strategy to build its rare disease business. In April 2026, the Japanese pharmaceutical company acquired global rights to edaravone, marketed as RADICAVA in the U.S., a treatment for amyotrophic lateral sclerosis (ALS).
By adding AQNEURSA and IntraBio’s development capabilities, Shionogi expects to expand its rare disease portfolio and accelerate the development of new treatments for patients with significant unmet medical needs.
The $2 billion transaction represents a major step in Shionogi’s plans to establish a stronger global presence in rare diseases while building on its existing expertise in neurological and other high-need conditions.