A Japanese drugmaker is making another bet on rare diseases. Shionogi & Co. said Monday that it will buy IntraBio, a biotech company based in Austin, Texas, for $2 billion paid upfront, Jiji Press reported via Nippon.com.

IntraBio would become a wholly owned unit of Shionogi's New Jersey-based U.S. subsidiary, Shionogi said in its announcement. The company expects the deal to close in November or December, once competition and other regulators sign off.

The prize is a drug called Aqneursa, which grew out of research at the University of Oxford, BioPharma Dive reported. The FDA approved it in September 2024 for the brain and nerve symptoms of Niemann-Pick disease type C, an ultra-rare inherited disorder in which fat and cholesterol build up in the brain and other organs. It affects about one in every 100,000 babies born, Shionogi said. European regulators approved it in January.

On Sept. 18, the FDA also approved Aqneursa to treat loss of movement control, called ataxia, in patients with ataxia-telangiectasia, another rare genetic disease. Shionogi said that makes it the first and only drug approved for that use. European regulators are now reviewing it for that use too.

IntraBio is still small. Founded in 2015, it had $67.9 million in sales in 2025, up from $3.5 million the year before, and it posted a net loss of $35.8 million, according to figures in Shionogi's announcement.

"The planned acquisition of IntraBio actively demonstrates Shionogi's solid commitment to building a leading global rare disease business," said CEO Isao Teshirogi.

Some background: Shionogi is best known for medicines that fight infections, including the COVID-19 drug Xocova. It took over global rights to edaravone, an ALS drug sold as Radicava in the United States, and says that deal built its base in rare diseases. Jiji noted that the company has made rare diseases a priority area. IntraBio is also testing treatments for Pompe disease, Fragile X syndrome and Jordan's syndrome.

Shionogi said it is still reviewing how the deal will affect its results for the fiscal year ending in March 2027, BioPharma Dive noted.

What we know: Shionogi will pay $2 billion upfront, with closing planned by year-end.
What we don't: How the deal affects Shionogi's profits, and how fast Aqneursa sales grow.