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Shionogi acquires rare disease startup IntraBio for $2 billion

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Shionogi & Co. agreed to acquire Austin, Texas-based IntraBio for $2 billion, adding an approved rare disease drug and a pipeline of neurodegenerative disease programs to its portfolio.

Under the terms of the deal, signed Monday, Shionogi's U.S. division — headquartered in New Jersey — will purchase all of IntraBio's shares, absorbing the biotech as a wholly owned subsidiary. The transaction is expected to close between November and December, the company said.

Through the acquisition, Shionogi will take ownership of worldwide rights to Aqneursa (levacetylleucine), encompassing both intellectual property and commercialization rights. The drug is approved in the U.S. for the treatment of neurological manifestations of Niemann-Pick disease type C, a rare inherited disorder that impairs the movement of cholesterol and other lipids within cells. The U.S. Food and Drug Administration approved Aqneursa for that condition in September 2024 and the European Medicines Agency followed in January 2026.

The FDA granted Aqneursa a second approval on September 18, 2026, for the treatment of ataxia in patients with Ataxia-Telangiectasia, a rare progressive neurodegenerative disease that typically begins in early childhood. The drug is currently under review by the EMA for that condition as well. IntraBio recorded $68 million in sales in 2025, the company said.

Shionogi said IntraBio's rare disease capabilities would also support its broader pipeline, which includes clinical programs in Pompe disease, Fragile X syndrome, and Jordan's syndrome.

The deal extends a rare disease push that Shionogi began when it brought the ALS therapy Radicava into its portfolio in April 2026 by securing worldwide rights to edaravone from Tanabe Pharma. "Bringing Aqneursa to Shionogi after our acquisition of Radicava will deepen our commitment to rare disease communities, expand our capabilities and strengthen our portfolio as we advance future innovation for patients with significant unmet needs," CEO Isao Teshirogi said in a statement.

Shionogi said the transaction's impact on its consolidated financial results for the fiscal year ending March 2027 is under review.

Goldman Sachs & Co. LLC provided financial advice to Shionogi, and Cleary Gottlieb Steen & Hamilton LLP served as its legal advisor. Centerview Partners LLC and Morgan Stanley & Co. LLC served as financial advisors to IntraBio, with Kirkland & Ellis LLP and DLA Piper LLP acting as legal advisors.

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