Biopharmaceutical company Ipsen (Euronext:IPN) (ADR:IPSEY) on Wednesday announced an exclusive, worldwide license agreement for the development and commercialization of BLU-782 for the treatment of fibrodysplasia ossificans progressiva (FOP), a rare, severely disabling genetic disorder characterised by the abnormal transformation of muscle, ligaments and tendons into bone.

The agreement is between Ipsen's subsidiary Clementia Pharmaceuticals and Blueprint Medicines Corporation (NASDAQ:BPMC).

Through this agreement, Ipsen enhances its Rare Diseases portfolio by advancing Blueprint Medicines' goal of rapidly and efficiently developing BLU-782 , an oral, highly selective investigational ALK2 inhibitor, as a potential treatment for patients with FOP.

BLU-782 was designed by Blueprint Medicines to selectively target mutant ALK2, the underlying cause of FOP, using its proprietary scientific platform. A Phase 1 clinical trial of BLU-782 in healthy volunteers showed that BLU-782 was well-tolerated at all doses tested.

Under the terms of the license agreement, Blueprint Medicines will be eligible to receive up to USD535m in upfront, milestone and other payments, including an upfront cash payment of USD25m and up to USD510m in potential milestone payments related to specified development, regulatory and sales-based milestones for licensed products in up to two indications, including FOP.

In conjunction with the agreement, Ipsen will pay Blueprint Medicines tiered percentage royalties ranging from the low- to mid-teens on worldwide aggregate annual net sales of licensed products, subject to adjustment in specified circumstances under the license agreement.