Article
TGF-Beta Induces Activin A Production in Dermal Fibroblasts Derived from Patients with Fibrodysplasia Ossificans Progressiva.
International journal of molecular sciences - 24 Jan 2023
de Ruiter Ruben D, Wisse Lisanne E, Schoenmaker Ton, Yaqub Maqsood, Sánchez-Duffhues Gonzalo, Eekhoff E Marelise W, Micha Dimitra
Abstract excerpt
Fibrodysplasia ossificans progressiva (FOP) is a catastrophic, ultra-rare disease of heterotopic ossification caused by genetic defects in the ACVR1 gene. The mutant ACVR1 receptor, when triggered by an inflammatory process, leads to heterotopic ossification of the muscles and ligaments. Activin A has been discovered as the main osteogenic ligand of the FOP ACVR1 receptor. However, the source of Activin A itself...
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