Two tissue-resident progenitor lineages drive distinct phenotypes of heterotopic ossification.

ABSTRACT: Fibrodysplasia ossificans progressiva (FOP), a congenital heterotopic ossification (HO) syndrome caused by gain-of-function mutations of bone morphogenetic protein (BMP) type I receptor ACVR1, manifests with progressive ossification of skeletal muscles, tendons, ligaments, and joints. In this disease, HO can occur in discrete flares, often triggered by injury or inflammation, or may progress incrementally without identified triggers. Mice harboring an Acvr1^R206H knock-in allele recapitulate the phenotypic spectrum of FOP, including injury-responsive intramuscular HO and spontaneous articular, tendon, and ligament ossification. The cells that drive HO in these diverse tissues can be compartmentalized into two lineages: an Scx⁺ tendon-derived progenitor that mediates en

SUBMITTER: Dey D 

PROVIDER: S-EPMC6407419 | biostudies-literature | 2016 Nov

REPOSITORIES: biostudies-literature