Ontology highlight
ABSTRACT:
SUBMITTER: Lees-Shepard JB
PROVIDER: S-EPMC5797136 | biostudies-literature | 2018 Feb
REPOSITORIES: biostudies-literature
Lees-Shepard John B JB Yamamoto Masakazu M Biswas Arpita A AA Stoessel Sean J SJ Nicholas Sarah-Anne E SE Cogswell Cathy A CA Devarakonda Parvathi M PM Schneider Michael J MJ Cummins Samantha M SM Legendre Nicholas P NP Yamamoto Shoko S Kaartinen Vesa V Hunter Jeffrey W JW Goldhamer David J DJ
Nature communications 20180202 1
Fibrodysplasia ossificans progressiva (FOP) is a rare autosomal-dominant disorder characterized by progressive and profoundly disabling heterotopic ossification (HO). Here we show that fibro/adipogenic progenitors (FAPs) are a major cell-of-origin of HO in an accurate genetic mouse model of FOP (Acvr1 <sup>tnR206H</sup> ). Targeted expression of the disease-causing type I bone morphogenetic protein (BMP) receptor, ACVR1(R206H), to FAPs recapitulates the full spectrum of HO observed in FOP patien ...[more]