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Lees-Shepard, John B; Yamamoto, Masakazu; Biswas, Arpita A; Stoessel, Sean J; Nicholas, Sarah-Anne E; Cogswell, Cathy A; Devarakonda, Parvathi M; Schneider, Jr, Michael J; Cummins, Samantha M; Legendre, Nicholas P; Yamamoto, Shoko; Kaartinen, Vesa; Hunter, Jeffrey W; Goldhamer, David J
Nature communications, 02/2018, Letnik: 9, Številka: 1Journal Article
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 ). 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 patients. ACVR1(R206H)-expressing FAPs, but not wild-type FAPs, activate osteogenic signaling in response to activin ligands. Conditional loss of the wild-type Acvr1 allele dramatically exacerbates FAP-directed HO, suggesting that mutant and wild-type ACVR1 receptor complexes compete for activin ligands or type II BMP receptor binding partners. Finally, systemic inhibition of activin A completely blocks HO and restores wild-type-like behavior to transplanted Acvr1 FAPs. Understanding the cells that drive HO may facilitate the development of cell-specific therapeutic approaches to inhibit catastrophic bone formation in FOP.
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