Activin-dependent signaling in fibro/adipogenic progenitors causes fibrodysplasia ossificans progressiva

Autor: John B. Lees-Shepard, Masakazu Yamamoto, Arpita A. Biswas, Sean J. Stoessel, Sarah-Anne E. Nicholas, Cathy A. Cogswell, Parvathi M. Devarakonda, Michael J. Schneider, Samantha M. Cummins, Nicholas P. Legendre, Shoko Yamamoto, Vesa Kaartinen, Jeffrey W. Hunter, David J. Goldhamer
Jazyk: angličtina
Rok vydání: 2018
Předmět:
Zdroj: Nature Communications, Vol 9, Iss 1, Pp 1-14 (2018)
Druh dokumentu: article
ISSN: 2041-1723
DOI: 10.1038/s41467-018-02872-2
Popis: Fibrodysplasia ossificans progressiva is a severe disorder characterized by heterotopic ossification, and is caused by mutations in ACVR1. Here, the authors show that expression of mutant ACVR1 in fibro/adipogenic progenitors recapitulates disease progression, and that this can be halted by systemic inhibition of activin A in mice.
Databáze: Directory of Open Access Journals