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

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...

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Auteurs principaux:	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
Format:	article
Langue:	EN
Publié:	Nature Portfolio 2018
Sujets:	Science Q
Accès en ligne:	https://doaj.org/article/cce620ded4304ad1a71c991a24918aac
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spelling	oai:doaj.org-article:cce620ded4304ad1a71c991a24918aac2021-12-02T17:32:02ZActivin-dependent signaling in fibro/adipogenic progenitors causes fibrodysplasia ossificans progressiva10.1038/s41467-018-02872-22041-1723https://doaj.org/article/cce620ded4304ad1a71c991a24918aac2018-02-01T00:00:00Zhttps://doi.org/10.1038/s41467-018-02872-2https://doaj.org/toc/2041-1723Fibrodysplasia 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.John B. Lees-ShepardMasakazu YamamotoArpita A. BiswasSean J. StoesselSarah-Anne E. NicholasCathy A. CogswellParvathi M. DevarakondaMichael J. SchneiderSamantha M. CumminsNicholas P. LegendreShoko YamamotoVesa KaartinenJeffrey W. HunterDavid J. GoldhamerNature PortfolioarticleScienceQENNature Communications, Vol 9, Iss 1, Pp 1-14 (2018)
institution	DOAJ
collection	DOAJ
language	EN
topic	Science Q
spellingShingle	Science Q 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 Activin-dependent signaling in fibro/adipogenic progenitors causes fibrodysplasia ossificans progressiva
description	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.
format	article
author	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
author_facet	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
author_sort	John B. Lees-Shepard
title	Activin-dependent signaling in fibro/adipogenic progenitors causes fibrodysplasia ossificans progressiva
title_short	Activin-dependent signaling in fibro/adipogenic progenitors causes fibrodysplasia ossificans progressiva
title_full	Activin-dependent signaling in fibro/adipogenic progenitors causes fibrodysplasia ossificans progressiva
title_fullStr	Activin-dependent signaling in fibro/adipogenic progenitors causes fibrodysplasia ossificans progressiva
title_full_unstemmed	Activin-dependent signaling in fibro/adipogenic progenitors causes fibrodysplasia ossificans progressiva
title_sort	activin-dependent signaling in fibro/adipogenic progenitors causes fibrodysplasia ossificans progressiva
publisher	Nature Portfolio
publishDate	2018
url	https://doaj.org/article/cce620ded4304ad1a71c991a24918aac
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Activin-dependent signaling in fibro/adipogenic progenitors causes fibrodysplasia ossificans progressiva

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