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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Autores principales: 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
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Publicado: Nature Portfolio 2018
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Acceso en línea: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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