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

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

doi:10.1038/s41467-018-02872-2

Nature Communications (Feb 2018)

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

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

Affiliations

John B. Lees-Shepard: Department of Molecular and Cell Biology, University of Connecticut Stem Cell Institute, University of Connecticut
Masakazu Yamamoto: Department of Molecular and Cell Biology, University of Connecticut Stem Cell Institute, University of Connecticut
Arpita A. Biswas: Department of Molecular and Cell Biology, University of Connecticut Stem Cell Institute, University of Connecticut
Sean J. Stoessel: Department of Molecular and Cell Biology, University of Connecticut Stem Cell Institute, University of Connecticut
Sarah-Anne E. Nicholas: Department of Molecular and Cell Biology, University of Connecticut Stem Cell Institute, University of Connecticut
Cathy A. Cogswell: Department of Molecular and Cell Biology, University of Connecticut Stem Cell Institute, University of Connecticut
Parvathi M. Devarakonda: Department of Molecular and Cell Biology, University of Connecticut Stem Cell Institute, University of Connecticut
Michael J. Schneider: Department of Molecular and Cell Biology, University of Connecticut Stem Cell Institute, University of Connecticut
Samantha M. Cummins: Department of Molecular and Cell Biology, University of Connecticut Stem Cell Institute, University of Connecticut
Nicholas P. Legendre: Department of Molecular and Cell Biology, University of Connecticut Stem Cell Institute, University of Connecticut
Shoko Yamamoto: Department of Molecular and Cell Biology, University of Connecticut Stem Cell Institute, University of Connecticut
Vesa Kaartinen: Department of Biological and Materials Sciences, School of Dentistry, University of Michigan
Jeffrey W. Hunter: Alexion Pharmaceuticals
David J. Goldhamer: Department of Molecular and Cell Biology, University of Connecticut Stem Cell Institute, University of Connecticut

DOI: https://doi.org/10.1038/s41467-018-02872-2
Journal volume & issue: Vol. 9, no. 1
pp. 1 – 14

Abstract

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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.

Published in Nature Communications

ISSN: 2041-1723 (Online)
Publisher: Nature Portfolio
Country of publisher: United Kingdom
LCC subjects: Science
Website: https://www.nature.com/ncomms/

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