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Muscle pathology from stochastic low level DUX4 expression in an FSHD mouse model

Authors :
Darko Bosnakovski
Sunny S. K. Chan
Olivia O. Recht
Lynn M. Hartweck
Collin J. Gustafson
Laura L. Athman
Dawn A. Lowe
Michael Kyba
Source :
Nature Communications, Vol 8, Iss 1, Pp 1-9 (2017)
Publication Year :
2017
Publisher :
Nature Portfolio, 2017.

Abstract

Facioscapulohumeral muscular dystrophy is a severe myopathy that is caused by abnormal activation of DUX4, and for which a suitable mouse model does not exist. Here, the authors generate a novel mouse model with titratable expression of DUX4, and show that it recapitulates several features of the human pathology.

Subjects

Subjects :
Science

Details

Language :
English
ISSN :
20411723
Volume :
8
Issue :
1
Database :
Directory of Open Access Journals
Journal :
Nature Communications
Publication Type :
Academic Journal
Accession number :
edsdoj.9516fa4c1ad1467299b0aa2dfdddf207
Document Type :
article
Full Text :
https://doi.org/10.1038/s41467-017-00730-1