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

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 hu...

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Autores principales:	Darko Bosnakovski, Sunny S. K. Chan, Olivia O. Recht, Lynn M. Hartweck, Collin J. Gustafson, Laura L. Athman, Dawn A. Lowe, Michael Kyba
Formato:	article
Lenguaje:	EN
Publicado:	Nature Portfolio 2017
Materias:	Science Q
Acceso en línea:	https://doaj.org/article/9516fa4c1ad1467299b0aa2dfdddf207
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spelling	oai:doaj.org-article:9516fa4c1ad1467299b0aa2dfdddf2072021-12-02T15:38:42ZMuscle pathology from stochastic low level DUX4 expression in an FSHD mouse model10.1038/s41467-017-00730-12041-1723https://doaj.org/article/9516fa4c1ad1467299b0aa2dfdddf2072017-09-01T00:00:00Zhttps://doi.org/10.1038/s41467-017-00730-1https://doaj.org/toc/2041-1723Facioscapulohumeral 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.Darko BosnakovskiSunny S. K. ChanOlivia O. RechtLynn M. HartweckCollin J. GustafsonLaura L. AthmanDawn A. LoweMichael KybaNature PortfolioarticleScienceQENNature Communications, Vol 8, Iss 1, Pp 1-9 (2017)
institution	DOAJ
collection	DOAJ
language	EN
topic	Science Q
spellingShingle	Science Q Darko Bosnakovski Sunny S. K. Chan Olivia O. Recht Lynn M. Hartweck Collin J. Gustafson Laura L. Athman Dawn A. Lowe Michael Kyba Muscle pathology from stochastic low level DUX4 expression in an FSHD mouse model
description	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.
format	article
author	Darko Bosnakovski Sunny S. K. Chan Olivia O. Recht Lynn M. Hartweck Collin J. Gustafson Laura L. Athman Dawn A. Lowe Michael Kyba
author_facet	Darko Bosnakovski Sunny S. K. Chan Olivia O. Recht Lynn M. Hartweck Collin J. Gustafson Laura L. Athman Dawn A. Lowe Michael Kyba
author_sort	Darko Bosnakovski
title	Muscle pathology from stochastic low level DUX4 expression in an FSHD mouse model
title_short	Muscle pathology from stochastic low level DUX4 expression in an FSHD mouse model
title_full	Muscle pathology from stochastic low level DUX4 expression in an FSHD mouse model
title_fullStr	Muscle pathology from stochastic low level DUX4 expression in an FSHD mouse model
title_full_unstemmed	Muscle pathology from stochastic low level DUX4 expression in an FSHD mouse model
title_sort	muscle pathology from stochastic low level dux4 expression in an fshd mouse model
publisher	Nature Portfolio
publishDate	2017
url	https://doaj.org/article/9516fa4c1ad1467299b0aa2dfdddf207
work_keys_str_mv	AT darkobosnakovski musclepathologyfromstochasticlowleveldux4expressioninanfshdmousemodel AT sunnyskchan musclepathologyfromstochasticlowleveldux4expressioninanfshdmousemodel AT oliviaorecht musclepathologyfromstochasticlowleveldux4expressioninanfshdmousemodel AT lynnmhartweck musclepathologyfromstochasticlowleveldux4expressioninanfshdmousemodel AT collinjgustafson musclepathologyfromstochasticlowleveldux4expressioninanfshdmousemodel AT lauralathman musclepathologyfromstochasticlowleveldux4expressioninanfshdmousemodel AT dawnalowe musclepathologyfromstochasticlowleveldux4expressioninanfshdmousemodel AT michaelkyba musclepathologyfromstochasticlowleveldux4expressioninanfshdmousemodel
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Muscle pathology from stochastic low level DUX4 expression in an FSHD mouse model

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