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

Facioscapulohumeral muscular dystrophy is a slowly progressive but devastating myopathy caused by loss of repression of the transcription factor DUX4; however, DUX4 expression is very low, and protein has not been detected directly in patient biopsies. Efforts to model DUX4 myopathy in mice have fou...

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Detalles Bibliográficos
Autores principales: Bosnakovski, Darko, Chan, Sunny S. K., Recht, Olivia O., Hartweck, Lynn M., Gustafson, Collin J., Athman, Laura L., Lowe, Dawn A., Kyba, Michael
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group UK 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5601940/
https://www.ncbi.nlm.nih.gov/pubmed/28916757
http://dx.doi.org/10.1038/s41467-017-00730-1