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Transgenic mice expressing tunable levels of DUX4 develop characteristic facioscapulohumeral muscular dystrophy-like pathophysiology ranging in severity

BACKGROUND: All types of facioscapulohumeral muscular dystrophy (FSHD) are caused by the aberrant activation of the somatically silent DUX4 gene, the expression of which initiates a cascade of cellular events ultimately leading to FSHD pathophysiology. Typically, progressive skeletal muscle weakness...

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Detalles Bibliográficos
Autores principales: Jones, Takako I., Chew, Guo-Liang, Barraza-Flores, Pamela, Schreier, Spencer, Ramirez, Monique, Wuebbles, Ryan D., Burkin, Dean J., Bradley, Robert K., Jones, Peter L.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7149937/
https://www.ncbi.nlm.nih.gov/pubmed/32278354
http://dx.doi.org/10.1186/s13395-020-00227-4