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Expression of FSHD-related DUX4-FL alters proteostasis and induces TDP-43 aggregation

OBJECTIVE: Pathogenesis in facioscapulohumeral muscular dystrophy (FSHD) appears to be due to aberrant expression, particularly in skeletal muscle nuclei, of the full-length isoform of DUX4 (DUX4-FL). Expression of DUX4-FL is known to alter gene expression and to be cytotoxic, but cell responses to...

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Detalles Bibliográficos
Autores principales:	Homma, Sachiko, Beermann, Mary Lou, Boyce, Frederick M, Miller, Jeffrey Boone
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	BlackWell Publishing Ltd 2015
Materias:	Research Articles
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4338956/ https://www.ncbi.nlm.nih.gov/pubmed/25750920 http://dx.doi.org/10.1002/acn3.158

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