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Bioenergetic Impairment in Congenital Muscular Dystrophy Type 1A and Leigh Syndrome Muscle Cells

Skeletal muscle has high energy requirement and alterations in metabolism are associated with pathological conditions causing muscle wasting and impaired regeneration. Congenital muscular dystrophy type 1A (MDC1A) is a severe muscle disorder caused by mutations in the LAMA2 gene. Leigh syndrome (LS)...

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Detalles Bibliográficos
Autores principales: Fontes-Oliveira, Cibely C., Steinz, Maarten, Schneiderat, Peter, Mulder, Hindrik, Durbeej, Madeleine
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5377256/
https://www.ncbi.nlm.nih.gov/pubmed/28367954
http://dx.doi.org/10.1038/srep45272