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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)...
Autores principales: | Fontes-Oliveira, Cibely C., Steinz, Maarten, Schneiderat, Peter, Mulder, Hindrik, Durbeej, Madeleine |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Nature Publishing Group
2017
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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 |
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