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Defects in Mitochondrial ATP Synthesis in Dystrophin-Deficient Mdx Skeletal Muscles May Be Caused by Complex I Insufficiency
Duchenne Muscular Dystrophy is a chronic, progressive and ultimately fatal skeletal muscle wasting disease characterised by sarcolemmal fragility and intracellular Ca(2+) dysregulation secondary to the absence of dystrophin. Mounting literature also suggests that the dysfunction of key energy system...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Public Library of Science
2014
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Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4277356/ https://www.ncbi.nlm.nih.gov/pubmed/25541951 http://dx.doi.org/10.1371/journal.pone.0115763 |