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Activation of the PPAR/PGC-1α pathway prevents a bioenergetic deficit and effectively improves a mitochondrial myopathy phenotype
Neuromuscular disorders with defects in the mitochondrial ATP generating system affect a large number of children and adults worldwide, but remain without treatment. We used a mouse model of mitochondrial myopathy, caused by a cytochrome c oxidase deficiency, to evaluate the effect of induced mitoch...
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| Autors principals: | , , , |
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| Format: | Artigo |
| Idioma: | Inglês |
| Publicat: |
2008
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| Matèries: | |
| Accés en línia: | https://ncbi.nlm.nih.gov/pmc/articles/PMC2613643/ https://ncbi.nlm.nih.gov/pubmed/18762025 https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.cmet.2008.07.006 |
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