Differential Expression of PGC-1α and Metabolic Sensors Suggest Age-Dependent Induction of Mitochondrial Biogenesis in Friedreich Ataxia Fibroblasts

BACKGROUND: Friedreich's ataxia (FRDA) is a mitochondrial rare disease, which molecular origin is associated with defect in the expression of frataxin. The pathological consequences are degeneration of nervous system structures and cardiomyopathy with necrosis and fibrosis, among others. PRINCI...

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Main Authors: García-Giménez, José Luis, Gimeno, Amparo, Gonzalez-Cabo, Pilar, Dasí, Francisco, Bolinches-Amorós, Arantxa, Mollá, Belén, Palau, Francesc, Pallardó, Federico V.
Formato: Artigo
Idioma:Inglês
Publicado em: Public Library of Science 2011
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Research Article
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC3110204/
https://ncbi.nlm.nih.gov/pubmed/21687738
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1371/journal.pone.0020666
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