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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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Detalles Bibliográficos
Autores principales:	García-Giménez, José Luis, Gimeno, Amparo, Gonzalez-Cabo, Pilar, Dasí, Francisco, Bolinches-Amorós, Arantxa, Mollá, Belén, Palau, Francesc, Pallardó, Federico V.
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Public Library of Science 2011
Materias:	Research Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3110204/ https://www.ncbi.nlm.nih.gov/pubmed/21687738 http://dx.doi.org/10.1371/journal.pone.0020666

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