Differential expression of PGC-1α and metabolic sensors suggest age-dependent induction of mitochondrial biogenesis in Friedreich ataxia fibroblasts.

Differential expression of PGC-1α and metabolic sensors suggest age-dependent induction of mitochondrial biogenesis in Friedreich ataxia fibroblasts.

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.Using FRDA fibrobla...

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Bibliographic Details
Main Authors: José Luis García-Giménez, Amparo Gimeno, Pilar Gonzalez-Cabo, Francisco Dasí, Arantxa Bolinches-Amorós, Belén Mollá, Francesc Palau, Federico V Pallardó
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2011-01-01
Series:PLoS ONE
Online Access:http://europepmc.org/articles/PMC3110204?pdf=render

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http://europepmc.org/articles/PMC3110204?pdf=render

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