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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Bibliografski detalji
Glavni autori: García-Giménez, José Luis, Gimeno, Amparo, Gonzalez-Cabo, Pilar, Dasí, Francisco, Bolinches-Amorós, Arantxa, Mollá, Belén, Palau, Francesc, Pallardó, Federico V.
Format: Artigo
Jezik:Inglês
Izdano: Public Library of Science 2011
Teme:
Research Article
Online pristup: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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