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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Detaylı Bibliyografya
Asıl Yazarlar: García-Giménez, José Luis, Gimeno, Amparo, Gonzalez-Cabo, Pilar, Dasí, Francisco, Bolinches-Amorós, Arantxa, Mollá, Belén, Palau, Francesc, Pallardó, Federico V.
Materyal Türü: Artigo
Dil:Inglês
Baskı/Yayın Bilgisi: Public Library of Science 2011
Konular:
Research Article
Online Erişim: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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