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pubmed-article:20383327pubmed:abstractTextCells from individuals with Friedreich's ataxia (FRDA) show reduced activities of antioxidant enzymes and cannot up-regulate their expression when exposed to oxidative stress. This blunted antioxidant response may play a central role in the pathogenesis. We previously reported that Peroxisome Proliferator Activated Receptor Gamma (PPARgamma) Coactivator 1-alpha (PGC-1alpha), a transcriptional master regulator of mitochondrial biogenesis and antioxidant responses, is down-regulated in most cell types from FRDA patients and animal models.lld:pubmed
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pubmed-article:20383327pubmed:articleTitlePGC-1alpha down-regulation affects the antioxidant response in Friedreich's ataxia.lld:pubmed
pubmed-article:20383327pubmed:affiliationLaboratoire de Neurologie Expérimentale, Université Libre de Bruxelles, Brussels, Belgium.lld:pubmed
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