PGC-1alpha down-regulation affects the antioxidant response in Friedreich's ataxia

Cells 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 P...

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Veröffentlicht in:	PloS one 2010-04, Vol.5 (4), p.e10025
Hauptverfasser:	Marmolino, Daniele, Manto, Mario, Acquaviva, Fabio, Vergara, Paola, Ravella, Ajay, Monticelli, Antonella, Pandolfo, Massimo
Format:	Artikel
Sprache:	eng
Schlagworte:	Animal models Animals Antioxidants Antioxidants (Nutrients) Antioxidants - metabolism Ataxia Bioenergetics Biosynthesis Case-Control Studies Cell Biology/Cellular Death and Stress Responses Cells, Cultured Cerebellum Control Cyclic AMP-Dependent Protein Kinases Deoxyribonucleic acid Diabetes DNA Down-regulation Down-Regulation - genetics Enzymes Fibroblasts Friedreich Ataxia - metabolism Friedreich Ataxia - pathology Friedreich's ataxia Genetics and Genomics/Gene Function Heat-Shock Proteins - genetics Humans Hydrogen Hydrogen peroxide Hydrogen Peroxide - pharmacology Hyperglycemia Kinases Medicin och hälsovetenskap Metabolism Mice Mice, Knockout Mitochondria Mitochondrial DNA Molecular biology Musculoskeletal system Mutation Neurological Disorders Neurological Disorders/Neurogenetics Neuroscience/Neurobiology of Disease and Regeneration Oxidative Stress Pathogenesis Patients Peroxisome Proliferator-Activated Receptor Gamma Coactivator 1-alpha Pharmacology Pioglitazone PPAR gamma Protein kinase Protein kinase A Protein kinases Proteins Rodents siRNA Spinal cord Stress response Superoxide dismutase Superoxide Dismutase - analysis Superoxides Transcription Transcription Factors - genetics Yeast
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