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PGC-1α/β induced expression partially compensates for respiratory chain defects in cells from patients with mitochondrial disorders

18 Mar 2009

Abstract excerpt

Members of the peroxisome proliferator-activated receptor gamma coactivator (PGC) family are potent inducers of mitochondrial biogenesis. We have tested the potential effect of increased mitochondrial biogenesis in cells derived from patients harboring oxidative phosphorylation defects due to either nuclear or mitochondrial DNA mutations. We found that the PGC-1alpha and/or PGC-1beta expression improved...

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PMID: 19297390
DOI: 10.1093/hmg/ddp093

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