We show that lack of PGC-1α induction parallels the lack of SOD2 induction in FRDA fibroblasts exposed to H2O2, that PGC-1α silencing by siRNA in normal fibroblasts mimics the lack of antioxidant response found in FRDA cells, and that pharmacological PGC-1α up-regulation obtained by stimulating its activators PPARγ or AMPK can restore the SOD2 response in H2O2 stressed FRDA cells. This evidence concerns the gene PPARGC1A and Friedreich ataxia.