Furthermore, it has been described that ALA induces mitochondrial biogenesis by increasing the SIRT1 and PGC1α expression, which have neuroprotective properties, reducing oxidative stress, inflammation and preventing cell death [85, 86].The inhibition of PPARγ using T0070907 [87, 88], avoided the beneficial effect of ALA, suggesting that PPARγ activation is at least one of the key mechanisms through which ALA ameliorates the mutant phenotype of FRDA fibroblasts. This evidence concerns the gene SIRT1 and Friedreich ataxia.