Under our opinion, these studies indicate that the levels of CuZnSOD and the MnSOD are lower per se in all FRDA patients, (and more drastically in FRDA 1 and FRDA 2 cells) suggesting that, the mitochondrial pathogenesis of FRDA could be aggravated by deficit in these variants of superoxide dismutase and that the critical evolution of the disease with the age of patients is accompanied by the depletion of the MnSOD. This evidence concerns the gene SOD2 and Friedreich ataxia.