A reduction in antioxidant defenses has been reported in fibroblasts from FRDA patients, highlighted by a deficiency in Nrf2 activation (Paupe et al., 2009), suggesting that FXN deficiency may cause lower Nrf2 activation and decreased mitochondrial antioxidant protection (Hayashi and Cortopassi, 2016). This evidence concerns the gene FXN and Friedreich ataxia.