NFE2L2 and Friedreich ataxia: 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).