Oxidative stress and increased levels of free radicals play an important role in FRDA pathogenesis (Lupoli et al., 2018), where frataxin deficiency has been shown to reduce the expression of Nrf2 (the Nuclear factor erythroid-derived 2)-like 2 transcription factor NE2FL2 either in human FRDA fibroblasts (Paupe et al., 2009; Petrillo et al., 2017) and in mouse models of the disease (D’Oria et al., 2013; Shan et al., 2013; Anzovino et al., 2017; Dinkova-Kostova et al., 2018). Here, FXN is linked to Friedreich ataxia.