These findings confirm previous studies showing a frataxin-mediated Nrf2 deficiency in cell and mouse models of FRDA (Paupe et al., 2009; D’Oria et al., 2013; Piermarini et al., 2016; Anzovino et al., 2017; Petrillo et al., 2017) but, additionally, they represent a progress in understanding the pathogenesis of FRDA because, for the first time, an early impairment of Nrf2 signaling is described already during neurogenesis. The gene discussed is FXN; the disease is Friedreich ataxia.