As several studies show Nrf2 impairment in post-natal tissues of FRDA patients and in frataxin-deficient cells (Paupe et al., 2009; D’Oria et al., 2013; Shan et al., 2013; Petrillo et al., 2017), we analyzed Nrf2 expression in KIKO and WT NSCs, in order to evidence a potential involvement of the transcription factor in the defects described above. Here, FXN is linked to Friedreich ataxia.