Albeit a disorganization of the cytoskeleton in FRDA cells [17] and an upregulation of the Nrf2 inhibitor Keap-1 in a FRDA mouse model [57] have been specifically involved in this disease, the molecular mechanism linking frataxin depletion to Nrf2 disablement still remains to be understood. This evidence concerns the gene FXN and Friedreich ataxia.