NFE2L2 and Friedreich ataxia: Other NRF2 activators, such as SFN, DMF, N-acetylcysteine and vatiquinone (EPI-743), increased NRF2 expression, rebalanced the GSH/GSSG ratio, and upregulated FXN expression in fibroblasts and lymphoblastoid cells from FRDA patients [179,180].