KEAP1 and Friedreich ataxia: Also, 1 μM EPI-743 has been found to increase mRNA expression and protein levels of NRF2 and its downstream genes, namely NQO1, heme-oxygenase-1 (HO-1) and γ-glutamyl cysteine ligase (GCL) regulated by of KEAP1-DJ-1-p62 in fibroblasts derived from patients with FRDA [117].