GCLC and Friedreich ataxia: Additionally, Petrillo et al. [117] have shown that regulation of KEAP1-DJ-1-p62 activates the NRF2-mediated antioxidant pathway in fibroblasts derived from patients with FRDA, leading to increased GSH content, mRNA expression and protein level of NQO1, HO-1 and GCL. The observation was associated with a robust increase in the expression of FXN mRNA [117,139].