Moreover, in the motor cortex of ALS patients, the levels of NRF2 mRNA and protein were reduced, whereas Keap1 mRNA expression was increased compared to control patients (Sarlette et al., 2008; Tanji et al., 2013), suggesting that the NRF2-EpRE pathway is dysfunctional in ALS. This evidence concerns the gene KEAP1 and amyotrophic lateral sclerosis.