SOD1 and amyotrophic lateral sclerosis: Interestingly, the TF regulating GCL expression, i.e., the nuclear factor erythroid-derived 2-like 2 (Nrf2), was found to be downregulated in post-mortem tissues from ALS patients [74] and in the hSOD1(G93A) mouse model [75], while Nrf2 overexpression in astrocytes had a significant beneficial effect in ALS mouse models [76].