In support of this hypothesis, a neuroprotective role played by Nrf2 pathway activation in mutSOD1-associated ALS has been demonstrated by Vargas and coworkers who generated SOD1-G93A mice overexpressing Nrf2 specifically in astrocytes [122] and in neurons or type II skeletal muscle fibers [123]. This evidence concerns the gene SOD1 and amyotrophic lateral sclerosis.