Motor neurons cultured from SOD1-G93A amyotrophic lateral sclerosis model mice or neuroblastoma spinal cord (NSC)34 motor neuron-like cells expressing mutated SOD1 have diminished Nrf2 activity [154,155], and transcriptomic analysis of NSC34 cells expressing mutated SOD1 show down-regulated Nrf2 and Nrf2 target genes [156]. This evidence concerns the gene NFE2L2 and amyotrophic lateral sclerosis.