Dysregulation of KEAP1-NRF2 activity has been extensively demonstrated in cellular and animal models of ALS and in postmortem ALS motor cortex and spinal cord samples, so dysregulation of the NRF2/ARE antioxidant and cytoprotective pathway could be a possible mechanism underlying the progressive neurodegeneration in ALS and severity of the disease. This evidence concerns the gene KEAP1 and amyotrophic lateral sclerosis.