Studies in mice carrying mutation for Sod1 (i.e., SOD1-G93A), one of the genes implicated in ALS pathogenesis, convergently showed the etiology at the NMJ, of which denervation causes retrograde and progressive death of spinal MNs, interneurons and ultimately corticospinal cells [117,118,119,120,121]. This evidence concerns the gene SOD1 and amyotrophic lateral sclerosis.