SOD1 aggregates prepared from transgenic mice overexpressing human SOD1 mutations or from an SOD1G127Xhet fALS patient, upon inoculation into the spinal cord of 100 day old mice result in propagation of SOD1 aggregation almost exclusively in the motor system with concomitant development of an ALS-like phenotype [8, 15]. This evidence concerns the gene SOD1 and amyotrophic lateral sclerosis.