This mouse is widely used and carries a human mutant transgene array with a glycine to alanine mutation at residue 93 of SOD1, which is causative for ALS in humans, and which results in a similar phenotype in the mice who succumb to endstage disease at ∼130 d of age (depending on genetic background)[19]. This evidence concerns the gene SOD1 and amyotrophic lateral sclerosis.