The well-studied G93A-hSOD1 ALS mouse, which models a subset of the inherited disease based on mutations in the superoxide dismutase-1 gene (SOD1), has been a vital tool in progressing our understanding of ALS; this mouse exhibits behavioral symptom onset at postnatal day 90 (P90) and death by P120-140 caused by a gain-of-function toxicity mediated by the mutant SOD1 enzyme (Gurney et al., 1994). This evidence concerns the gene SOD1 and amyotrophic lateral sclerosis.