Gurney et al. (1994) were the first to recapitulate the hallmarks of ALS using a transgenic mouse model (SOD1 G93A mutant mouse) that expresses human copies of SOD1 enzyme with glycine-alanine substitution at the 93rd codon of the coding sequence (Gurney et al., 1994; Rothstein et al., 1995). Over the years, a variation of SOD1 mutants have been subsequently developed as G37R, G85R, and G86R transgenic; although these models differ in protein content, they develop the same MN degeneration that is characteristic of ALS (Turner and Talbot, 2008). Here, SOD1 is linked to amyotrophic lateral sclerosis.