SOD1 and amyotrophic lateral sclerosis: Since the first mutation identified in familiar ALS was in the SOD1 gene (Rosen et al., 1993), the SOD1G93A mouse is the most widely used ALS model, which develops the main clinical, electrophysiological and histopathological features of both familial and sporadic forms of the disease (Turner and Talbot, 2008; Mancuso et al., 2011b).