SOD1 and amyotrophic lateral sclerosis: As the majority of familial ALS cases are linked to the mutations in SOD1 gene (Dion et al., 2009), transgenic mice expressing human mutant SOD1 (mSOD1) developing age-dependent clinical and pathological features of human ALS are current largely employed in the physiopathological studies of the disorder (Turner and Talbot, 2008).