Since the identification of mutations in the SOD1 gene as an important cause of familial ALS [3] and the generation of the SOD1 mouse model of ALS [4], genetic modifications on SOD1 mouse model have been described, mostly focusing on the putative disease mechanisms, such as oxidative stress, excitotoxicity, axonal transport defects, mitochondrial dysfunction, and apoptosis [49]. Here, SOD1 is linked to amyotrophic lateral sclerosis.