Among several genes responsible for familial ALS (fALS), mutations in a gene encoding Cu,Zn-superoxide dismutase (SOD1) have been the most intensely studied [4], and, therefore, experimental data accumulated on fALS with SOD1 mutations (SOD1-ALS) would provide useful insight into the therapeutic development for the treatment of this devastating disease. Here, SOD1 is linked to amyotrophic lateral sclerosis.