Since the different phenotypes observed depended not only on the transgene expression level but also on the cellular type targeted [225,226], a new model has been generated by introducing ALS-related human SOD1 mutations at the conserved residues in fly SOD1, thereby creating fly SOD1G85R, SOD1H71Y, and SOD1H48R mutants [227]. This evidence concerns the gene SOD1 and amyotrophic lateral sclerosis.