SOD1 and amyotrophic lateral sclerosis: SOD1G93A variant led to the development of the firstmouse model for ALS where mutant human SOD1G93A is ubiquitouslyoverexpressed. Despite 3 decades of investigation,the primary molecular mechanisms by which SOD1 mutations cause MNdisease remain unclear, with a plethora of toxic mechanisms proposed(see below), likely through the generation of misfolded SOD1, independentof alterations in its dismutase activity.