Likely reflecting timelines associated with the discovery of SOD1 mutations in ALS, mutant SOD1 mouse models of the disease provide the most well established and broadly used models, with transgenic mice expressing high levels of human mutant SOD1 under control of the natural SOD1 gene promoter [25] developing a robust ALS-like phenotype. The gene discussed is SOD1; the disease is amyotrophic lateral sclerosis.