Studies in a variety of model systems have reported that the ALS-associated mutations in SOD1 cause the protein to be more prone to misfold and aggregate (Johnston et al. 2000; Shinder et al. 2001; Wang et al. 2002; Elam et al. 2003; Jonsson et al. 2004; Prudencio et al. 2009). The gene discussed is SOD1; the disease is amyotrophic lateral sclerosis.