Indeed, the G93A mutation of SOD1 has been widely used to generate model systems of ALS, either animals or in vitro, as they mimic the main clinical, pathological, and cellular features of the disease (Gurney et al., 1994; Pasinelli et al., 1998; Arciello et al., 2011). The gene discussed is SOD1; the disease is amyotrophic lateral sclerosis.