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; Arciello et al., 2011; DuVal et al., 2014). This evidence concerns the gene SOD1 and amyotrophic lateral sclerosis.