In fact, the observations showing that ALS-related genes such as C9orf72 (the most frequent genetic cause of ALS; DeJesus-Hernandez et al., 2011), TARDBP, SOD1 and FUS are involved in several and different biological processes (e.g., RNA metabolism, autophagy, DNA repair; Gao et al., 2017; Hardiman et al., 2017) makes the pathological landscape of this disorder extremely puzzling. This evidence concerns the gene SOD1 and amyotrophic lateral sclerosis.