About 60% of familiar and 10% of sporadic ALS cases are due to pathogenic mutation in superoxide dismutase 1 (SOD1), TAR DNA-binding protein (TARDBP), fused in sarcoma (FUS) and chromosome 9 open reading frame 72 (C9orf72), the four most common ALS-associated genes [6]. The gene discussed is SOD1; the disease is amyotrophic lateral sclerosis.