The study of disease-associated mutations, the most common of which occur in SOD1 (Superoxide dismutase 1), C9ORF72 (C9 open reading frame 72), FUS (Fused in sarcoma) and TARDBP (Transactive response DNA binding protein 43 kDa) indicate that abnormalities in pathways downstream of these mutated proteins are causal for neurodegeneration in ALS [9–13]. The gene discussed is SOD1; the disease is amyotrophic lateral sclerosis.