The prime examples of ALS proteinopathy are inclusions formed by mutated superoxide dismutase 1 (SOD1) (3, 4) and ubiquitylated/phosphorylated TAR DNA-binding protein 43 (TDP43) (5) that cannot be cleared by proteasomal and autophagic degradation machinery. The gene discussed is SOD1; the disease is amyotrophic lateral sclerosis.