Recent work has linked the UPS to the secretion of aggregate-prone proteins, such as amyloid beta and hyperphosphorylated tau in Alzheimer’s disease, mutant huntingtin in Huntington’s disease, and superoxide dismutase 1 (SOD1) and TDP43 in Amyotrophic Lateral Sclerosis (ALS)10–15. The gene discussed is SOD1; the disease is juvenile Huntington disease.