Natively folded proteins involved in amyloidosis are for instance transthyretin (TTR) associated to familial amyloidotic cardiomyopathy, superoxide dismutase-1 (SOD-1) and transactive response DNA binding protein 43 (TDP-43) associated to familial amyotrophic lateral sclerosis (fALS), Huntingtin (Htt) associated to Huntington’s disease (HD), or the cellular form of prion protein PrPC associated to prion diseases or transmissible spongiform encephalopathies (TSEs). The gene discussed is SOD1; the disease is Huntington disease.