These observations remind to the general concept of prion-like induction and spreading of pathogenic proteins that has been recently expanded to include aggregates of tau, α-synuclein, huntingtin, superoxide dismutase-1, and TDP-43, which characterize several human neurodegenerative disorders such as frontotemporal lobar degeneration, Parkinson’s/Lewy body disease, Huntington’s disease and amyotrophic lateral sclerosis46,49,50. Here, SOD1 is linked to juvenile Huntington disease.