To examine the autophagy pathway in SCA2 we used different disease models: (i) mouse neuroblastoma (Neuro2a) cells expressing the causative mutant protein (expanded ATXN2 [ataxin 2]); (ii) a new SCA2 lentiviral mouse model with striatal pathology, generated and characterized in this study; and (iii) striatal and cerebellar patient-derived post-mortem tissues [1]. This evidence concerns the gene ATXN2 and neuroblastoma.