Ataxin-1 (ATX1) is the protein responsible for the currently incurable spinocerebellar ataxia type 1 (SCA1), a dominant neurodegenerative misfolding disease characterised by ataxia, progressive motor deterioration [1], [2] and degeneration of selected neuronal subtypes [3]. The gene discussed is ATXN1; the disease is cerebellar ataxia.