ATXN8 and spinocerebellar ataxia type 8: Unexpectedly, SCA8 patients and BAC-EXP mice were found to have 1C2-positive intranuclear inclusions in Purkinje cells and brainstem neurons that result from the expression of a nearly pure polyglutamine protein (ataxin 8) from a novel gene spanning the repeat in the opposite CAG direction (ATXN8).