Despite their common genetic cause, each SCA type has a different gene location of the repeat and presents unique clinical features, potentially differing in certain mechanisms involved in pathology progression. In SCA1, a cross-species screen (human cells vs. Drosophila) identified 22 regulators of the mutant ATXN1 protein aggregation. Here, ATXN1 is linked to autosomal dominant cerebellar ataxia.