Mice expressing mutant ataxin-1 without NLS did not show any SCA1 disease signatures, while mice expressing mutant ataxin-1 containing a deletion within the self-association region spanning amino acids 495–605 developed ataxia and PC pathology in the absence of nuclear ataxin-1 aggregates [33,34,35]. This evidence concerns the gene ATXN1 and cerebellar ataxia.