Cytoplasmic and nuclear polyglutamine aggregates in SCA6 Purkinje cells

Aggregations of the alpha1A-calcium channel protein have been previously de monstrated in spinocerebellar ataxia type 6 (SCA6). Here the authors show t hat small aggregates, labeled by a monoclonal antibody 1C2 that preferentia lly detects expanded polyglutamine larger than that in SCA6 mutation, are p resent mainly in the cytoplasm but also in the nucleus of Purkinje cells. A lthough the length of expansion is small in SCA6, the current finding might indicate that SCA6 conforms to the pathogenic mechanism(s) in other polygl utamine diseases.