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Neurology. 2001 Jun 26;56(12):1753-6.

Cytoplasmic and nuclear polyglutamine aggregates in SCA6 Purkinje cells.

Author information

1
Department of Neurology, (Graduate School of Medicine, Tokyo Medical and Dental University, Bunkyo-ku, Japan.

Abstract

Aggregations of the alpha1A-calcium channel protein have been previously demonstrated in spinocerebellar ataxia type 6 (SCA6). Here the authors show that small aggregates, labeled by a monoclonal antibody 1C2 that preferentially detects expanded polyglutamine larger than that in SCA6 mutation, are present mainly in the cytoplasm but also in the nucleus of Purkinje cells. Although the length of expansion is small in SCA6, the current finding might indicate that SCA6 conforms to the pathogenic mechanism(s) in other polyglutamine diseases.

PMID:
11425948
DOI:
10.1212/wnl.56.12.1753
[Indexed for MEDLINE]

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