Cytoplasmic and nuclear polyglutamine aggregates in SCA6 Purkinje cells

K. Ishikawa, K. Owada, K. Ishida, H. Fujigasaki, M. Shun Li, T. Tsunemi, N. Ohkoshi, S. Toru, T. Mizutani, M. Hayashi, N. Arai, K. Hasegawa, T. Kawanami, T. Kato, T. Makifuchi, S. Shoji, T. Tanabe, Hidehiro Mizusawa*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

70 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)1753-1756
Number of pages4
JournalNeurology
Volume56
Issue number12
DOIs
StatePublished - Jun 26 2001

ASJC Scopus subject areas

  • Clinical Neurology

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