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

doi:10.1212/WNL.56.12.1753

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 journal › Article › peer-review

79 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 language	English (US)
Pages (from-to)	1753-1756
Number of pages	4
Journal	Neurology
Volume	56
Issue number	12
DOIs	https://doi.org/10.1212/WNL.56.12.1753
State	Published - Jun 26 2001
Externally published	Yes

ASJC Scopus subject areas

Clinical Neurology

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Ishikawa, K., Owada, K., Ishida, K., Fujigasaki, H., Shun Li, M., Tsunemi, T., Ohkoshi, N., Toru, S., Mizutani, T., Hayashi, M., Arai, N., Hasegawa, K., Kawanami, T., Kato, T., Makifuchi, T., Shoji, S., Tanabe, T., & Mizusawa, H. (2001). Cytoplasmic and nuclear polyglutamine aggregates in SCA6 Purkinje cells. Neurology, 56(12), 1753-1756. https://doi.org/10.1212/WNL.56.12.1753

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title = "Cytoplasmic and nuclear polyglutamine aggregates in SCA6 Purkinje cells",

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.",

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

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Ishikawa, K, Owada, K, Ishida, K, Fujigasaki, H, Shun Li, M, Tsunemi, T, Ohkoshi, N, Toru, S, Mizutani, T, Hayashi, M, Arai, N, Hasegawa, K, Kawanami, T, Kato, T, Makifuchi, T, Shoji, S, Tanabe, T & Mizusawa, H 2001, 'Cytoplasmic and nuclear polyglutamine aggregates in SCA6 Purkinje cells', Neurology, vol. 56, no. 12, pp. 1753-1756. https://doi.org/10.1212/WNL.56.12.1753

TY - JOUR

T1 - Cytoplasmic and nuclear polyglutamine aggregates in SCA6 Purkinje cells

AU - Ishikawa, K.

AU - Owada, K.

AU - Ishida, K.

AU - Fujigasaki, H.

AU - Shun Li, M.

AU - Tsunemi, T.

AU - Ohkoshi, N.

AU - Toru, S.

AU - Mizutani, T.

AU - Hayashi, M.

AU - Arai, N.

AU - Hasegawa, K.

AU - Kawanami, T.

AU - Kato, T.

AU - Makifuchi, T.

AU - Shoji, S.

AU - Tanabe, T.

AU - Mizusawa, Hidehiro

PY - 2001/6/26

Y1 - 2001/6/26

N2 - 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.

AB - 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.

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Cytoplasmic and nuclear polyglutamine aggregates in SCA6 Purkinje cells

Abstract

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