Cytoplasmic and nuclear polyglutamine aggregates in SCA6 Purkinje cells.

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

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.

Afiliación

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

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

Article en En | MEDLINE | ID: mdl-11425948

RESUMEN

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.

Asunto(s)

Citoplasma/metabolismo; Péptidos/análisis; Células de Purkinje/metabolismo; Ataxias Espinocerebelosas/metabolismo; Encéfalo/metabolismo; Humanos; Péptidos/genética; Ataxias Espinocerebelosas/genética; Repeticiones de Trinucleótidos/genética

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Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Péptidos / Células de Purkinje / Ataxias Espinocerebelosas / Citoplasma Límite: Humans Idioma: En Revista: Neurology Año: 2001 Tipo del documento: Article País de afiliación: Japón Pais de publicación: Estados Unidos

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