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.
authors: Department of Neurology, (Graduate School of Medicine, Tokyo Medical and Dental University, Bunkyo-ku, Japan.
authors: K
authors: K
authors: Ishikawa
authors: K
authors: K
authors: Owada
authors: K
authors: K
authors: Ishida
authors: H
authors: H
authors: Fujigasaki
authors: M
authors: M
authors: Shun Li
authors: T
authors: T
authors: Tsunemi
authors: N
authors: N
authors: Ohkoshi
authors: S
authors: S
authors: Toru
authors: T
authors: T
authors: Mizutani
authors: M
authors: M
authors: Hayashi
authors: N
authors: N
authors: Arai
authors: K
authors: K
authors: Hasegawa
authors: T
authors: T
authors: Kawanami
authors: T
authors: T
authors: Kato
authors: T
authors: T
authors: Makifuchi
authors: S
authors: S
authors: Shoji
authors: T
authors: T
authors: Tanabe
authors: H
authors: H
authors: Mizusawa
mesh_terms: D001921:Brain / Q000378:metabolism; D003593:Cytoplasm / Q000378:metabolism*; D006801:Humans; D010455:Peptides / Q000032:analysis* / Q000235:genetics; D011689:Purkinje Cells / Q000378:metabolism*; D020754:Spinocerebellar Ataxias / Q000235:genetics / Q000378:metabolism*; D018911:Trinucleotide Repeats / Q000235:genetics
chemical_list: D010455:Peptides; C097188:polyglutamine