Neurology 2001;56:1753-1756
© 2001 American Academy of Neurology
Brief Communications
Cytoplasmic and nuclear polyglutamine aggregates in SCA6 Purkinje cells
K. Ishikawa, MD, PhD;,
K. Owada, MD, PhD;,
K. Ishida, MD, PhD;,
H. Fujigasaki, MD, PhD;,
M. Shun Li, MD;,
T. Tsunemi, MD;,
N. Ohkoshi, MD, PhD;,
S. Toru, MD, PhD;,
T. Mizutani, MD, PhD;,
M. Hayashi, MD, PhD;,
N. Arai, MD, PhD;,
K. Hasegawa, MD, PhD;,
T. Kawanami, MD, PhD;,
T. Kato, MD, PhD;,
T. Makifuchi, MD, PhD;,
S. Shoji, MD, PhD;,
T. Tanabe, PhD; and
H. Mizusawa, MD, PhD
From the Departments of Neurology (Drs. Ishikawa, Owada, Ishida, Fujigasaki, Li, Tsunemi, Toru, and Mizusawa) and Pharmacology (Dr. Tanabe), Graduate School of Medicine, Tokyo Medical and Dental University, Bunkyo-ku; CREST (Drs. Ishikawa, Toru, Tanabe, and Mizusawa), Japan Science and Technology Corporation, Wako, Saitama; Department of Neurology (Dr. Ishida), Tamagawa Hospital, Setagaya-ku, Tokyo; Departments of Neurology (Dr. Fujigasaki) and Pathology (Dr. Mizutani), Tokyo Metropolitan Neurological Hospital, Fuchu; Department of Neurology (Drs. Ohkoshi and Shoji), Institute of Clinical Medicine, University of Tsukuba, Ibaraki; Department of Neuropathology (Drs. Hayashi and Arai), Tokyo Metropolitan Institute of Neurological Sciences, Fuchu; Department of Neurology (Dr. Hasegawa), Kitasato University, Sagamihara, Kanagawa; Third Department of Internal Medicine (Drs. Kawanami and Kato), Yamagata University School of Medicine; and the Department of Neuropathology (Dr. Makifuchi), National Saigata Hospital, Niigata, Japan.
Address correspondence and reprint requests to Dr. Hidehiro Mizusawa, Professor and Chairman, Department of Neurology, Graduate School of Medicine, Tokyo Medical and Dental University, Yushima 1-chome 5-45, Bunkyo-ku 113-8519, Tokyo, Japan; e-mail: h-mizusawa.nuro{at}tmd.ac.jp
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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