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Glutamate dehydrogenase deficiency in spinocerebellar degenerations

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Abstract

Glutamate dehydrogenase (GDH) activity in leukocytes and platelets in spinocerebellar degenerations (SCD) was determined. In the same subject, GDH activity was higher and more reproducible in platelets than in leukocytes. GDH was decreased significantly in olivopontocerebellar atrophy (OPCA) (Ca. 30% decrease). Pyruvate dehydrogenase (PDH) in platelets showed non specific decreased activity in SCD and amyotropic lateral sclerosis. Energy metabolism in cerebellum may be diminished in some types of ataxia, and glutaminergic neurons may be more affected in OPCA than in other SCD.

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Authors and Affiliations

  1. Department of Biochemistry, Tokyo Women's Medical College, 10-Kawada-cho Shinjuku-ku, Tokyo, Japan

    Tomoko Yamaguchi & Keirei Hayashi

  2. Department of Neurology, Tokyo Women's Medical College, 10-Kawada-cho Shinjuku-ku, Tokyo, Japan

    Hirohiko Murakami, Kohei Ota & Shoichi Maruyama

Authors
  1. Tomoko Yamaguchi
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  2. Keirei Hayashi
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  3. Hirohiko Murakami
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  4. Kohei Ota
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  5. Shoichi Maruyama
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Yamaguchi, T., Hayashi, K., Murakami, H. et al. Glutamate dehydrogenase deficiency in spinocerebellar degenerations. Neurochem Res 7, 627–636 (1982). https://doi.org/10.1007/BF00965128

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