Glutamate dehydrogenase deficiency in spinocerebellar degenerations

descriptionPublicationkeyboard_double_arrow_right Article 01 May 1982 English Publisher:Springer Science and Business Media LLCJournal:Neurochemical Research, volume 7, pages 627-636 (issn: 0364-3190, eissn: 1573-6903,

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Authors: T, Yamaguchi; K, Hayashi; H, Murakami; K, Ota; S, Maruyama;

doi: 10.1007/bf00965128

pmid: 6811963

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.

Keywords

Adult, Blood Platelets, Cerebellar Ataxia, Middle Aged, Olivary Nucleus, Spinal Cord Diseases, Glutamate Dehydrogenase, Cerebellar Diseases, Cerebellum, Pons, Leukocytes, Humans, Atrophy, Pyruvate Dehydrogenase Complex Deficiency Disease

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