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Cerebellar glutamate metabolizing enzymes in spinocerebellar ataxia type I

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Abstract

We measured the levels of three glutamate metabolizing enzymes, namely, glutamate dehydrogenase (GDH), aspartate aminotransferase (AAT), and glutamine synthetase (GS) in cerebellar and occipital cortices of nine patients with dominantly-inherited olivopontocerebellar atrophy (OPCA; spinocerebellar ataxia type I). As compared with the controls, mean GDH activities in cerebellar cortex of the OPCA patients were normal whereas levels of AAT (−17%) and the glial enzyme GS (−27%) were significantly reduced. No statistically significant changes were observed in occipital cortex, a morphologically unaffected brain area. We suggest that the decreased GS levels could reflect impaired capacity of astrocytes to metabolize glutamate which might contribute to the degenerative processes in OPCA cerebellum.

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Authors and Affiliations

  1. Human Neurochemical Pathology Lab, Clarke Institute of Psychiatry, 250 College St., M5T 1R8, Toronto, Ontario, Canada

    Stephen J. Kish, Li-Jan Chang, Lori M. Dixon & Linda DiStefano

  2. Department of Pathology, University of Montreal, Quebec, Canada

    Yves Robitaille

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  1. Stephen J. Kish
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  2. Li-Jan Chang
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  3. Lori M. Dixon
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  4. Yves Robitaille
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  5. Linda DiStefano
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Kish, S.J., Chang, LJ., Dixon, L.M. et al. Cerebellar glutamate metabolizing enzymes in spinocerebellar ataxia type I. Metab Brain Dis 9, 97–103 (1994). https://doi.org/10.1007/BF01996077

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  • DOI: https://doi.org/10.1007/BF01996077

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