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Neurotransmitter amino acids in dominantly inherited cerebellar disorders

From the Department of Pharmacology (Drs. Perry and Kish and Ms. Hansen), University of British Columbia, Vancouver, Canada, and the Department of Neurology (Dr. Currier), University of Mississippi Medical Center, Jackson, MS.

We measured amino acid contents in the brains of 11 patients with dominantly inherited cerebellar disorders. Despite clinical similarities, three biochemically different disorders were found. One disorder, with demonstrated HLA linkage in one pedigree, was characterized by moderate reduction of aspartate and glutamate contents in cerebellar cortex alone. In a second disorder, aspartate and glutamate contents were reduced markedly in other brain areas as well as in cerebellar cortex. Aspartate and glutamate contents were normal in cerebellar cortex in the third disorder. GABA content in cerebellar cortex and dentate nucleus was reduced in some patients with each disorder, whereas cerebellar taurine content was normal in all patients. Aspartate deficiency in cerebellar cortex did not result from lack of aspartate aminotransferase or pyruvate carboxylase activity. These amino acid abnormalities probably imply loss of specific cerebellar neurons.

Address correspondence and reprint requests to Dr. Perry, Department of Pharmacology, University of British Columbia, Vancouver, BC, Canada.

This study was supported by the Medical Research Council of Canada.

Accepted for publication June 10, 1980.

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