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NEUROLOGY 1977;27:257
© 1977 American Academy of Neurology

Aspartate-taurine imbalance in dominantly inherited olivopontocerebellar atrophy

THOMAS L. PERRY, M.D., ROBERT D. CURRIER, M.D., SHIRLEY HANSEN, B.A. and JANET MacLEAN, BSc.

From the Department of Pharmacology, University of British Columbia, Vancouver. Canada, and the Department of Medicine, Division of Neurology, University of Mississippi Medical Center, Jackson.

Amino acids were measured in autopsied brain from two patients who died with a dominantly inherited form of olivopontocerebellar atrophy. Neuropathologic changes found in the brain of these patients suggested a loss of cerebellar climbing fibers. The contents of aspartic acid, gamma-aminobutyric acid, and homocarnosine were reduced in the cerebellar cortex and the. dentate nucleus, while taurine content was markedly elevated in the same brain regions. These findings are compatible with the possibility that aspartic acid is the excitatory synaptic transmitter of the climbing fibers and taurine is the inhibitory neurotransmitter of one or more types of interneurons in the cerebellum.

Reprint requests should be directed to Dr. Perry at the Department of Pharmacology, University of British Columbia, Vancouver V6T 1W5, Canada.

Received for publication April 30, 1976.

Supported by grants from the Medical Research Council of Canada, the Huntington's Chorea Foundation, and the National Ataxia Foundation.




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