Aspartate‐taurine imbalance in dominantly inherited olivopontocerebellar atrophy

Abstract

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.

Regional and Synaptosomal Levels of Amino Acid Neurotransmitters in the 3‐Acetylpyridine Deafferentated Rat Cerebellum

“…The decreased content of aspartate observed after 3-AP treatment is most likely not due to a general metabolic effect since no decreases were observed in other regions of the CNS where 3-AP is known to be taken up (Herken, 1968). Our findings are consistent with those of Perry et al (1977). who reported that the content of aspartate was reduced in the cerebellar cortex and dentate nucleus of two patients who died with a neuropathological disorder in which there was a loss of cerebellar climbing fibers.…”

Benzodiazepine Receptor Binding in Cerebellar Cortex: Observations in Olivopontocerebellar Atrophy

“…The disorder has affected at least five successive generations (Currier et al, 1972). A neuropathological study of two of these patients (C1 and C2) revealed marked loss of Purkinje cells in cerebellar cortex (Perry et al, 1977).…”

“…42, 466-469 (1984). mark and Weiner, 1970;Landis et al, 1974;Perry et al, 1977;Colan et al, 1981;Berciano, 1982). In this regard, autoradiographic experiments using [3H]benzodiazepines have suggested the presence of benzodiazepine receptors on both human (Young and Kuhar, 1979) and rodeni (Palacios and Kuhar, 1982) Purkinje cell dendrites and somata.…”

Neurotransmitter glutamate: its clinical importance

“…An abnormal metaboIism of transmitter glutamate and aspartate may exist in the 4 different subtypes of OPCA so far surmised (148). In 3 of the 4 forms cerebellar cortical glutamate and aspartate levels decrease (148).…”

“…An abnormal metaboIism of transmitter glutamate and aspartate may exist in the 4 different subtypes of OPCA so far surmised (148). In 3 of the 4 forms cerebellar cortical glutamate and aspartate levels decrease (148). The close correlation between such changes in cerebellar glutamate and aspartate con-tents with morphological changes in the cerebellum and inferior olive of OPCA patients suggest that changes in the amino acid content of the tissue reflect changes in the transmitter pool of these amino acids (21).…”