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Abnormal Glutamate Metabolism in an Adult-Onset Degenerative Neurological Disorder
Abstract
In patients with recessive, adult-onset olivopontocerebellar degeneration associated with a partial deficiency of glutamate dehydrogenase, the concentration of glutamate in plasma was significantly higher than that in controls. Plasma α-ketoglutarate was significantly lower. Oral administration of monosodium glutamate resulted in excessive accumulation of this amino acid in plasma and lack of increase in the ratio of plasma lactate to pyruvate in the glutamate dehydrogenase-deficient patients. Decreased glutamate catabolism may result in an excess of glutamate in the nervous system and cause neuronal degeneration.
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Citations
Cited by
Pharmacology of glutamate neurotoxicity in cortical cell culture: attenuation by NMDA antagonists
Choi, D., Koh, J., & Peters, S. (1988) · The Journal of Neuroscience · doi:10.1523/jneurosci.08-01-00185.1988 · Cited by 986
Beal, M. F. (1992) · Annals of Neurology · doi:10.1002/ana.410310202 · Cited by 961
The neurotoxicity of excitatory amino acids is produced by passive chloride influx
Rothman, S. (1985) · The Journal of Neuroscience · doi:10.1523/jneurosci.05-06-01483.1985 · Cited by 631
Historical Overview: Calcium, Ischemia, and Death of Brain Cellsa
SIESJÖ, B. K. (1988) · Annals of the New York Academy of Sciences · doi:10.1111/j.1749-6632.1988.tb33410.x · Cited by 422
Abnormal glutamate metabolism in amyotrophic lateral sclerosis
Plaitakis, A., & Caroscio, J. T. (1987) · Annals of Neurology · doi:10.1002/ana.410220503 · Cited by 305
Self-citation
Metadata
- Authors
- Plaitakis, A., Berl, S., & Yahr, M. D.
- Venue
- Science
- Published
- 1982-04-09
- Volume
- 216
- Issue
- 4542
- Pages
- 193-196
- ISSN
- 0036-8075 (ISSN-L)
- 1095-9203
- OpenAlex
- W2023552997
- Cited by
- 225
- References
- 0
- FWCI
- 13.83
- Type
- journal article
- Language
- en
- Open access
- closed
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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.
