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Huntington's Chorea: Deficiency of γ-Aminobutyric Acid in Brain
Abstract
Amino acids and related compounds were measured in various regions of brain obtained at autopsy from eight neurologically normal persons, one patient with Parkinson's disease, and eight patients with Huntington's chorea. γ-Aminobutyric acid (GABA) and homocarnosine, a related dipeptide, were reduced in the substantia nlgra, putamen-globus-pallidus, and caudate nucleus of the choreic patients, as compared to the normal subjects and the patient with Parkinsonism. Glycerophosphoethanolamine concentration was elevated in these three regions of choreic brain. Deficiency of γ-aminobutyric acid, a possible inhibitory synaptic transmitter in brain, might produce the symptoms characteristic of Huntington's chorea. Such a deficiency could result from reduced activity of the enzyme glutamic acid decarboxylase, or might reflect a decrease in the number of neurons that normally utilize γ-aminobutyric acid as a transmitter.
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Cited by
The Dopamine Hypothesis of Schizophrenia: A Review*
Meltzer, H. Y., & Stahl, S. M. (1976) · Schizophrenia Bulletin · doi:10.1093/schbul/2.1.19 · Cited by 883
Swerdlow, N. R., & Koob, G. F. (1987) · Behavioral and Brain Sciences · doi:10.1017/s0140525x00047488 · Cited by 674
Rajkowska, G., Selemon, L. D., & Goldman-Rakic, P. S. (1998) · Archives of General Psychiatry · doi:10.1001/archpsyc.55.3.215 · Cited by 537
Psychiatric syndromes in Huntington’s disease.
(1983) · American Journal of Psychiatry · doi:10.1176/ajp.140.6.728 · Cited by 255
Striatal D1 and D2 dopamine receptor loss in asymptomatic mutation carriers of Huntington's disease
Weeks, R. A., Piccini, P., Harding, A. E., & Brooks, D. J. (1996) · Annals of Neurology · doi:10.1002/ana.410400110 · Cited by 166
Metadata
- Authors
- Perry, T. L., Hansen, S., & Kloster, M.
- Venue
- New England Journal of Medicine
- Publisher
- Massachusetts Medical Society
- Published
- 1973-02-15
- Volume
- 288
- Issue
- 7
- Pages
- 337-342
- ISSN
- 0028-4793 (ISSN-L)
- 1533-4406
- OpenAlex
- W2087147633
- Cited by
- 833
- References
- 0
- FWCI
- 16.20
- Type
- journal article
- Language
- en
- Open access
- closed
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- Perry, T. L., Hansen, S., & Kloster, M. (1973). Huntington's Chorea: Deficiency of γ-Aminobutyric Acid in Brain. New England Journal of Medicine. https://doi.org/10.1056/nejm197302152880703
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