Abnormal glutamate metabolism in an adult-onset degenerative neurological disorder.
Abnormal glutamate metabolism in an adult-onset degenerative neurological disorder.
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DOI：
10.1126/science.6121377
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发表时间：
1982-04
期刊：
影响因子：
56.9
通讯作者：
A. Plaitakis;S. Berl;Yahr
中科院分区：
文献类型：
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作者：
A. Plaitakis;S. Berl;Yahr
与谷氨酸脱氢酶部分缺乏相关的成人隐性橄榄桥小脑变性患者，血浆中谷氨酸的浓度明显高于对照组。血浆α -酮戊二酸显著降低。在谷氨酸脱氢酶缺乏的患者中，口服谷氨酸钠导致血浆中这种氨基酸的过度积累，血浆乳酸与丙酮酸的比例没有增加。谷氨酸分解代谢减少可导致神经系统谷氨酸过量，引起神经元变性。
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 alpha-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.