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脊髓小脑变性中的谷氨酸脱氢酶缺乏症。

Glutamate dehydrogenase deficiency in spinocerebellar degenerations.

作者信息

Yamaguchi T, Hayashi K, Murakami H, Ota K, Maruyama S

出版信息

Neurochem Res. 1982 May;7(5):627-36. doi: 10.1007/BF00965128.

DOI:10.1007/BF00965128
PMID:6811963
Abstract

Glutamate dehydrogenase (GDH) activity in leukocytes and platelets in spinocerebellar degenerations (SCD) was determined. In the same subject, GDH activity was higher and more reproducible in platelets than in leukocytes. GDH was decreased significantly in olivopontocerebellar atrophy (OPCA) (Ca. 30% decrease). Pyruvate dehydrogenase (PDH) in platelets showed non specific decreased activity in SCD and amyotropic lateral sclerosis. Energy metabolism in cerebellum may be diminished in some types of ataxia, and glutaminergic neurons may be more affected in OPCA than in other SCD.

摘要

测定了脊髓小脑变性(SCD)患者白细胞和血小板中的谷氨酸脱氢酶(GDH)活性。在同一受试者中,血小板中的GDH活性高于白细胞,且更具重复性。橄榄桥脑小脑萎缩(OPCA)患者的GDH活性显著降低(约降低30%)。血小板中的丙酮酸脱氢酶(PDH)在SCD和肌萎缩侧索硬化症中表现出非特异性的活性降低。在某些类型的共济失调中,小脑的能量代谢可能会减弱,并且OPCA中的谷氨酰胺能神经元可能比其他SCD更易受影响。

相似文献

1
Glutamate dehydrogenase deficiency in spinocerebellar degenerations.脊髓小脑变性中的谷氨酸脱氢酶缺乏症。
Neurochem Res. 1982 May;7(5):627-36. doi: 10.1007/BF00965128.
2
Glutamate and pyruvate dehydrogenase deficiency in spinocerebellar degeneration.脊髓小脑变性中的谷氨酸和丙酮酸脱氢酶缺乏症。
Adv Neurol. 1984;41:255-65.
3
Oral glutamate loading in disorders with spinocerebellar and extrapyramidal involvement: effect on plasma glutamate, aspartate and taurine.在伴有脊髓小脑和锥体外系受累的疾病中口服谷氨酸负荷:对血浆谷氨酸、天冬氨酸和牛磺酸的影响
J Neural Transm Suppl. 1983;19:65-74.
4
Abnormal platelet glutamate dehydrogenase activity and activation in dominant and nondominant olivopontocerebellar atrophy.在显性和隐性橄榄体脑桥小脑萎缩中血小板谷氨酸脱氢酶活性异常及激活
Ann Neurol. 1986 Mar;19(3):239-45. doi: 10.1002/ana.410190304.
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Glutamate dehydrogenase deficiency in patients with olivopontocerebellar atrophy.橄榄体脑桥小脑萎缩患者的谷氨酸脱氢酶缺乏症
Neurology. 1983 Oct;33(10):1322-6. doi: 10.1212/wnl.33.10.1332.
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Glutamate dehydrogenase deficiency in three patients with spinocerebellar syndrome.三名脊髓小脑综合征患者的谷氨酸脱氢酶缺乏症
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Pathology of olivopontocerebellar atrophy with glutamate dehydrogenase deficiency.伴有谷氨酸脱氢酶缺乏的橄榄体脑桥小脑萎缩的病理学
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Glutamate dehydrogenase and its isozyme activity in olivopontocerebellar atrophy.橄榄体脑桥小脑萎缩中的谷氨酸脱氢酶及其同工酶活性
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Multiple system degeneration with glutamate dehydrogenase deficiency: pathology and biochemistry.伴有谷氨酸脱氢酶缺乏的多系统变性:病理学与生物化学
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The Markers of Glutamate Metabolism in Peripheral Blood Mononuclear Cells and Neurological Complications in Lung Cancer Patients.肺癌患者外周血单个核细胞中谷氨酸代谢标志物与神经并发症
Dis Markers. 2016;2016:2895972. doi: 10.1155/2016/2895972. Epub 2016 Dec 4.
2
Distribution and characterization of the glutamate receptors in the CNS of ataxic mutant mouse.共济失调突变小鼠中枢神经系统中谷氨酸受体的分布与特征
Neurochem Res. 1984 Apr;9(4):497-505. doi: 10.1007/BF00964376.
3
Low leukocyte glutamate dehydrogenase activity does not correlate with a particular type of multiple system atrophy.

本文引用的文献

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Glutamate dehydrogenase deficiency in three patients with spinocerebellar syndrome.三名脊髓小脑综合征患者的谷氨酸脱氢酶缺乏症
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Multiple system degeneration with glutamate dehydrogenase deficiency: pathology and biochemistry.伴有谷氨酸脱氢酶缺乏的多系统变性:病理学与生物化学
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Regulation of glutamate dehydrogenase by histidine.组氨酸对谷氨酸脱氢酶的调节作用。
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Inhibition of glutamate dehydrogenase by bilirubin.胆红素对谷氨酸脱氢酶的抑制作用。
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A defect in pyruvate decarboxylase in a child with an intermittent movement disorder.一名患有间歇性运动障碍儿童的丙酮酸脱羧酶缺陷。
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Conformational changes and the regulation of glutamate-dehydrogenase activity.构象变化与谷氨酸脱氢酶活性的调节
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Contents of several amino acids in the cerebellum, brain stem and cerebrum of the 'staggerer', 'weaver' and 'nervous' neurologically mutant mice.“蹒跚者”“编织者”和“神经质”神经突变小鼠的小脑、脑干和大脑中几种氨基酸的含量。
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