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1
Decreased glutamate dehydrogenase protein in spinocerebellar degeneration.脊髓小脑变性中谷氨酸脱氢酶蛋白减少。
J Neurol Neurosurg Psychiatry. 1988 Aug;51(8):1078-80. doi: 10.1136/jnnp.51.8.1078.
2
Low leukocyte glutamate dehydrogenase activity does not correlate with a particular type of multiple system atrophy.低白细胞谷氨酸脱氢酶活性与特定类型的多系统萎缩无关。
J Neurol Neurosurg Psychiatry. 1988 Dec;51(12):1508-11. doi: 10.1136/jnnp.51.12.1508.
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Leukocyte glutamate dehydrogenase and CSF amino acids in late onset ataxias.迟发性共济失调中的白细胞谷氨酸脱氢酶和脑脊液氨基酸
Acta Neurol Scand. 1990 Oct;82(4):225-9. doi: 10.1111/j.1600-0404.1990.tb01610.x.
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Leukocyte glutamate dehydrogenase activity in patients with degenerative neurological disorders.退行性神经疾病患者的白细胞谷氨酸脱氢酶活性
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Glutamate dehydrogenase deficiency in Machado-Joseph disease.马查多-约瑟夫病中的谷氨酸脱氢酶缺乏症。
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Degenerative neurological disorders associated with deficiency of glutamate dehydrogenase.与谷氨酸脱氢酶缺乏相关的退行性神经疾病
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Partially deficient glutamate dehydrogenase activity and attenuated oscillatory potentials in patients with spinocerebellar degeneration.脊髓小脑变性患者谷氨酸脱氢酶活性部分缺乏及振荡电位减弱。
Invest Ophthalmol Vis Sci. 1992 Feb;33(2):447-52.
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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.
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Glutamate dehydrogenase deficiency in cerebellar degenerations: clinical, biochemical and molecular genetic aspects.小脑变性中的谷氨酸脱氢酶缺乏症:临床、生化及分子遗传学方面
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Glutamate dehydrogenase in OPCA.橄榄体脑桥小脑萎缩中的谷氨酸脱氢酶
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引用本文的文献

1
Normal cerebellar glutamate dehydrogenase protein in spinocerebellar degeneration.脊髓小脑变性中正常的小脑谷氨酸脱氢酶蛋白
J Neurol Neurosurg Psychiatry. 1989 May;52(5):666-8. doi: 10.1136/jnnp.52.5.666.
2
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
J Neurol Neurosurg Psychiatry. 1990 Dec;53(12):1099-101. doi: 10.1136/jnnp.53.12.1099.

本文引用的文献

1
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
Ann Neurol. 1980 Apr;7(4):297-303. doi: 10.1002/ana.410070403.
2
Neurological disorders associated with deficiency of glutamate dehydrogenase.与谷氨酸脱氢酶缺乏相关的神经障碍
Ann Neurol. 1984 Feb;15(2):144-53. doi: 10.1002/ana.410150206.
3
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.
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.
5
Glutamate dehydrogenase in olivopontocerebellar atrophies: leukocytes, fibroblasts, and muscle mitochondria.橄榄体脑桥小脑萎缩中的谷氨酸脱氢酶:白细胞、成纤维细胞和肌肉线粒体。
Neurology. 1986 Apr;36(4):550-3. doi: 10.1212/wnl.36.4.550.

脊髓小脑变性中谷氨酸脱氢酶蛋白减少。

Decreased glutamate dehydrogenase protein in spinocerebellar degeneration.

作者信息

Kajiyama K, Ueno S, Tatsumi T, Yorifuji S, Takahashi M, Tarui S

机构信息

Second Department of Internal Medicine, Osaka University, Medical School, Japan.

出版信息

J Neurol Neurosurg Psychiatry. 1988 Aug;51(8):1078-80. doi: 10.1136/jnnp.51.8.1078.

DOI:10.1136/jnnp.51.8.1078
PMID:3216208
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC1033118/
Abstract

A radioimmunoassay system for determining content of glutamate dehydrogenase (GDH) in human leukocytes was established and studied in 14 patients with spinocerebellar ataxia or atypical Parkinsonism. The protein content of leukocyte GDH was decreased in four patients and the reduction in the protein content was proportional to that in the enzyme activity. The ratio of GDH activity to protein content was invariable in healthy controls, diseased controls and patients with reduced GDH activity. These results suggested that at least a portion of the partial GDH deficiency was due to the decreased level of the enzyme protein.

摘要

建立了一种用于测定人白细胞中谷氨酸脱氢酶(GDH)含量的放射免疫分析系统,并对14例脊髓小脑共济失调或非典型帕金森病患者进行了研究。4例患者白细胞GDH的蛋白质含量降低,且蛋白质含量的降低与酶活性的降低成正比。在健康对照、疾病对照和GDH活性降低的患者中,GDH活性与蛋白质含量的比值不变。这些结果表明,至少部分GDH部分缺乏是由于酶蛋白水平降低所致。