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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.

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。

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