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[地方性肾病患者及其家族健康成员红细胞中δ-氨基乙酰丙酸脱水酶活性和谷胱甘肽水平]

[Activity of delta-aminolevulinate dehydratase and glutathione levels in the erythrocytes of patients with endemic nephropathy and in healthymembers of their families].

作者信息

Djordjević V, Pavlović D, Djordjević V, Stefanović V

出版信息

Srp Arh Celok Lek. 1990 Jan-Feb;118(1-2):47-50.

PMID:2218732
Abstract

In a previous paper a markedly decreased activity of delta-aminolevulinate dehydratase (ALA-D) in patients with endemic nephropathy and in 24.6% of their healthy family members, was found. In this study the possible mechanisms of the decreased enzyme activity and the level of erythrocyte glutathione in patients and in healthy members from nephropathic families, were studied. We established decreased ALA-D activity in endemic nephropathy patients and in some healthy persons from their families. The normal concentration of erythrocyte glutathione was found in all groups. However, it was possible to reactivate the ALA-D enzyme in vitro by addition of exogenous glutathione. The obtained results suggested a normal synthesis of ALA-D apoenzyme and the existence of some factors whose effect upon enzyme activity was mediated through a binding to the reactive groups in the active centre of his enzyme or by oxidation of he reactive groups. Therefore, increased concentrations of sulfhydryl compounds, mostly of reduced glutathione are necessary for an optimal ALA-D activity.

摘要

在之前的一篇论文中,发现地方性肾病患者及其24.6%的健康家庭成员体内的δ-氨基乙酰丙酸脱水酶(ALA-D)活性显著降低。在本研究中,对肾病患者及其患病家族中健康成员体内酶活性降低以及红细胞谷胱甘肽水平的可能机制进行了研究。我们确定了地方性肾病患者及其家族中一些健康人的ALA-D活性降低。所有组中红细胞谷胱甘肽浓度均正常。然而,通过添加外源性谷胱甘肽可在体外重新激活ALA-D酶。所得结果表明ALA-D脱辅酶合成正常,且存在一些因素,其对酶活性的影响是通过与该酶活性中心的反应基团结合或通过反应基团的氧化介导的。因此,为使ALA-D活性达到最佳,巯基化合物(主要是还原型谷胱甘肽)浓度的增加是必要的。

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