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肌萎缩侧索硬化症中的铜、铜蓝蛋白和超氧化物歧化酶(SOD)

Copper, ceruloplasmin and superoxide dismutase (SOD) in amyotrophic lateral sclerosis.

作者信息

Tórsdóttir G, Kristinsson J, Gudmundsson G, Snaedal J, Jóhannesson T

机构信息

Department of Pharmacology and Toxicology, The Institute of Pharmacy, Pharmacology and Toxicology, University of Iceland, Reykjavik.

出版信息

Pharmacol Toxicol. 2000 Sep;87(3):126-30. doi: 10.1111/j.0901-9928.2000.870305.x.

Abstract

In two previous studies we found copper dyshomeostasis in patients with Alzheimer's disease and in patients with Parkinson's disease. In this study, the levels of copper in plasma, of ceruloplasmin in serum, ceruloplasmin oxidative activity, ceruloplasmin specific oxidative activity (activity related to mass) as well as superoxide dismutase (SOD) activity in erythrocytes have been determined in 14 patients with amyotrophic lateral sclerosis and their healthy age- and gender-matched controls. Three of the patients had a familial form of the disease or were suspected of having it. The mean values of all parameters were found not to differ significantly between the patients and their controls (Student's t-test; P>0.05). By testing the equality of variances (F distribution) we found that the variances of individual results for ceruloplasmin specific oxidative activity and SOD activity differed significantly between the patients group and the controls group (P= 0.021 and P=0.003), but the individual results of these two activities were not correlated (P>0.05). We conclude that disturbances in ceruloplasmin specific oxidative activity and SOD activity could contribute to motor neurone death in amyotrophic lateral sclerosis, and since the two enzyme activities are not correlated it is uncertain which one is more closely related to the pathology of the disease.

摘要

在之前的两项研究中,我们发现阿尔茨海默病患者和帕金森病患者存在铜稳态失衡。在本研究中,我们测定了14例肌萎缩侧索硬化症患者及其年龄和性别匹配的健康对照者血浆中的铜水平、血清中的铜蓝蛋白水平、铜蓝蛋白氧化活性、铜蓝蛋白比氧化活性(与质量相关的活性)以及红细胞中的超氧化物歧化酶(SOD)活性。其中3例患者患有家族性该疾病或疑似患有该疾病。通过学生t检验发现,患者组和对照组所有参数的平均值无显著差异(P>0.05)。通过检验方差齐性(F分布),我们发现患者组和对照组铜蓝蛋白比氧化活性和SOD活性的个体结果方差存在显著差异(P = 0.021和P = 0.003),但这两种活性的个体结果不相关(P>0.05)。我们得出结论,铜蓝蛋白比氧化活性和SOD活性的紊乱可能导致肌萎缩侧索硬化症中的运动神经元死亡,并且由于这两种酶活性不相关,因此不确定哪一种与疾病病理更密切相关。

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