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

G Tórsdóttir, J Kristinsson, G Gudmundsson, J Snaedal, T Jóhannesson
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引用次数: 22

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.

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肌萎缩性侧索硬化症中的铜、铜蓝蛋白和超氧化物歧化酶(SOD)。
在之前的两项研究中,我们在阿尔茨海默病患者和帕金森病患者中发现了铜平衡失调。在这项研究中,测定了14例肌萎缩性侧索硬化症患者及其年龄和性别匹配的健康对照者血浆铜水平、血清铜蓝蛋白水平、铜蓝蛋白氧化活性、铜蓝蛋白特异性氧化活性(与质量相关的活性)以及红细胞超氧化物歧化酶(SOD)活性。其中三名患者患有家族性疾病或被怀疑患有这种疾病。发现所有参数的平均值在患者和对照组之间没有显著差异(学生t检验;P > 0.05)。通过方差相等性检验(F分布),我们发现患者组与对照组之间铜蓝蛋白特异性氧化活性和SOD活性的个体结果方差差异显著(P= 0.021和P=0.003),但两者的个体结果不相关(P>0.05)。我们得出结论,铜蓝蛋白特异性氧化活性和SOD活性的紊乱可能导致肌萎缩性侧索硬化症的运动神经元死亡,由于这两种酶的活性不相关,因此不确定哪一种与疾病的病理关系更密切。
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