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首页> 外文期刊>Neurological Research: An Interdisciplinary Quarterly Journal >Oxidative stress and metal content in blood and cerebrospinal fluid of amyotrophic lateral sclerosis patients with and without a Cu, Zn-superoxide dismutase mutation.
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Oxidative stress and metal content in blood and cerebrospinal fluid of amyotrophic lateral sclerosis patients with and without a Cu, Zn-superoxide dismutase mutation.

机译：有和没有Cu，Zn-超氧化物歧化酶突变的肌萎缩性侧索硬化患者血液和脑脊液中的氧化应激和金属含量。

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摘要

Hydroxyl radical, ascorbate free radical, superoxide dismutase (SOD) activities, Cu,Zn-SOD protein, Mn-SOD protein, 8-hydroxy-2' -deoxyguanosine (8-OHdG) and metals were compared in red blood cells (RBC), plasma and/or cerebrospinal fluid (CSF) between patients with sporadic amyotrophic lateral sclerosis (SALS), familial ALS (FALS) showing the Leu126Ser mutation in the Cu, Zn-SOD gene and controls. In patients with FALS or SALS, concentrations of hydroxyl radical in blood and ascorbate free radical and 8-OHdG in CSF were higher than control group values, while SOD activities in RBC and CSF were lower. In contrast, Cu, Zn-SOD protein concentrations in RBC were low only in FALS patients. Concentrations of Cu in CSF of SALS patients were higher than in controls. Thus, the pathogenesis of increased oxidative stress differs between SALS patients and FALS patients with a mutant Leu126Ser SOD1 gene.
机译：在红细胞（RBC）中比较了羟基自由基，抗坏血酸自由基，超氧化物歧化酶（SOD）活性，Cu，Zn-SOD蛋白，Mn-SOD蛋白，8-羟基-2'-脱氧鸟苷（8-OHdG）和金属，散发性肌萎缩性侧索硬化症（SALS）和家族性ALS（FALS）患者之间的血浆和/或脑脊液（CSF）表现出Cu，Zn-SOD基因和对照组的Leu126Ser突变。在患有FALS或SALS的患者中，CSF中血液中的羟基自由基和抗坏血酸自由基的浓度以及8-OHdG均高于对照组，而RBC和CSF中的SOD活性较低。相反，仅在FALS患者中，RBC中的Cu，Zn-SOD蛋白浓度较低。 SALS患者CSF中的Cu浓度高于对照组。因此，SALS患者和具有突变Leu126Ser SOD1基因的FALS患者之间氧化应激增加的发病机制是不同的。

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