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肌萎缩侧索硬化症患者脑脊液中铜/锌超氧化物歧化酶(SOD1)水平的性别差异。

Gender difference in levels of Cu/Zn superoxide dismutase (SOD1) in cerebrospinal fluid of patients with amyotrophic lateral sclerosis.

作者信息

Frutiger Katrin, Lukas Thomas J, Gorrie George, Ajroud-Driss Senda, Siddique Teepu

机构信息

Davee Department of Neurology and Clinical Neurosciences, Northwestern University Feinberg School of Medicine, Chicago, Illinois 60611, USA.

出版信息

Amyotroph Lateral Scler. 2008 Jun;9(3):184-7. doi: 10.1080/17482960801984358.

Abstract

Currently the best studied mechanism for amyotrophic lateral sclerosis (ALS) is the one caused by mutations in the gene for cytosolic Cu/Zn-binding superoxide dismutase (SOD1). Mutant SOD1 protein causes motor neuron degeneration due to the gain of a novel toxic function. To evaluate the relevance of SOD1 levels in cerebrospinal fluid (CSF) in ALS patients, the SOD1 concentration was immunoassayed in the CSF of 11 patients with ALS and 19 neurological controls. The mean level of SOD1 in CSF from all samples was 45.5+/-11.3 ng/ml. There was no statistically significant difference between the levels of SOD1 in CSF of ALS patients and neurological control subjects. Here we show that the SOD1 concentration in the CSF is significantly higher in male ALS patients (54.0+/-9.0 ng/ml) compared to female ALS patients (38.1+/-6.4 ng/ml) (p=0.007). This gender difference is not observed in the CSF of neurological controls. This is the first report of a potential gender difference in levels of SOD1 in CSF of ALS patients. Further investigation of larger sample groups is needed to determine whether it is relevant to gender related differences in disease incidence.

摘要

目前,对肌萎缩侧索硬化症(ALS)研究得最为透彻的机制是由胞质铜/锌结合超氧化物歧化酶(SOD1)基因突变所引发的机制。突变的SOD1蛋白由于获得了一种新的毒性功能而导致运动神经元退化。为了评估ALS患者脑脊液(CSF)中SOD1水平的相关性,对11例ALS患者和19例神经学对照者的脑脊液进行了SOD1浓度免疫测定。所有样本脑脊液中SOD1的平均水平为45.5±11.3 ng/ml。ALS患者脑脊液中SOD1水平与神经学对照者之间无统计学显著差异。在此我们表明,男性ALS患者脑脊液中SOD1浓度(54.0±9.0 ng/ml)显著高于女性ALS患者(38.1±6.4 ng/ml)(p = 0.007)。在神经学对照者的脑脊液中未观察到这种性别差异。这是关于ALS患者脑脊液中SOD1水平存在潜在性别差异的首次报道。需要对更大样本组进行进一步研究,以确定其是否与疾病发病率的性别相关差异有关。

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