Laboratory of Experimental Neurobiology, IRCCS, Foundation Neurological Institute C. Mondino, Via Mondino, 2, 27100 Pavia, Italy.
Neurochem Int. 2010 Apr;56(5):687-93. doi: 10.1016/j.neuint.2010.02.004. Epub 2010 Feb 10.
Oxidative stress markers have been found in nervous and peripheral tissues of familial and sporadic amyotrophic lateral sclerosis patients. Here, we evaluated the activity of some antioxidant enzymes glutathione peroxidase, glutathione reductase and Cu-Zn superoxide dismutase in erythrocyte, the marker of non-enzymatic antioxidant response (total antioxidant status), as well as plasma reactive oxygen species, at the enrolment and during disease progression in 88 patients affected by the sporadic form of amyotrophic lateral sclerosis. Our study has been performed along 72 months by grouping the patients according to the ALS functional rating score or rate of disease progression. Our results showed a significant impairment of erythrocytes glutathione peroxidase activity in all groups of patients that remained low during disease time course. SOD1 activity significantly decreased along disease course in subjects with a more impaired functional status. A decreasing activity of all assayed enzymes was found in patients who have a faster disease progression rate. By this work we have the evidence that different ALS phenotypes present with different profile of enzymatic and non-enzymatic antioxidant response.
氧化应激标志物已在家族性和散发性肌萎缩侧索硬化症患者的神经和周围组织中被发现。在这里,我们评估了红细胞中一些抗氧化酶谷胱甘肽过氧化物酶、谷胱甘肽还原酶和 Cu-Zn 超氧化物歧化酶的活性、非酶抗氧化反应的标志物(总抗氧化状态),以及血浆活性氧,在 88 名患有散发性肌萎缩侧索硬化症的患者中入组时和疾病进展过程中。我们的研究在 72 个月内进行,根据肌萎缩侧索硬化症功能评分或疾病进展率对患者进行分组。我们的结果表明,所有患者组的红细胞谷胱甘肽过氧化物酶活性均显著受损,且在疾病过程中一直保持较低水平。SOD1 活性在功能状态受损更严重的患者中随疾病进程显著降低。在疾病进展速度较快的患者中,检测到所有酶活性均降低。通过这项工作,我们有证据表明,不同的肌萎缩侧索硬化症表型具有不同的酶和非酶抗氧化反应特征。