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Detection and Quantification of Free Radicals in Peroxisomal Disorders:A Comparative Study withOxidative Stress Parameters

机译:过氧化物酶体紊乱中自由基的检测和定量:氧化应激参数的比较研究

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Introduction: Free radicals have been thought to participate in pathogenesis of peroxisomal disorders. Objective: The aim of the work is to detect free oxide radicals in blood of patients with peroxisomal disorders and to study their relation with various oxidative stress parameters. Materials and Methods: Twenty patients with peroxisomal disorders and 14 age and sex matched healthy subjects were included in the study. Patients with peroxisomal disorders were subdivided according to diagnosis into peroxisomal biogenesis disorders and single enzyme deficiency. Oxidative stress was evaluated in both patients and control subjects by assessment of free radicals, malondialdehyde, nitric oxide metabolites and superoxide dismutase. Results: There was increase in free radicals, malondialdehyde, nitric oxide metabolites in patients compared with control subjects. However, there was decrease in superoxide dismutase levels in patients compared with control subjects. Conclusion: We concluded that there is excess free radicals production accompanied with decrease in antioxidant defenses in patients with peroxisomal disorders. These results strongly support a role of free radicals in the pathophysiology of peroxisomal disorders and strengthen the importance of oxidative stress phenomenon in peroxisomal disorders pathogenesis.
机译:简介:人们认为自由基参与过氧化物酶体紊乱的发病机理。目的:这项工作的目的是检测过氧化物酶体异常患者血液中的自由基,并研究其与各种氧化应激参数的关系。材料和方法:20名过氧化物酶体紊乱患者和14位年龄和性别相匹配的健康受试者被纳入研究。根据诊断将过氧化物酶体疾病患者分为过氧化物酶体生物发生疾病和单一酶缺乏症。通过评估自由基,丙二醛,一氧化氮代谢产物和超氧化物歧化酶评估了患者和对照组的氧化应激。结果:与对照组相比,患者的自由基,丙二醛,一氧化氮代谢产物增加。但是,与对照组相比,患者的超氧化物歧化酶水平降低了。结论:我们得出结论,过氧化物酶体紊乱患者存在过多的自由基产生,同时抗氧化防御能力下降。这些结果有力地支持了自由基在过氧化物酶体疾病的病理生理中的作用,并增强了氧化应激现象在过氧化物酶体疾病的发病机理中的重要性。

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