通过测定戈谢病患者的总抗氧化能力和总氧化能力来研究氧化过程。

IF 1.2 4区 医学 Q3 PEDIATRICS
Aynur Küçükçongar Yavaş, Eylem Şerife Kalkan, Özlem Ünal Uzun, Mehmet Gunduz, Özcan Erel
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引用次数: 0

摘要

戈谢病是由编码β-葡萄糖脑苷酶的GBA1基因突变引起的。这种酶的缺乏导致糖基神经酰胺和糖基鞘氨苷在溶酶体内积累,并导致炎症,积累的底物影响多个器官系统,导致进行性细胞损伤。有证据表明,巨噬细胞中葡萄糖神经酰胺的积累与炎症过程、活性氧的产生以及促氧化剂和抗氧化剂储备之间的不平衡有关,从而导致氧化应激和炎症。本研究的目的是比较戈谢病患者和健康对照者的氧化应激状态。本研究纳入10例年龄在2-40岁之间的戈谢病患者和30例健康对照。比较各组总抗氧化能力和总抗氧化能力参数。虽然病例组总抗氧化能力值较高,但差异不显著。患者组总氧化能力和氧化应激指数值明显高于对照组(p
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Investigation of the Oxidative Process by Measuring Total Antioxidant Capacity and Total Oxidant Capacity in Patients with Gaucher Disease.

Gaucher disease is caused by mutations in the GBA1 gene, which encodes the β-glucocerebrosidase enzyme. The deficiency of this enzyme result in glucosylceramide and glucosylsphingosine accumulation, within lysosomes and resulted triggering inflammation and accumulating substrates affect multiple organ systems, causing progressive cellular damage. Evidence has shown that the accumulation of glucosylceramide in macrophages is associated with inflammatory processes, reactive oxygen species production and an imbalance between the pro-oxidants and the antioxidant reserve, resulting in oxidative stress and inflammation. The aim of this study was to compare the oxidative stress status between patients with Gaucher disease and healthy controls.Case-control study This study included 10 patients with Gaucher disease aged between 2-40 years, and 30 healthy controls. Total antioxidant capacity and total oxidant capacity parameters between these groups were compared. Although the total antioxidant capacity value of the case group was higher, it was not significant. The total oxidant capacity and oxidative stress index values were significantly higher in the patient group (p<0.05). Our study showed that oxidative stress increased in patients with Gaucher disease. The results of our study may guide the application of antioxidant treatment in Gaucher disease patients as a supportive treatment.

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来源期刊
Klinische Padiatrie
Klinische Padiatrie 医学-小儿科
CiteScore
1.10
自引率
0.00%
发文量
135
审稿时长
6-12 weeks
期刊介绍: Das Forum für wissenschaftliche Information in der Kinderheilkunde ausgewählte Originalarbeiten aus allen Bereichen der Pädiatrie Visite: Ihr Forum für interessante Krankengeschichten und außergewöhnliche Kasuistiken aktuelle Fortschritte in Diagnostik und Therapie jährliche Schwerpunkthefte: Ergebnisse der pädiatrischen Onkologie plus Medizin und Markt topaktuelle Informationen aus der Industrie
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