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[携带线粒体DNA G11778A突变的Leber遗传性视神经病变患者血清超氧化物歧化酶和丙二醛水平的评估]

[Evaluation of serum levels of SOD and MDA in patients with Leber's hereditary optic neuropathy carrying the mitochondrial DNA G11778A mutation].

作者信息

Liu Zhe, Sun Chuan-bin, Tong Yi, Qu Jia

机构信息

School of Medicine, Zhejiang University, Hangzhou 310009, China.

出版信息

Zhonghua Yan Ke Za Zhi. 2009 Aug;45(8):719-23.

Abstract

OBJECTIVE

To determine the serum levels of total superoxide dismutase (SOD) activity and malondialdehyde (MDA), and to evaluate the oxidant-antioxidant status in patients with Leber's hereditary optic neuropathy (LHON) carrying the mitochondrial G11778A mutation.

METHODS

Nineteen patients and 12 carriers from three Chinese G11778A LHON families were enrolled in this study, and 30 age-matched healthy volunteers were recruited as normal controls. The serum levels of total SOD activity and MDA in all subjects were measured by xanthine oxidase test and thiobarbituric acid technique, respectively.

RESULTS

The serum level of total SOD activity in LHON patients was significantly less than that in carriers and normal controls (q = 7.085 and 8.351, respectively, both P < 0.05), however, there was no significant difference between the carriers and normal controls (q = 0.269, P > 0.05). The serum level of MDA in patients and carriers was significantly higher than that in normal controls (q = 9.069 and 4.748, respectively, both P < 0.05), and it was also significantly higher in patients than that in carriers (q = 3.618, P < 0.05).

CONCLUSIONS

Antioxidant capacity decreased significantly in patients with LHON, which indicates that the onset of LHON was related with the oxidation-antioxidation imbalance.

摘要

目的

测定携带线粒体G11778A突变的Leber遗传性视神经病变(LHON)患者血清中总超氧化物歧化酶(SOD)活性和丙二醛(MDA)水平,评估其氧化-抗氧化状态。

方法

本研究纳入了来自三个中国G11778A LHON家系的19例患者和12例携带者,并招募了30名年龄匹配的健康志愿者作为正常对照。分别采用黄嘌呤氧化酶法和硫代巴比妥酸法测定所有受试者血清中总SOD活性和MDA水平。

结果

LHON患者血清总SOD活性水平显著低于携带者和正常对照(q分别为7.085和8.351,均P<0.05),然而,携带者与正常对照之间差异无统计学意义(q=0.269,P>0.05)。患者和携带者血清MDA水平显著高于正常对照(q分别为9.069和4.748,均P<0.05),且患者血清MDA水平显著高于携带者(q=3.618,P<0.05)。

结论

LHON患者抗氧化能力显著下降,提示LHON的发病与氧化-抗氧化失衡有关。

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