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帕金森病患者线粒体功能缺陷的研究 被引量:8

The study of mitochondrial dysfunction in patients with Parkinson disease
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摘要 目的 探讨帕金森病 (PD)患者线粒体功能缺陷类型及其基因突变基础。方法 用溴化乙啶抑制人食管癌细胞系的线粒体DNA复制 ,制备出无线粒体DNA细胞系。将 2 0例PD患者组及2 0名正常对照组血小板与之融合 ,用选择性培养液挑选出融合细胞 ,大量培养后用极谱法测定线粒体酶复合体活性及抗氰呼吸。结果 以苹果酸和谷氨酸为底物时患者组 ( 1.2 5± 0 .0 8)明显低于正常对照组 ( 1.75± 0 .0 8) ,降低 2 8.6% ;以琥珀酸、维生素C和TMPD为底物时患者组与正常对照组差别无统计学意义。这些结果表明 ,线粒体酶复合体II~Ⅴ活性正常 ,以苹果酸和谷氨酸为底物的氧耗率的降低来源于酶复合体I活性受损。由于融合细胞核背景一致 ,其线粒体功能仅受mtDNA影响 ,因此本试验发现的患者酶复合体I缺陷来源于mtDNA的突变。抗氰呼吸PD患者组平均为 ( 8.76±0 .2 5 ) % ,正常对照组平均为 ( 6.2 0± 0 .10 ) % ,差异有显著意义 (P <0 .0 5 )。结论 PD患者线粒体酶复合体I活性降低 ,来源于线粒体DNA突变 ,可能导致自由基增多 ,在PD神经元变性中起重要作用。 Objective The mitochondrial complex I, which is encoded by both mitochondrial DNA and nuclear DNA, is defective in multiple tissues in patients with Parkinson disease (PD). The origin of the lesion is unknown. In order to address the question, we used esophageal carcinoma cell lines containing no mitochondrial DNA to detect mitochondrial functions of PD patients or control subjects. Methods We established mitochondrial DNA depleted cell lines from human esophageal carcinoma cells with ethidium bromide (EB). The platelets were fused with ρ 0 cell in PEG. The cybrids were selected with the selective medium (without pyruvate and uridine). Mitochondriasl respiration defects and anti cyano respiration were detected with the polarography with different substrates. Results In the PD cybrids, the complex I activity decreased 28.6% and anti cyano respiration increased. Conclusion The complex I defect in PD appeared to be genetic, arsing from mitochondrial DNA, and might play an important role in the degeneration of PD by forstering reactive oxygen species production.
出处 《中华神经科杂志》 CAS CSCD 1999年第4期199-201,共3页 Chinese Journal of Neurology
基金 国家自然科学基金!No .3 9870 2 75 国家教委博士点基金
关键词 帕金森病 线粒体功能缺陷 基因突变 Parkinson disease Mitochondrial
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