线粒体呼吸链复合物线粒体呼吸链复合物Ⅰ缺陷与-南方医科大学学报.PDF

J South Med Univ, 2013, 33(9): 1357-1361 doi 10.3969/j.issn. 1673-4254.2013.09.22 ·1357 · 临床研究 线粒体呼吸链复合物线粒体呼吸链复合物 ⅠⅠ缺陷与海洛因海绵状白质脑病的关系缺陷与海洛因海绵状白质脑病的关系 周 亮，林敏仕，尹 恝 南方医科大学南方医院神经内科，广东 广州 510515 摘要：目的探讨线粒体呼吸链复合物Ⅰ缺陷与海洛因海绵状白质脑病的关系。方法 采用酶联免疫分析方法，应用双抗体夹 心，即线粒体呼吸链复合物Ⅰ抗体-线粒体呼吸链复合物Ⅰ抗原-酶标抗体复合物，加底物TMB 显色，用酶标仪在450 nm 波长下 测定吸光度。采用上述方法分别测定36 例急性期海洛因海绵状白质脑病患者与36 例正常对照者外周血白细胞线粒体呼吸链 复合物Ⅰ活性的吸光度，并比对标准曲线，计算样品中人线粒体呼吸链复合物I 浓度。结果 正常对照组和病患组线粒体呼吸链 复合物Ⅰ活性测定结果分别为5.6±2.4、4.2±2.1 U/ml ，两组间有显著差异（t=2.634 ，P0.05）。结论 急性期海洛因海绵状白质脑 病患者线粒体呼吸链复合物Ⅰ活性降低，存在线粒体功能缺陷。推测脑白质的广泛脱髓鞘与能量代谢障碍有关，线粒体功能障 碍在海洛因海绵状白质脑病发病过程中可能起到重要作用。 关键词：海洛因；白质脑病；线粒体呼吸链酶；复合物 Ⅰ缺陷 Relationship between heroin spongiform leucoencephalopathy and respiratory chain complex I deficiency ZHOU Liang, LIN Minshi, YIN Jia Department of Neurology, Nanfang Hospital, Southern Medical University, Guangzhou 510515, China Abstract: Objective To investigate the relationship between heroin spongiform leucoencephalopathy and respiratory chain complex I deficiency. Methods The activity of respiratory chain complex I in peripheral white blood cell mitochondria was compared between 36 cases of heroin spongiform leucoencephalopathy and 36 healthy subjects using enzyme-linked immunosorbent assay (ELISA). Results The activity of respiratory chain complex I was 5.6± 2.4 U/ml in patients with heroin spongiform leucoencephalopathy, signficiantly higher than that in the normal subjects (4.2 ± 2.1 U/ml, t=2.634, P0.05). Conclusion In patients with heroin spongiform leucoencephalopathy, mitochondrial dysfunction results in energy metabolism disorder to cause extensive demyelination of the cerebral white matter. Respiratory chain complex I deficiency of the mitochondria plays a significant role in the pathogenesis of heroin spongiform leucoencephalopathy. Key words: heroin; leucoencephalopathy; mitochondrial respiratory chain; co