莱伯遗传性视神经病变中复合物II活性的代偿性升高。
Compensatory elevation of complex II activity in Leber's hereditary optic neuropathy.
作者信息
Yen M Y, Lee H C, Liu J H, Wei Y H
机构信息
Department of Ophthalmology, Taipei Veterans General Hospital, Taiwan.
出版信息
Br J Ophthalmol. 1996 Jan;80(1):78-81. doi: 10.1136/bjo.80.1.78.
AIMS
To evaluate the mitochondrial respiratory enzyme activities in blood cells of Leber's hereditary optic neuropathy (LHON) with 11778 point mutation of mitochondrial DNA.
METHODS
Assays for the activities of NADH-cytochrome c reductase (complex I+complex III), succinate-cytochrome c reductase (complex II+complex III), and cytochrome c oxidase (complex IV) on blood cell mitochondria of seven LHON patients and 15 normal controls.
RESULTS
There was no statistically significant difference in NADH-cytochrome c reductase and cytochrome c oxidase activities between LHON patients and controls, but activities of succinate-cytochrome c reductase in LHON patients was significantly elevated compared with normal controls.
CONCLUSION
The observations that the activity of NADH-cytochrome c reductase is normal but that of succinate-cytochrome c reductase is increased in LHON patients with 11778 point mutation of mitochondrial DNA indicate an elevation of complex II activity, which may be due to a nuclear compensatory effect for defects of the respiratory function of mitochondria.
目的
评估线粒体DNA发生11778点突变的Leber遗传性视神经病变（LHON）患者血细胞中线粒体呼吸酶活性。
方法
对7例LHON患者和15名正常对照者血细胞线粒体中的NADH - 细胞色素c还原酶（复合体I + 复合体III）、琥珀酸 - 细胞色素c还原酶（复合体II + 复合体III）以及细胞色素c氧化酶（复合体IV）活性进行测定。
结果
LHON患者与对照者之间，NADH - 细胞色素c还原酶和细胞色素c氧化酶活性无统计学显著差异，但LHON患者的琥珀酸 - 细胞色素c还原酶活性相较于正常对照者显著升高。
结论
线粒体DNA发生11778点突变的LHON患者中，NADH - 细胞色素c还原酶活性正常而琥珀酸 - 细胞色素c还原酶活性升高这一观察结果表明复合体II活性升高，这可能是由于对线粒体呼吸功能缺陷的核补偿效应所致。
Zotero 插件