评估依地酸和相关醌类似物在莱伯遗传性视神经病变中的治疗潜力。
Evaluating the therapeutic potential of idebenone and related quinone analogues in Leber hereditary optic neuropathy.
机构信息
Wellcome Trust Centre for Mitochondrial Research, Institute of Genetic Medicine, Newcastle University, Newcastle upon Tyne, UK; Newcastle Eye Centre, Royal Victoria Infirmary, Newcastle upon Tyne, UK; NIHR Biomedical Research Centre at Moorfields Eye Hospital and UCL Institute of Ophthalmology, London, UK; Department of Clinical Neurosciences, School of Clinical Medicine, University of Cambridge, Cambridge, UK.
Monique and Jacques Roboh Department of Genetic Research, Hadassah-Hebrew University Medical Center, Jerusalem, Israel; Department of Genetics and Metabolic Diseases, Hadassah-Hebrew University Medical Center, Jerusalem, Israel.
出版信息
Mitochondrion. 2017 Sep;36:36-42. doi: 10.1016/j.mito.2017.01.004. Epub 2017 Jan 16.
Leber hereditary optic neuropathy (LHON) is an important cause of mitochondrial blindness among young adults. In this study, we investigated the potential of four quinone analogues (CoQ, CoQ, decylubiquinone and idebenone) in compensating for the deleterious effect of the m.11778G>A mitochondrial DNA mutation. The LHON fibroblast cell lines tested exhibited reduced cell growth, impaired mitochondrial bioenergetics and elevated levels of reactive oxygen species (ROS). Idebenone increased ATP production and reduced ROS levels, but the effect was partial and cell-specific. The remaining quinone analogues had variable effects and a negative impact on certain mitochondrial parameters was observed in some cell lines.
Leber 遗传性视神经病变（LHON）是年轻人线粒体失明的一个重要原因。在这项研究中，我们研究了四种醌类似物（CoQ、CoQ、癸基泛醌和艾地苯醌）在补偿 m.11778G>A 线粒体 DNA 突变的有害影响方面的潜力。测试的 LHON 成纤维细胞系表现出细胞生长减少、线粒体生物能量受损和活性氧（ROS）水平升高。艾地苯醌增加了 ATP 的产生并降低了 ROS 水平，但这种效果是部分的，并且是细胞特异性的。其余的醌类似物的作用各不相同，并且在某些细胞系中观察到某些线粒体参数的负面影响。
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