PAHTOGENESIS ON MITOCHONDRIAL ANGIOPATHY AND ITS REPAIR BY GENE INJECTION

线粒体脑病、肌病、乳酸酸中毒和卒中样发作（MELAS）综合征以线粒体DNA （mtDNA）突变（A3243G）和脑线粒体血管病变为特征。为了研究线粒体血管病变的致病机制，我们试图构建来自人脑血管内皮细胞的携带MELAS突变的细胞系。首先，我们确定了溴化乙啶（EtBr）减少或去除血管内皮细胞mtDNA的最佳浓度。然后，我们将MELAS患者的血小板线粒体导入缺乏mtDNA的血管内皮细胞。携带mtDNA突变的杂交体很难存活。我们需要进一步研究使杂交体存活的最佳培养基条件。

Mitochondrial encephalplathy, myopathy, lactic acidosis and strokelike episodes (MELAS) syndrome is characterized by a mitochondrial DNA (mtDNA) mutation (A3243G) and mitochondrial angiopathy in the brain. To investigate the pathogenic mechanism for mitochondrial angiopathy, we tried to construct cybrids that carry the MELAS mutation and were derived from human brain vessel endothelial cells. First, we determined the optimal concentration of ethidium bromide (EtBr) to reduce or remove mtDNA from the blood vessel endothelial cells. We then introduced the platelet mitochondria from MELAS patients into the blood vessel endothelial cells lacking mtDNA. Cybrids carrying the mtDNA mutation were hard to survive. We should further investigate the optimal media condition to get cybrids survived.