PAHTOGENESIS ON MITOCHONDRIAL ANGIOPATHY AND ITS REPAIR BY GENE INJECTION

线粒体脑瘫、肌病、乳酸酸中毒和卒中样发作(MELAS)综合征的特征是线粒体DNA(MtDNA)突变(A3243G)和脑内线粒体血管病变。为了研究线粒体血管病变的发病机制，我们试图构建携带MELAS突变的来源于人脑血管内皮细胞的胞质杂交体。首先，我们确定了减少或去除血管内皮细胞线粒体DNA的最佳溴化乙锭浓度。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突变的线虫很难生存。我们应该进一步研究使线虫存活的最佳培养条件。

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.