线粒体脑肌病伴乳酸血症和卒中样发作（MELAS）患者的内皮紧密连接破坏
Disruption of endothelial tight junctions in a patient with mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes (MELAS).
作者信息
Matsuzaki M, Takahashi R, Nakayama T, Shishikura K, Suzuki H, Hirayama Y, Osawa M, Oda H
机构信息
Department of Pediatrics, Tokyo Women's Medical University, Tokyo, Japan.
出版信息
Neuropediatrics. 2010 Aug;41(2):72-4. doi: 10.1055/s-0030-1261886. Epub 2010 Aug 26.
An electron microscopic study revealed disruption of capillary endothelial tight junctions (TJs) in both biopsied muscle, taken at 5 years and 1 month of age, and the autopsied brain, taken at 13 years and 6 months of age, in a patient with mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes (MELAS) and mitochondrial DNA (mtDNA) point mutation A3243G. This endothelial barrier disruption might result in vasogenic edema and systemic lactic acidosis, possibly the critical pathology of MELAS.
一项电子显微镜研究显示，在一名患有线粒体脑肌病、乳酸酸中毒和卒中样发作（MELAS）以及线粒体DNA（mtDNA）点突变A3243G的患者中，在5岁1个月时获取的活检肌肉以及13岁6个月时获取的尸检大脑中，毛细血管内皮紧密连接（TJ）均遭到破坏。这种内皮屏障破坏可能导致血管源性水肿和全身性乳酸酸中毒，这可能是MELAS的关键病理改变。
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