Disruption of Endothelial Tight Junctions in a Patient with Mitochondrial Encephalomyopathy, Lactic Acidosis and Stroke-Like Episodes (MELAS)

被引：8
|
作者：
Matsuzaki, M. [1 ,3 ]
Takahashi, R.
Nakayama, T.
Shishikura, K.
Suzuki, H.
Hirayama, Y.
Osawa, M.
Oda, H. [2 ]
机构：
[1] Tokyo Womens Med Univ, Dept Pediat, Shinjuku Ku, Tokyo 1628666, Japan
[2] Tokyo Womens Med Univ, Dept Pathol, Tokyo 1628666, Japan
[3] Hosp Disabled, Saitama, Japan
来源：
NEUROPEDIATRICS | 2010年 / 41卷 / 02期
关键词：
mitochondrial encephalomyopathy; lactic acidosis and stroke-like episodes (MELAS); tight junctions; disruption of endothelial tight junctions; INVOLVEMENT;
D O I：
10.1055/s-0030-1261886
中图分类号：
R74 [神经病学与精神病学];
学科分类号：
摘要：
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.
引用
收藏
页码：72 / 74
页数：3
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