Heparan sulfate--rich anionic sites in the human glomerular basement membrane. Decreased concentration in congenital nephrotic syndrome.
Heparan sulfate--rich anionic sites in the human glomerular basement membrane. Decreased concentration in congenital nephrotic syndrome.
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硫酸乙酰肝素——人肾小球基底膜中富含阴离子的位点。
DOI：
10.1056/nejm198310273091701
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发表时间：
1983
期刊：
影响因子：
--
通讯作者：
Brown,DM
中科院分区：
文献类型：
--
作者：
Vernier,RL;Klein,DJ;Sisson,SP;Mahan,JD;Oegema,TR;Brown,DM
最近的工作表明，阴离子血浆蛋白穿透肾小球基底膜的正常屏障可能部分依赖于膜内负电荷部位的存在。我们描述了一种体外细胞化学方法，用于定量显示正常人肾小球基底膜中的阴离子位置。在5例年龄为10天~57岁的正常人胸骨外膜上分布有规律的斑点，平均每1000 nm膜有23.8±6.8个斑点。在三个正常人胎儿的基底膜中也观察到类似的分布。一个身体肾脏的体外灌流显示出类似的阴离子位置分布。5例先天性肾病综合征患者肾小球基底膜阴离子位点数减少至8.90±3.7P<；0.001。在纯化的肝素酶中预先孵育正常肾脏的切片，可显著减少阴离子部位的数量。我们得出结论，先天性肾病是由于富含硫酸肝素的阴离子部位未能在肾小球基底膜的rara外层形成所致。《英国医学杂志》1983；309：1001-9。
Recent work suggests that the normal barrier to penetration of the renal glomerular basement membrane by anionic plasma proteins may depend in part on the existence of negatively charged sites within the membrane. We describe an in vitro cytochemical method for the quantitative demonstration of anionic sites in the normal human glomerular basement membrane. In five normal subjects, ranging in age from 10 days to 57 years, the sites were distributed at regular intervals in the lamina rara externa, with a frequency of 23.8±6.8 sites per 1000-nm length of membrane. A similar distribution was observed in the basement membranes from three normal human fetuses. Ex vivo perfusion of one cadaver kidney revealed a similar distribution of anionic sites. The number of anionic sites in the glomerular basement membranes of five patients with the congenital nephrotic syndrome was reduced to 8.9±3.7 (P<0.001). Prior incubation of sections of normal kidney in purified heparinase resulted in a marked reduction in the number of anionic sites. We conclude that congenital nephrosis results from failure of heparan sulfate–rich anionic sites to develop in the lamina rara externa of the glomerular basement membrane. (N Engl J Med 1983; 309:1001–9.)