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首页> 外文期刊>Proceedings of the National Academy of Sciences of the United States of America >Nephrin is specifically located at the slit diaphragm of glomerular podocytes
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Nephrin is specifically located at the slit diaphragm of glomerular podocytes

机译：肾素特别位于肾小球足细胞的裂隙膜上

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摘要

We describe here the size and location of nephrin , the first protein to be identified at the glomerular podocyte slit diaphragm. In Western blots, nephrin antibodies generated against the two terminal extracellular Ig domains of recombinant human nephrin recognized a 180-kDa protein in lysates of human glomeruli and a 150-kDa protein in atransfected COS-7 cell lysates. In immunofluorescence, antibodies to this transmembrane protein revealed reactivity in the glomerylar basement membrane region, whereas the podocyte cell bodies remained negative. In immunogold-stained thin sections, nephrin label was found at the slit between podocyte foot processes. The congenital nephrotic syndrome of the Finnish type NPHS1), a disease in which the nephrin gene is mutated, is characterized by massive proteinuria already in uatero and lack of slit diaphragm and foot processe. These features, together with the now demonstrated localizaation of nephrin to the slit deaphragm area, suggests an essential role for this protein in the normal glomerular filtration barrier. A zipper-like model for nephrin assembly in athe slit deaphragm is discussed, based on the present and previous data.
机译：我们在这里描述nephrin的大小和位置，nephrin是第一个在肾小球足细胞裂隙隔膜处被识别的蛋白质。在蛋白质印迹中，针对重组人肾素的两个末端胞外Ig结构域产生的肾素抗体识别人肾小球裂解液中的180 kDa蛋白和未转染的COS-7细胞裂解液中的150 kDa蛋白。在免疫荧光中，针对该跨膜蛋白的抗体显示出在肾小球基底膜区域的反应性，而足细胞细胞体保持阴性。在免疫金染色的薄切片中，在足细胞足突之间的缝隙中发现了肾素标记。芬兰型NPHS1）的先天性肾病综合征是一种肾素基因突变的疾病，其特征是尿液中已经存在大量蛋白尿，缺乏diaphragm肌和足突。这些特征，以及现已证明的肾素定位在狭缝无瓣膜区域，提示该蛋白在正常肾小球滤过屏障中起着至关重要的作用。基于当前和以前的数据，讨论了缝隙无囊膜中肾素组装的拉链状模型。

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