Arif Ehtesham, Nihalani Deepak
Renal Electrolytes and Hypertension Division, University of Pennsylvania, Philadelphia, PA 19104.
Postdoc J. 2013 Apr;1(4):33-45.
A glomerulus is the network of capillaries that resides in the Bowman's capsule that functions as a filtration unit of kidney. The glomerular function ensures that essential plasma proteins are retained in blood and the filtrate is passed on as urine. The glomerular filtration assembly is composed of three main cellular barriers that are critical for the ultrafiltration process, the fenestrated endothelium, glomerular basement membrane and highly specialized podocytes. The podocytes along with their specialized junctions "slit diaphragm" form the basic backbone of this filtration assembly. The presence of high amounts of protein in urine a condition commonly referred as proteinuria indicates a defective glomerular filtration barrier. Various glomerular disorders including Nephrotic syndrome are characterized by significant alteration in the structure of podocytes that is associated with prolonged increase in the glomerular permeability leading to heavy proteinuria. Recent identification of proteins that are specifically localized at the slit diaphragm whose mutations and knockouts are known to result in loss of renal function has significantly advanced our understanding of the molecular makeup of this filtration assembly. The present review is an effort to summarize the recent developments in this field and highlight our understanding of the glomerular filtration barrier assembly.
肾小球是位于鲍曼囊内的毛细血管网络,起着肾脏滤过单位的作用。肾小球功能可确保血液中保留必需的血浆蛋白,并使滤液作为尿液排出。肾小球滤过组件由对超滤过程至关重要的三个主要细胞屏障组成,即有孔内皮、肾小球基底膜和高度特化的足细胞。足细胞及其特化连接“裂孔隔膜”构成了这个滤过组件的基本框架。尿液中存在大量蛋白质(一种通常称为蛋白尿的情况)表明肾小球滤过屏障存在缺陷。包括肾病综合征在内的各种肾小球疾病的特征是足细胞结构发生显著改变,这与肾小球通透性长期增加导致大量蛋白尿有关。最近发现了特异性定位于裂孔隔膜的蛋白质,已知其突变和敲除会导致肾功能丧失,这极大地推进了我们对这个滤过组件分子组成的理解。本综述旨在总结该领域的最新进展,并突出我们对肾小球滤过屏障组件的理解。