Department of Nephrology, University Hospital, Zürich, Switzerland.
Pediatr Nephrol. 2010 Jun;25(6):1017-23. doi: 10.1007/s00467-009-1425-1. Epub 2010 Feb 4.
Proteinuria is an early sign of kidney disease and has gained increasing attention over the past decade because of its close association with cardio-vascular and renal morbidity and mortality. Podocytes have emerged as the cell type that is critical in maintaining proper functioning of the kidney filter. A few genes have been identified that explain genetic glomerular failure and recent insights shed light on the pathogenesis of acquired proteinuric diseases. This review highlights the unique role of the cysteine protease cathepsin L as a regulatory rather than a digestive protease and its action on podocyte structure and function. We provide arguments why many glomerular diseases can be regarded as podocyte enzymatic disorders.
蛋白尿是肾脏疾病的早期迹象,由于其与心血管和肾脏发病率和死亡率密切相关,近年来受到越来越多的关注。足细胞已成为维持肾脏滤过功能正常的关键细胞类型。已经确定了一些基因,这些基因解释了肾小球的遗传衰竭,最近的研究结果揭示了获得性蛋白尿疾病的发病机制。本文重点介绍半胱氨酸蛋白酶组织蛋白酶 L 作为调节而不是消化蛋白酶的独特作用及其对足细胞结构和功能的作用。我们提供了一些论据,说明为什么许多肾小球疾病可以被视为足细胞酶紊乱。