Quaggin Susan E
Division of Nephrology, St. Michael's Hospital and Department of Maternal and Fetal Health, The Samuel Lunenfeld Research Inst., Mt. Sinai Hospital, Toronto, Ontario, M5G 1X5 Canada.
Microsc Res Tech. 2002 May 15;57(4):208-11. doi: 10.1002/jemt.10076.
Glomerular visceral epithelial cells (podocytes) are highly specialized cells found in the vertebrate and invertebrate kidney and make up a major portion of the filtration barrier between blood and urinary spaces. During development, specification and differentiation of the podocyte lineage must be tightly orchestrated to produce highly specialized characteristics such as foot processes and slit diaphragms. Furthermore, podocytes are poised to direct incoming endothelial and mesangial cells during glomerular development. They express a number of growth factors that likely play a major role in these processes. Recent findings from transgenic and knockout mouse models and the identification of genes responsible for human podocyte disease have provided insight into transcriptional regulation of some of these processes. These transcription factors include Pax2, WT1 (the Wilms tumor suppressor gene), Pod1 (capsulin, epicardin), Kreisler (maf-1), lmx1b, and mf2. Furthermore, regulatory regions from a podocyte-restricted gene, NPHS1 (nephrin) that are required to direct podocyte-specific expression have been identified from both human and murine genes and provide a tool to further dissect the transcriptional regulation of podocyte-specific gene expression. This article reviews the present state of knowledge regarding transcriptional regulation of podocyte specification and differentiation.
肾小球脏层上皮细胞（足细胞）是在脊椎动物和无脊椎动物肾脏中发现的高度特化细胞，构成了血液与尿液空间之间滤过屏障的主要部分。在发育过程中，足细胞谱系的特化和分化必须精确协调，以产生诸如足突和裂孔隔膜等高度特化的特征。此外，足细胞在肾小球发育过程中对传入的内皮细胞和系膜细胞具有引导作用。它们表达多种生长因子，这些因子可能在这些过程中起主要作用。来自转基因和基因敲除小鼠模型的最新发现以及对人类足细胞疾病相关基因的鉴定，为深入了解其中一些过程的转录调控提供了线索。这些转录因子包括Pax2、WT1（威尔姆斯瘤抑制基因）、Pod1（荚膜菌素、心外膜蛋白）、克雷斯勒（maf-1）、lmx1b和mf2。此外，已从人类和小鼠基因中鉴定出足细胞特异性基因NPHS1（nephrin）的调控区域，这些区域是指导足细胞特异性表达所必需的，为进一步剖析足细胞特异性基因表达的转录调控提供了工具。本文综述了有关足细胞特化和分化转录调控的当前知识状态。
