Rascle Anne, Suleiman Hani, Neumann Tanja, Witzgall Ralph
Institute for Molecular and Cellular Anatomy, University of Regensburg, Regensburg, Germany.
Nephron Exp Nephrol. 2007;106(2):e60-6. doi: 10.1159/000101794. Epub 2007 Jun 6.
Despite a wealth of information on structural proteins, comparatively little is known on the transcriptional regulation of podocyte structure and function. In this review we will highlight those transcription factors which, by gene inactivation or classical transgenic experiments, have been shown to be essential for podocytes or probably will turn out to be so. The tumor suppressor protein WT1 is not only indispensable for the initial stages of kidney development, but also very likely maintains the integrity of the fully differentiated podocyte. In the kidney, the LIM homeodomain transcription factor LMX1B is specifically synthesized in podocytes, and mutations in LMX1B lead to nail-patella syndrome and the associated nephropathy. Other transcription factors such as hypoxia-inducible factors and PAX2 are likely to play a role in podocytes, whereas the significance of others, e.g. of POD1 and CITED2, is more speculative at this point.
尽管关于结构蛋白已有大量信息,但对于足细胞结构和功能的转录调控却知之甚少。在本综述中,我们将重点介绍那些通过基因失活或经典转基因实验已被证明对足细胞至关重要或可能最终被证明如此的转录因子。肿瘤抑制蛋白WT1不仅对肾脏发育的初始阶段不可或缺,而且很可能维持完全分化的足细胞的完整性。在肾脏中,LIM同源结构域转录因子LMX1B在足细胞中特异性合成,LMX1B的突变会导致指甲-髌骨综合征及相关肾病。其他转录因子,如缺氧诱导因子和PAX2,可能在足细胞中发挥作用,而其他一些转录因子,如POD1和CITED2,目前其意义更多是推测性的。