Tian Xuefei, Ishibe Shuta
Section of Nephrology, Department of Internal Medicine, Yale University School of Medicine, New Haven, CT, USA.
Nephrol Dial Transplant. 2016 Oct;31(10):1577-83. doi: 10.1093/ndt/gfw021. Epub 2016 Mar 10.
Glomerular injury often incites a progression to chronic kidney disease, which affects millions of patients worldwide. Despite our current understanding of this disease's pathogenesis, there is still a lack of therapy available to curtail its progression. However, exciting new data strongly suggest the podocyte-an actin-rich, terminally differentiated epithelial cell that lines the outside of the glomerular filtration barrier-as a therapeutic target. The importance of podocytes in the pathogenesis of human nephrotic syndrome is best characterized by identification of genetic mutations, many of which regulate the actin cytoskeleton. The intricate regulation of the podocyte actin cytoskeleton is fundamental in preserving an intact glomerular filtration barrier, and this knowledge has inspired new research targeting actin-regulating proteins in these cells. This review will shed light on recent findings, which have furthered our understanding of the molecular mechanisms regulating podocyte actin dynamics, as well as discoveries that have therapeutic implications in the treatment of proteinuric kidney disease.
肾小球损伤常常会促使病情进展为慢性肾病,全球有数百万患者受其影响。尽管我们目前对这种疾病的发病机制有所了解,但仍然缺乏能够遏制其进展的治疗方法。然而,令人振奋的新数据有力地表明,足细胞——一种富含肌动蛋白、终末分化的上皮细胞,排列在肾小球滤过屏障外侧——可作为一个治疗靶点。足细胞在人类肾病综合征发病机制中的重要性,通过基因突变的鉴定得到了最好的体现,其中许多基因突变都调节肌动蛋白细胞骨架。足细胞肌动蛋白细胞骨架的复杂调控对于维持完整的肾小球滤过屏障至关重要,这一认识激发了针对这些细胞中肌动蛋白调节蛋白的新研究。本综述将阐明近期的研究发现,这些发现进一步加深了我们对调节足细胞肌动蛋白动力学分子机制的理解,以及对蛋白尿性肾病治疗具有潜在治疗意义的发现。