肾小球基底膜发育及相关疾病的研究进展

Progress in development of glomerular basement membrane and related diseases

肾小球是位于肾皮质的球形毛细血管簇,血液经肾小球滤过形成原尿。其中,肾小球滤过屏障起重要作用,是位于循环系统和鲍曼囊之间由肾小球基底膜(glomerular basement membrane,GBM)、鲍曼囊脏层上皮细胞(即足细胞)、足细胞裂孔膜、肾小球毛细血管内皮细胞和内皮细胞表面膜共同构成的有机整体,将血液与尿液分隔开,在阻碍血浆蛋白(主要是白蛋白和免疫球蛋白)通过的同时,确保水和构成初级滤液的小溶质有效滤过。

The glomerular basement membrane(GBM)is a complex reticular protein structure composed of various macromolecules and is a key component of renal filtration.As the development of the glomerulus,including its components and structure,continuously change until maturity,the exact mechanisms contributing to its development remain unclear.Furthermore,it has been revealed that abnormal development of the GBM can lead to various primary and secondary GBM diseases.Therefore,this article aims to enhance the current understanding by briefly reviewing research progress on the GBM,including its components,developmental processes,and GBM-related diseases.