Monnens L A
Tijdschr Kindergeneeskd. 1982 Oct;50(5):121-30.
The glomerular basement membrane consists of the lamina densa and two laminae rarae. The glomerular basement membrane (GBM) functions as both the size and the charge barrier to filtration of macromolecules. Several substances have been localized in the GBM: collagen type IV, V, laminin, entactin, fibronectin, amyloid P and proteoglycans. Anionic sites are present on the epithelial and endothelial cell surface (the cell coat or glycocalyx) due to the presence of sialic acid. The negative charges in the GBM concentrate in the lamina rara interna and lamina rara externa. This anionic charge is in large part caused by heparansulphate. Congenital abnormalities of the biochemical composition are expected in the congenital nephrotic syndrome, in minimal change nephropathy, in Alport's syndrome, in Laurence-Moon-Biedl syndrome and in nail-patella syndrome. The main function of the tubular basement membrane (TBM) is to provide support to the epithelium. The chemical composition of TBM differs from the composition of GBM. Some forms of polycystic disease can perhaps be explained by an abnormal chemical composition of TBM.
肾小球基底膜由致密层和两层疏松层组成。肾小球基底膜（GBM）对大分子物质的滤过起着大小屏障和电荷屏障的作用。几种物质已定位在GBM中：IV型、V型胶原蛋白、层粘连蛋白、内动蛋白、纤连蛋白、淀粉样蛋白P和蛋白聚糖。由于唾液酸的存在，上皮细胞和内皮细胞表面（细胞被或糖萼）存在阴离子位点。GBM中的负电荷集中在内疏松层和外疏松层。这种阴离子电荷很大程度上是由硫酸乙酰肝素引起的。先天性肾病综合征、微小病变性肾病、阿尔波特综合征、劳-穆-比综合征和指甲-髌骨综合征中预计存在生化组成的先天性异常。肾小管基底膜（TBM）的主要功能是为上皮细胞提供支持。TBM的化学成分与GBM的成分不同。某些形式的多囊性疾病可能可以用TBM的异常化学成分来解释。
