Glomerular basement membrane composition and the filtration barrier
- نوع فایل : کتاب
- زبان : انگلیسی
- مؤلف : Jeffrey H. Miner
- چاپ و سال / کشور: 2011
Description
The glomerular basement membrane (GBM) is an especially thick basement membrane that contributes importantly to the kidney’s filtration barrier. The GBMderives from the fusion of separate podocyte and endothelial cell basement membranes during glomerulogenesis and consists primarily of laminin-521 (ل5â2م1), collagen ل3ل4ل5(IV), nidogens-1 and -2, and agrin. Of these nine proteins, mutations in the genes encoding four of them (LAMB2, COL4A3, COL4A4, and COL4A5) cause glomerular disease in humans as well as in mice. Furthermore, mutation of a fifth (Lama5) gene in podocytes in mice causes proteinuria, nephrotic syndrome, and progression to renal failure. These results highlight the importance of the GBM for establishing and maintaining a properly functioning glomerular filtration barrier.
Pediatr Nephrol (2011) 26:1413–1417 DOI 10.1007/s00467-011-1785-1 Received: 3 November 2010 / Revised: 20 January 2011 / Accepted: 21 January 2011 / Published online: 15 February 2011
