Glomerular basement membrane composition and the filtration barrier
- 1 September 2011
- journal article
- review article
- Published by Springer Science and Business Media LLC in Pediatric Nephrology
- Vol. 26 (9), 1413-1417
- https://doi.org/10.1007/s00467-011-1785-1
Abstract
The glomerular basement membrane (GBM) is an especially thick basement membrane that contributes importantly to the kidney’s filtration barrier. The GBM derives from the fusion of separate podocyte and endothelial cell basement membranes during glomerulogenesis and consists primarily of laminin-521 (α5β2γ1), collagen α3α4α5(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.Keywords
This publication has 39 references indexed in Scilit:
- The Molecular Basis of Goodpasture and Alport SyndromesJournal of the American Society of Nephrology, 2004
- Congenital nephrosis, mesangial sclerosis, and distinct eye abnormalities with microcoria: An autosomal recessive syndromeAmerican Journal of Medical Genetics Part A, 2004
- Glomerular endothelial cells and podocytes jointly synthesize laminin-1 and -11 chainsKidney International, 2001
- Alport syndromes: phenotypic heterogeneity of progressive hereditary nephritis.Pediatric Nephrology, 2000
- Disruption of Laminin β2 Chain Production Causes Alterations in Morphology and Function in the CNSJournal of Neuroscience, 1999
- Distribution of the α1 and α2 chains of collagen IV and of collagens V and VI in Alport syndromeKidney International, 1992
- The tight junction protein ZO-1 is concentrated along slit diaphragms of the glomerular epithelium.The Journal of cell biology, 1990
- Identification of Mutations in the COL4A5 Collagen Gene in Alport SyndromeScience, 1990
- Origin of the glomerular basement membrane visualized after in vivo labeling of laminin in newborn rat kidneys.The Journal of cell biology, 1985
- The primary glomerular filtration barrier—basement membrane or epithelial slits?Kidney International, 1975