Pediatric Nephrology

, Volume 26, Issue 9, pp 1413–1417

Glomerular basement membrane composition and the filtration barrier

Review

DOI: 10.1007/s00467-011-1785-1

Cite this article as:
Miner, J.H. Pediatr Nephrol (2011) 26: 1413. doi: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

Laminin Collagen IV Podocyte Basement membrane Nephrotic syndrome 

Copyright information

© IPNA 2011

Authors and Affiliations

  1. 1.Renal Division, Department of Internal Medicine, School of MedicineWashington UniversitySt. LouisUSA

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