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Pediatric Nephrology
Published in: Pediatric Nephrology 9/2011

01-09-2011 | Review

Glomerular basement membrane composition and the filtration barrier

Author: Jeffrey H. Miner

Published in: Pediatric Nephrology | Issue 9/2011

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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.
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Metadata
Title
Glomerular basement membrane composition and the filtration barrier
Author
Jeffrey H. Miner
Publication date
01-09-2011
Publisher
Springer Berlin Heidelberg
Published in
Pediatric Nephrology / Issue 9/2011
Print ISSN: 0931-041X
Electronic ISSN: 1432-198X
DOI
https://doi.org/10.1007/s00467-011-1785-1

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