Glomerular basement membrane composition and the filtration barrier

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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.

Original languageEnglish
Pages (from-to)1413-1417
Number of pages5
JournalPediatric Nephrology
Volume26
Issue number9
DOIs
StatePublished - Sep 2011

Keywords

  • Basement membrane
  • Collagen IV
  • Laminin
  • Nephrotic syndrome
  • Podocyte

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