Journal
EXPERIMENTAL CELL RESEARCH
Volume 318, Issue 9, Pages 973-978Publisher
ELSEVIER INC
DOI: 10.1016/j.yexcr.2012.02.031
Keywords
Laminin; Collagen IV; Alport syndrome; Pierson syndrome
Categories
Funding
- NIH [R01DK078314, R01GM060432, P30DK079333]
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The kidney's glomerular filtration barrier consists of two cells-podocytes and endothelial cells- and the glomerular basement membrane (GBM), a specialized extracellular matrix that lies between them. Like all basement membranes, the GBM consists mainly of laminin, type IV collagen, nidogen, and heparan sulfate proteoglycan. However, the GBM is unusually thick and contains particular members of these general protein families, including laminin-521, collagen alpha 3 alpha 4 alpha 5(IV), and agrin. Knockout studies in mice and genetic findings in humans show that the laminin and type IV collagen components are particularly important for GBM structure and function, as laminin or collagen IV gene mutations cause filtration defects and renal disease of varying severities, depending on the nature of the mutations. These studies suggest that the GBM plays a crucial role in establishing and maintaining the glomerular filtration barrier. (C) 2012 Elsevier Inc. All rights reserved.
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