Monday 24 October 2005
The glomerular filtration barrier is composed of endothelial cells, basement membrane, and podocytes.
The slit diaphragm, which is seen as a membrane covering the space between adjacent foot processes close to the basement membrane, is an extremely important structure with a crucial role in permselectivity of the filtration barrier. Its composition is now understood to consist primarily of a unique protein called nephrin.
Mutations in the gene-encoding nephrin are known to result in the Finnish type of nephrotic syndrome. The exact mechanism by which nephrin controls permselectivity is not yet clear, but it is known to interact with several podocyte proteins including CD2AP, podocin, and alpha-actinin-4.
Abnormalities of any of these proteins may result in proteinuria.
common acquired glomerulopathies
transcription factors (WT1, PAX2)
Tryggvason K, Patrakka J, Wartiovaara J. Hereditary proteinuria syndromes and mechanisms of proteinuria. N Engl J Med. 2006 Mar 30;354(13):1387-401. PMID: 16571882
Akhtar M, Al Mana H. Molecular basis of proteinuria. Adv Anat Pathol. 2004 Nov;11(6):304-9. PMID: 15505531