[New findings on the pathogenesis of nephrotic syndrome (review article)].
Increased glomerular permeability of the glomerular capillary wall for macromolecules caused by the changes of the structure of the glomerular basement membrane, or podocytes and slit diaphragm between foot processes of podocytes is the main cause of nephrotic syndrome. Recently new information about podocyte proteins emerged. Mutation of the basic structural protein of slit diaphragm, nephrin, results in the Finnish type of the congenital nephrotic syndrome, mutations of other podocyte proteins, e.g. podocin, or alpha-actinin-4 result in congenital focal segmental glomerulosclerosis. Primary focal segmental glomerulosclerosis is a clinical syndrome, caused either by the mutation of podocyte proteins, or by circulating permeability factors, or by the deficiency of their circulating inhibitors. New information about the role of cubilin and megalin in the reabsorption of filtered albumin in the proximal tubule may contribute to the elucidation of the mechanisms of the tubulotoxicity of proteinuria; inhibition of albumin reabsorption in nephrotic subjects could lower the risk of interstitial fibrosis and progressive renal insufficiency.
Tesar
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V
I. interní klinika 1. lékarské fakulty Univerzity Karlovy a Vseobecné fakultní nemocnice, U nemocnice 2, 128 08 Praha 2, Czech Republic.
Zima
T
T
Kalousová
M
M
D006801:Humans; D007678:Kidney Glomerulus / Q000503:physiopathology; D008565:Membrane Proteins / Q000235:genetics / Q000502:physiology; D009404:Nephrotic Syndrome / Q000235:genetics / Q000503:physiopathology*; D010539:Permeability
D008565:Membrane Proteins
Novinky v patogenezi nefrotického syndromu (prehledový clánek).