Distribution of renal integrin receptors and their ligands in congenital nephrotic syndrome of the Finnish type.

Abstract

The aim of this study was to examine the distribution of beta 1 and alpha v integrins (Ints) and some of their ligands in the kidneys of patients with congenital nephrotic syndrome of the Finnish type (CNF) and in controls using indirect immunofluorescence with monoclonal antibodies. The mesangial reactivity of Int alpha 1 and Int beta 1 subunits was more variable and an increased glomerular reactivity with Int alpha 3 and Int-alpha 6 antibodies was found in CNF kidneys than in controls. Int alpha 2 subunit was either completely missing from or found in significantly lesser amounts in CNF kidney glomeruli. The immunoreactivity for Int alpha v was more variable, fainter and also more granular in CNF samples than in control kidneys. The glomerular reactivity for Int beta 5 was more diffuse and weaker, and in sclerotic Bowman's capsules more intense in CNF kidneys than in controls. Immunoreactivity for Int beta 6 was restricted and was comparable in extent in CNF and control kidneys. Of the extracellular matrix components studied, the expression of EDAFn, EDBFn, OncFn, Ln alpha 2 chain, Ln beta 1 chain and tenascin was increased. This is also seen in several glomerular diseases with inflammation and sclerosis. Immunoreactivity for vitronectin was decreased. Several differences were found in the intensity or location of the immunostaining for the beta 1 and alpha v Ints and their ligands in CNF kidneys compared with controls, which have not been found in any other proteinuric disease. Disturbed Int expression pattern in CNF may specifically reflect the disturbance of glomerular function caused by the primary defect in this disease.