Increased expression of platelet-derived growth factor A and collagenous matrix proteins in congenital multicystic renal dysplasia.

The expression of platelet-derived growth factor A (PDGF-A), and its spatial and temporal relationship to interstitial collagens in kidneys with congenital multicystic dysplasia using in situ hybridization, have been examined. Seventeen dysplastic kidneys (16 weeks to 7 months) and 20 normal age-matched controls were used in the study. Increased PDGF-A mRNA was detected in dysplastic compared to normal kidneys in all age groups including extensively fibrotic postnatal kidneys. An abundant PDGF-A mRNA signal was seen within the epithelial cells of cystically dilated or dysplastic tubules and within interstitial fibroblasts and disorganized primitive mesenchyme. A comparable amount of PDGF-A protein was detected by Western blotting. Procollagen I and III mRNA were increased in fibroblasts surrounding cystic and dysplastic tubules. We conclude that tubular epithelial production of PDGF-A may induce collagenous matrix production by adjacent fibroblasts, while marked up-regulation of PDGF-A by interstitial cells may be responsible for sustainable fibrogenic effects in the fetal kidney contributing to renal maldevelopment.