title: Increased expression of platelet-derived growth factor A and collagenous matrix proteins in congenital multicystic renal dysplasia.
abstract: 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.
authors.affiliation: Department of Pathology, Washington University School of Medicine, St. Louis, Mo. 63110, USA. liapis@path.wustl.edu
authors.forename: H
authors.initials: H
authors.lastname: Liapis
authors.forename: H
authors.initials: H
authors.lastname: Yu
authors.forename: A
authors.initials: A
authors.lastname: Flath
authors.forename: G F
authors.initials: GF
authors.lastname: Steinhardt
mesh_terms: D003094:Collagen / Q000096:biosynthesis*; D004848:Epithelium / Q000378:metabolism / Q000473:pathology; D005260:Female; D005355:Fibrosis; D006801:Humans; D017403:In Situ Hybridization; D007223:Infant; D007231:Infant, Newborn; D007668:Kidney / Q000002:abnormalities* / Q000378:metabolism* / Q000473:pathology; D008297:Male; D010982:Platelet-Derived Growth Factor / Q000096:biosynthesis*; D007690:Polycystic Kidney Diseases / Q000151:congenital / Q000378:metabolism* / Q000473:pathology; D011347:Procollagen / Q000096:biosynthesis*; D012333:RNA, Messenger / Q000096:biosynthesis; D014158:Transcription, Genetic
chemical_list: D010982:Platelet-Derived Growth Factor; D011347:Procollagen; D012333:RNA, Messenger; D003094:Collagen