title: Adult and paediatric patients with minimal change nephrotic syndrome show no major alterations in glomerular expression of sulphated heparan sulphate domains.
abstract: BACKGROUND
  Minimal change nephrotic syndrome (MCNS) is the most frequent form of nephrotic syndrome in childhood. In the glomerular basement membrane (GBM) of adult patients with MCNS, a reduced expression of a specific heparan sulphate (HS) domain has been reported. In children with MCNS, urinary activity of the HS-degrading enzyme heparanase was increased. It is, therefore, possible that a decreased GBM HS expression is associated with the pathogenesis of proteinuria in patients with MCNS.
  
  
  METHODS
  In this study, HS in glomeruli of five adult and six paediatric patients with MCNS were analysed by immunofluorescence staining using four different antibodies, each defining a specific sulphated HS domain. The pediatric patients were subdivided into three groups depending on the presence or absence of podocyte foot process effacement, the level of proteinuria and prednisone administration at the time of the biopsy. In addition, kidneys of rats with adriamycin nephropathy (ADRN), a model for MCNS, were included in the study.
  
  
  RESULTS
  Expression of sulphated HS domains was not aberrant in adult or paediatric patients compared with control subjects. Children with and without proteinuria had the same HS content. In contrast, rats with ADRN showed a decreased glomerular expression of sulphated HS domains.
  
  
  CONCLUSIONS
  These results suggest that in patients with MCNS proteinuria is not associated with major changes in glomerular expression of sulphated HS domains.
authors.affiliation: Department of Matrix Biochemistry, Nijmegen Centre for Molecular Life Sciences, Nijmegen, P.O. Box 9101, 6500 HB Nijmegen, The Netherlands.
authors.forename: Tessa J M
authors.initials: TJ
authors.lastname: Wijnhoven
authors.forename: Joyce M
authors.initials: JM
authors.lastname: Geelen
authors.forename: Marinka
authors.initials: M
authors.lastname: Bakker
authors.forename: Joost F M
authors.initials: JF
authors.lastname: Lensen
authors.forename: Angelique L W M M
authors.initials: AL
authors.lastname: Rops
authors.forename: Andrea B
authors.initials: AB
authors.lastname: Kramer
authors.forename: Gerjan
authors.initials: G
authors.lastname: Navis
authors.forename: Mabel J W
authors.initials: MJ
authors.lastname: van den Hoven
authors.forename: Johan
authors.initials: J
authors.lastname: van der Vlag
authors.forename: Jo H M
authors.initials: JH
authors.lastname: Berden
authors.forename: Jack F M
authors.initials: JF
authors.lastname: Wetzels
authors.forename: Lambert P W J
authors.initials: LP
authors.lastname: van den Heuvel
authors.forename: Leo A H
authors.initials: LA
authors.lastname: Monnens
authors.forename: Toin H
authors.initials: TH
authors.lastname: van Kuppevelt
mesh_terms: D000328:Adult; D000368:Aged; D000818:Animals; D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D004317:Doxorubicin / Q000494:pharmacology; D005260:Female; D005786:Gene Expression Regulation; D006497:Heparan Sulfate / Q000737:chemistry; D006801:Humans; D007668:Kidney / Q000378:metabolism / Q000473:pathology; D007678:Kidney Glomerulus / Q000378:metabolism*; D008297:Male; D008875:Middle Aged; D008954:Models, Biological; D009404:Nephrotic Syndrome / Q000378:metabolism*; D050199:Podocytes / Q000378:metabolism; D051381:Rats; D017208:Rats, Wistar
chemical_list: D004317:Doxorubicin; D006497:Heparan Sulfate