Document Detail


Altered ultrastructural distribution of nephrin in minimal change nephrotic syndrome.
MedLine Citation:
PMID:  12480962     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
BACKGROUND: Nephrin is a cell-adhesion protein that is defective in congenital nephrotic syndrome of the Finnish type (CNF). Nephrin is synthesized by the podocytes and is localized to the slit membrane between individual foot processes of the podocytes. Although nephrin is apparently pivotal in the development of CNF, the role of nephrin in other causes of nephrotic syndrome is not fully understood. METHODS: Renal biopsy specimens from patients with minimal change nephrotic syndrome (MCNS) were investigated. Nephrin distribution was studied with immunohistochemical and semiquantitative immunoelectron microscopic techniques and the results were related to the degree of foot process effacement found under the electron microscope. RESULTS: In normal kidney, immunofluorescence revealed a linear staining along the capillary basement membranes, corresponding to the localization of nephrin in the slit membranes. In the biopsies from patients with MCNS, the nephrin pattern had become granular. The degree of granularization corresponded to the degree of foot process effacement. Ultrastructural semiquantification showed the amount of nephrin to be reduced both in areas with and without foot process effacement compared with the control specimens. The concentration of nephrin was lowest in the areas with foot process effacement and there was redistribution from the slits into the cytoplasm. CONCLUSIONS: These findings demonstrate that nephrin expression is altered in MCNS. Whether this reflects a pathogenetic role for nephrin in MCNS or a phenomenon secondary to other causes of foot process effacement remains to be elucidated.
Authors:
Annika Wernerson; Fredrik Dunér; Erna Pettersson; Silwa Mengarelli Widholm; Ulla Berg; Vesa Ruotsalainen; Karl Tryggvason; Kjell Hultenby; Magnus Söderberg
Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association     Volume:  18     ISSN:  0931-0509     ISO Abbreviation:  Nephrol. Dial. Transplant.     Publication Date:  2003 Jan 
Date Detail:
Created Date:  2002-12-13     Completed Date:  2003-07-07     Revised Date:  2007-11-14    
Medline Journal Info:
Nlm Unique ID:  8706402     Medline TA:  Nephrol Dial Transplant     Country:  England    
Other Details:
Languages:  eng     Pagination:  70-6     Citation Subset:  IM    
Affiliation:
Department of Pathology, Karolinska Institutet, Huddinge University Hospital, Sweden. Annika.Wernerson@pathlab.hs.sll.se
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MeSH Terms
Descriptor/Qualifier:
Adult
Aged
Child
Child, Preschool
Humans
Kidney Glomerulus / pathology*,  ultrastructure
Membrane Proteins
Microscopy, Immunoelectron
Middle Aged
Nephrotic Syndrome / pathology*
Proteins / analysis*
Grant Support
ID/Acronym/Agency:
DK-54724/DK/NIDDK NIH HHS
Chemical
Reg. No./Substance:
0/Membrane Proteins; 0/Proteins; 0/nephrin

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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