title: Expression of nephrin in acquired human glomerular disease.
abstract: BACKGROUND
  Nephrin is a recently identified protein, which is synthesized in the podocytes and localized in the slit diaphragm area. Nephrin is a cell adhesion molecule of the immunoglobulin superfamily, and presumably is a part of the zipper-like structure of the slit membrane. As the mutation of the gene coding nephrin induces congenital nephrotic syndrome of Finnish type, which is a prototype of nephrotic syndrome, it has been suggested that nephrin also plays a role in acquired proteinuric kidney disease.
  
  
  METHODS
  To address the above issue, the expression of nephrin in acquired human glomerular disease was studied by immunoelectron microscopy employing a polyclonal antibody against nephrin. Four normal human kidneys from nephrectomy specimens and eight kidney biopsy specimens from glomerular disease patients (one minimal change disease, one membranous glomerulonephritis (GN), one membranoproliferative GN, four IgA nephropathy, and one lupus nephritis) were studied. Proteinuria of the patients ranged from 448 to 11725 mg/day. Effacement of the foot processes was observed in all patients.
  
  
  RESULTS
  The study demonstrated that the number and distribution of gold particles in the glomerular region, where the podocyte foot process was well preserved, were similar to that found in normal kidneys; however, gold particles were almost always absent in regions where the foot processes were effaced. The number of gold particles per foot process interspace was not different between normal controls and GN patients; however, the number of gold particles per defined length (1000 nm) of the glomerular basement membrane underlying the foot processes was significantly reduced in GN patients.
  
  
  CONCLUSIONS
  Using immunoelectron microscopy, we observed that the expression of nephrin in GN was lower in regions where the foot processes were effaced, and comparable with that of normal controls where the foot process interspaces were preserved. The significance of our observation in the context of proteinuria in acquired GN needs further clarification.
authors.affiliation: Division of Nephrology, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
authors.forename: Wooseong
authors.initials: W
authors.lastname: Huh
authors.forename: Dae Joong
authors.initials: DJ
authors.lastname: Kim
authors.forename: Mi-Kyung
authors.initials: MK
authors.lastname: Kim
authors.forename: Yoon Goo
authors.initials: YG
authors.lastname: Kim
authors.forename: Ha-Young
authors.initials: HY
authors.lastname: Oh
authors.forename: Vesa
authors.initials: V
authors.lastname: Ruotsalainen
authors.forename: Karl
authors.initials: K
authors.lastname: Tryggvason
mesh_terms: D000328:Adult; D016022:Case-Control Studies; D005260:Female; D015870:Gene Expression; D005921:Glomerulonephritis / Q000235:genetics / Q000378:metabolism* / Q000473:pathology; D005922:Glomerulonephritis, IGA / Q000378:metabolism / Q000473:pathology; D015432:Glomerulonephritis, Membranoproliferative / Q000378:metabolism / Q000473:pathology; D015433:Glomerulonephritis, Membranous / Q000378:metabolism / Q000473:pathology; D006801:Humans; D007678:Kidney Glomerulus / Q000378:metabolism / Q000648:ultrastructure; D008181:Lupus Nephritis / Q000378:metabolism / Q000473:pathology; D008297:Male; D008565:Membrane Proteins; D008856:Microscopy, Fluorescence; D016253:Microscopy, Immunoelectron; D008875:Middle Aged; D009402:Nephrosis, Lipoid / Q000378:metabolism / Q000473:pathology; D011506:Proteins / Q000235:genetics / Q000378:metabolism*
chemical_list: D008565:Membrane Proteins; D011506:Proteins; C113267:nephrin