Nephrin is specifically located at the slit diaphragm of glomerular podocytes
| Autor: | Ulla Lenkkeri, Hannu Jalanko, Marjo Kestilä, Päivi Ljungberg, Christer Holmberg, Karl Tryggvason, Jorma Wartiovaara, Vesa Ruotsalainen |
|---|---|
| Jazyk: | angličtina |
| Rok vydání: | 1999 |
| Předmět: |
Nephrotic Syndrome
Kidney Glomerulus Molecular Sequence Data Podocyte foot urologic and male genital diseases Polymerase Chain Reaction Podocyte Nephrin medicine Humans Cloning Molecular Microscopy Immunoelectron Finland Multidisciplinary biology urogenital system Glomerular basement membrane Cell Membrane Membrane Proteins Proteins Epithelial Cells Biological Sciences Slit Molecular biology female genital diseases and pregnancy complications Recombinant Proteins medicine.anatomical_structure Mutation Glomerular Filtration Barrier Slit diaphragm biology.protein Podocin Glomerular Filtration Rate |
| Popis: | We describe here the size and location of nephrin, the first protein to be identified at the glomerular podocyte slit diaphragm. In Western blots, nephrin antibodies generated against the two terminal extracellular Ig domains of recombinant human nephrin recognized a 180-kDa protein in lysates of human glomeruli and a 150-kDa protein in transfected COS-7 cell lysates. In immunofluorescence, antibodies to this transmembrane protein revealed reactivity in the glomerular basement membrane region, whereas the podocyte cell bodies remained negative. In immunogold-stained thin sections, nephrin label was found at the slit between podocyte foot processes. The congenital nephrotic syndrome of the Finnish type (NPHS1), a disease in which the nephrin gene is mutated, is characterized by massive proteinuria alreadyin uteroand lack of slit diaphragm and foot processes. These features, together with the now demonstrated localization of nephrin to the slit diaphragm area, suggests an essential role for this protein in the normal glomerular filtration barrier. A zipper-like model for nephrin assembly in the slit diaphragm is discussed, based on the present and previous data. |
| Databáze: | OpenAIRE |
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