Literature DB >> 14566460

[Pathogenetic aspectics of nephrotic syndrome].

G Walz1.   

Abstract

Nephrotic syndrome is characterized by protein loss in the urine, hypoalbuminemia, hyperlipidemia and edema. Several diseases cause a nephrotic syndrome, as they damage the glomerular podocytes. These specialized epithelial cells, together with endothelial cells of the glomerular capillaries and the basal membrane, form a filter that retains plasma proteins in the circulation. A disturbance of this filter causes proteinuria. The three most common primary glomerular diseases are minimal change, membranous glomerulonephritis, and the primary focal segmental glomerulosclerosis. The familiar forms are rare; however, the identification of the relevant gene defects has greatly advanced our understanding of podocyte function as well as the pathogenesis of nephrotic syndrome.

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Year:  2003        PMID: 14566460     DOI: 10.1007/s00108-003-1031-4

Source DB:  PubMed          Journal:  Internist (Berl)        ISSN: 0020-9554            Impact factor:   0.743


  23 in total

1.  Elevated frequency and allelic heterogeneity of congenital nephrotic syndrome, Finnish type, in the old order Mennonites.

Authors:  S Bolk; E G Puffenberger; J Hudson; D H Morton; A Chakravarti
Journal:  Am J Hum Genet       Date:  1999-12       Impact factor: 11.025

2.  Mutations in ACTN4, encoding alpha-actinin-4, cause familial focal segmental glomerulosclerosis.

Authors:  J M Kaplan; S H Kim; K N North; H Rennke; L A Correia; H Q Tong; B J Mathis; J C Rodríguez-Pérez; P G Allen; A H Beggs; M R Pollak
Journal:  Nat Genet       Date:  2000-03       Impact factor: 38.330

3.  NPHS2, encoding the glomerular protein podocin, is mutated in autosomal recessive steroid-resistant nephrotic syndrome.

Authors:  N Boute; O Gribouval; S Roselli; F Benessy; H Lee; A Fuchshuber; K Dahan; M C Gubler; P Niaudet; C Antignac
Journal:  Nat Genet       Date:  2000-04       Impact factor: 38.330

4.  Proteinuria and perinatal lethality in mice lacking NEPH1, a novel protein with homology to NEPHRIN.

Authors:  D B Donoviel; D D Freed; H Vogel; D G Potter; E Hawkins; J P Barrish; B N Mathur; C A Turner; R Geske; C A Montgomery; M Starbuck; M Brandt; A Gupta; R Ramirez-Solis; B P Zambrowicz; D R Powell
Journal:  Mol Cell Biol       Date:  2001-07       Impact factor: 4.272

5.  Mutation spectrum in the nephrin gene (NPHS1) in congenital nephrotic syndrome.

Authors:  O Beltcheva; P Martin; U Lenkkeri; K Tryggvason
Journal:  Hum Mutat       Date:  2001-05       Impact factor: 4.878

6.  Podocin, a raft-associated component of the glomerular slit diaphragm, interacts with CD2AP and nephrin.

Authors:  K Schwarz; M Simons; J Reiser; M A Saleem; C Faul; W Kriz; A S Shaw; L B Holzman; P Mundel
Journal:  J Clin Invest       Date:  2001-12       Impact factor: 14.808

7.  Interaction with podocin facilitates nephrin signaling.

Authors:  T B Huber; M Kottgen; B Schilling; G Walz; T Benzing
Journal:  J Biol Chem       Date:  2001-09-18       Impact factor: 5.157

8.  Association of parvovirus B19 infection with idiopathic collapsing glomerulopathy.

Authors:  A Moudgil; C C Nast; A Bagga; L Wei; A Nurmamet; A H Cohen; S C Jordan; M Toyoda
Journal:  Kidney Int       Date:  2001-06       Impact factor: 10.612

9.  Cbl-CIN85-endophilin complex mediates ligand-induced downregulation of EGF receptors.

Authors:  Philippe Soubeyran; Katarzyna Kowanetz; Iwona Szymkiewicz; Wallace Y Langdon; Ivan Dikic
Journal:  Nature       Date:  2002-03-14       Impact factor: 49.962

10.  Positionally cloned gene for a novel glomerular protein--nephrin--is mutated in congenital nephrotic syndrome.

Authors:  M Kestilä; U Lenkkeri; M Männikkö; J Lamerdin; P McCready; H Putaala; V Ruotsalainen; T Morita; M Nissinen; R Herva; C E Kashtan; L Peltonen; C Holmberg; A Olsen; K Tryggvason
Journal:  Mol Cell       Date:  1998-03       Impact factor: 17.970

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