Literature DB >> 2493725

Inherited defects of renal basement membranes.

L H Noël1, M C Gubler, G Bobrie, C O Savage, C M Lockwood, J P Grünfeld.   

Abstract

For 15 to 20 years, electron microscopy studies have shown that some hereditary renal diseases are characterized by various abnormalities of the basement membranes. In the nail-patella syndrome, in Alport's syndrome and variants, and in benign familial hematuria, the changes involve the glomerular basement membrane (GBM). In nephronophthisis, the tubular basement membrane (TBM) is affected. These histopathologic features are not uniform. The progress afforded by biochemistry, immunology, and molecular biology allows us to compare these various lesions, to analyze their biochemical characteristics and their antigenicity, and to hypothesize as to the mechanisms involved.

Entities:  

Mesh:

Year:  1989        PMID: 2493725

Source DB:  PubMed          Journal:  Adv Nephrol Necker Hosp        ISSN: 0084-5957


  6 in total

Review 1.  Alport syndrome, basement membranes and collagen.

Authors:  C E Kashtan; M M Kleppel; R J Butkowski; A F Michael; A J Fish
Journal:  Pediatr Nephrol       Date:  1990-09       Impact factor: 3.714

2.  Clinical quiz. Nail-patella syndrome (NPS) (hereditary osteo-onychodysplasia).

Authors:  E M Hodson; M R Kluckow; P O'Neill
Journal:  Pediatr Nephrol       Date:  1992-05       Impact factor: 3.714

3.  Poor renal uptake of 99mtechnetium-dimercaptosuccinic acid and near-normal 99mtechnetium-mercaptoacetyltriglycine renogram in nephronophthisis.

Authors:  H Hecht; J Ohlsson; S A Starck
Journal:  Pediatr Nephrol       Date:  1996-04       Impact factor: 3.714

Review 4.  Molecular aspects of Alport's syndrome.

Authors:  M Weber; K O Netzer; O Pullig
Journal:  Clin Investig       Date:  1992-09

5.  New familial nephropathy involving glomerular and tubular basement membranes.

Authors:  M Yasui; K Narahara; M Kobayashi; K Iyoda; H Tanaka; H Makino; H Ohmori; Y Seino
Journal:  Pediatr Nephrol       Date:  1994-10       Impact factor: 3.714

6.  The development of anti-glomerular basement membrane nephritis in two children with Alport's syndrome after renal transplantation: characterization of the antibody target.

Authors:  L P vd Heuvel; C H Schröder; C O Savage; D Menzel; K J Assmann; L A Monnens; J H Veerkamp
Journal:  Pediatr Nephrol       Date:  1989-10       Impact factor: 3.714

  6 in total

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