Skip Navigation

This Article
Right arrow Full Text (PDF)
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My Personal Archive
Right arrow Download to citation manager
Right arrow Search for citing articles in:
ISI Web of Science (8)
Right arrowRequest Permissions
Right arrow Disclaimer
Google Scholar
Right arrow Articles by van den Heuvel, L. P. W. J.
Right arrow Articles by Lockwood, C. M.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by van den Heuvel, L. P. W. J.
Right arrow Articles by Lockwood, C. M.
Social Bookmarking
Add to CiteULike   Add to Connotea   Add to Del.icio.us  
What's this?

Nephrol Dial Transplant (1989) 4: 770-775
© 1989 European Renal Association-European Dialysis and Transplant Association

research-article

The Glomerular Basement Membrane Defect in Alport-Type Hereditary Nephritis: Absence of Cationic Antigenic Components

L. P. W. J. van den Heuvel1,, C. O. S. Savage1, M. Wong2, R. G. Price2, L. Noel3, J. P. Grunfeld3 and C. M. Lockwood1

1Department of Medicine, Hammersmith Hospital London 2Department of Biochemistry, King's College London 3Département de Néphrologie, Hôpital Necker Paris

Correspondence and offprint requests to: Correspondence and offprint requests to: L. P. W. J. van den Heuvel, Department of Biochemistry, University of Nijmegen, P.O. Box 9101, 6500 HB Nijmegen, The Netherlands. Tel: 80-514302 or 80-514303

Alport-type hereditary nephritis is a familial disorder which results in progressive renal insufficiency and sensorineural hearing loss. It is thought to result from a biochemical defect affecting basement membranes. To study this further, non-collagenous components of type IV collagen were prepared from the glomerular basement membrane (GBM) by collagenase digestion from three male patients with hereditary nephritis. The normal Goodpasture antigenicity of the 28 and 26 kD monomers and 54 and 50 kD dimers which may be isolated from the GBM was absent on one-dimensional immunoblots. Two-dimensional electrophoresis and immunoblotting studies showed absence of Goodpasture antigenicity of these molecular weight components as well as all cationic monomeric and dimeric spots. It is concluded that the expression of the Goodpasture antigen is altered in basement membranes of hereditary nephritis patients. The altered antigenicity thus acts as a marker for the underlying abnormality.

Keywords: Alport's syndrome; Glomerular basement membrane; Goodpasture serum; Hereditary nephritis; One-and two-dimensional immunoblotting studies


Add to CiteULike CiteULike   Add to Connotea Connotea   Add to Del.icio.us Del.icio.us    What's this?




Disclaimer:
Please note that abstracts for content published before 1996 were created through digital scanning and may therefore not exactly replicate the text of the original print issues. All efforts have been made to ensure accuracy, but the Publisher will not be held responsible for any remaining inaccuracies. If you require any further clarification, please contact our Customer Services Department.