JCB logo
Avanti Polar Lipids, Inc.
  Home | Help | Feedback | Subscriptions | Archive | Search | Table of Contents
Published online 2 October 2006. doi:10.1083/jcb.200603073
The Rockefeller University Press, 0021-9525 $8.00
JCB, Volume 175, Number 1, 33-39
This Article
Right arrow Full Text
Right arrow Full Text (PDF, 3627K)
Right arrow PPT slides of all figures
Right arrow Supplemental Material Index
Right arrow Alert me when this article is cited
Right arrow Citation Map
Services
Right arrow Email this article
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new content in the JCB
Right arrow Download to citation manager
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via CrossRef
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Sachs, N.
Right arrow Articles by Sonnenberg, A.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Sachs, N.
Right arrow Articles by Sonnenberg, A.
Social Bookmarking
Add to CiteULike   Add to Complore   Add to Connotea   Add to Del.icio.us   Add to Digg   Add to Facebook   Add to Reddit   Add to Technorati   Add to Twitter  
What's this?

Report

Kidney failure in mice lacking the tetraspanin CD151



Norman Sachs1, Maaike Kreft1, Marius A. van den Bergh Weerman2, Andy J. Beynon3, Theo A. Peters3, Jan J. Weening2, and Arnoud Sonnenberg1

1 Division of Cell Biology, The Netherlands Cancer Institute, 1066 CX Amsterdam, Netherlands
2 Department of Pathology, Academic Medical Center, University of Amsterdam, 1105 AZ Amsterdam, Netherlands
3 Department of Otorhinolaryngology, Radboud University Nijmegen Medical Center, 6500 HC Nijmegen, Netherlands

Correspondence to Arnoud Sonnenberg: a.sonnenberg{at}nki.nl

The tetraspanin CD151 is a cell-surface molecule known for its strong lateral interaction with the laminin-binding integrin {alpha}3ß1. Patients with a nonsense mutation in CD151 display end-stage kidney failure associated with regional skin blistering and sensorineural deafness, and mice lacking the integrin {alpha}3 subunit die neonatally because of severe abnormalities in the lung and kidney epithelia. We report the generation of Cd151-null mice that recapitulate the renal pathology of human patients, i.e., with age they develop massive proteinuria caused by focal glomerulosclerosis, disorganization of the glomerular basement membrane, and tubular cystic dilation. However, neither skin integrity nor hearing ability are impaired in the Cd151-null mice. Furthermore, we generated podocyte-specific conditional knockout mice for the integrin {alpha}3 subunit that show renal defects similar to those in the Cd151 knockout mice. Our results support the hypothesis that CD151 plays a key role in strengthening {alpha}3ß1-mediated adhesion in podocytes.

N. Sachs and M. Kreft contributed equally to this paper.

Abbreviations used in this paper: ABR, auditory brainstem response; FP, foot process; GBM, glomerular basement membrane; GESD, glomerular epithelial slit diaphragm; pAb, polyclonal antibody.


Add to CiteULike CiteULike   Add to Complore Complore   Add to Connotea Connotea   Add to Del.icio.us Del.icio.us   Add to Digg Digg   Add to Facebook Facebook   Add to Reddit Reddit   Add to Technorati Technorati   Add to Twitter Twitter    What's this?


This article has been cited by other articles:



  Home | Help | Feedback | Subscriptions | Archive | Search | Table of Contents