JCB logo
Accuri Cytometers
  Home | Help | Feedback | Subscriptions | Archive | Search | Table of Contents
Published 11 October 2004. doi:10.1083/jcb.200402096
The Rockefeller University Press, 0021-9525 $8.00
JCB, Volume 167, Number 1, 149-160
This Article
Right arrow Full Text
Right arrow PDF (Full Text)
Right arrow PPT slides of all figures
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 Grossmann, K. S.
Right arrow Articles by Birchmeier, W.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Grossmann, K. S.
Right arrow Articles by Birchmeier, W.
Social Bookmarking
Add to CiteULike   Add to Complore   Add to Connotea   Add to Del.icio.us   Add to Digg   Add to Reddit   Add to Technorati  
What's this?

Article

 Requirement of plakophilin 2 for heart morphogenesis and cardiac junction formation

Katja S. Grossmann1, Christine Grund2, Joerg Huelsken1, Martin Behrend1, Bettina Erdmann1, Werner W. Franke2, and Walter Birchmeier1

1 Max Delbrueck Center for Molecular Medicine (MDC), D-13092 Berlin, Germany
2 Division of Cell Biology, German Cancer Research Center (DKFZ), D-69120 Heidelberg, Germany

Correspondence to Walter Birchmeier: wbirch{at}mdc-berlin.de

Plakophilins are proteins of the armadillo family that function in embryonic development and in the adult, and when mutated can cause disease. We have ablated the plakophilin 2 gene in mice. The resulting mutant mice exhibit lethal alterations in heart morphogenesis and stability at mid-gestation (E10.5–E11), characterized by reduced trabeculation, disarrayed cytoskeleton, ruptures of cardiac walls, and blood leakage into the pericardiac cavity. In the absence of plakophilin 2, the cytoskeletal linker protein desmoplakin dissociates from the plaques of the adhering junctions that connect the cardiomyocytes and forms granular aggregates in the cytoplasm. By contrast, embryonic epithelia show normal junctions. Thus, we conclude that plakophilin 2 is important for the assembly of junctional proteins and represents an essential morphogenic factor and architectural component of the heart.

J. Huelsken's present address is ISREC, CH-1066 Lausanne, Switzerland.

M. Behrend's present address is Franz Volhard Clinic, D-13125 Berlin, Germany.

Abbreviations used in this paper: ES, embryonic stem; IF, intermediate-sized filament; wt, wild-type.


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 Reddit Reddit   Add to Technorati Technorati    What's this?


This article has been cited by other articles:



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