• Register
X
Forgot Password

If you have forgotten your password you can enter your email here and get a temporary password sent to your email.

X

Leaving Community

Are you sure you want to leave this community? Leaving the community will revoke any permissions you have been granted in this community.

No
Yes

Requirement of plakophilin 2 for heart morphogenesis and cardiac junction formation.

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.

Pubmed ID: 15479741

Authors

  • Grossmann KS
  • Grund C
  • Huelsken J
  • Behrend M
  • Erdmann B
  • Franke WW
  • Birchmeier W

Journal

The Journal of cell biology

Publication Data

October 11, 2004

Associated Grants

None

Mesh Terms

  • Alleles
  • Animals
  • Blotting, Western
  • Crosses, Genetic
  • Detergents
  • Gene Expression Regulation, Developmental
  • Genetic Vectors
  • Heart
  • Immunohistochemistry
  • Mice
  • Mice, Transgenic
  • Microscopy, Fluorescence
  • Microscopy, Immunoelectron
  • Models, Genetic
  • Mutation
  • Octoxynol
  • Phenotype
  • Plakophilins
  • Proteins
  • Time Factors