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

Détails

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Etat: Public
Version: Final published version
Licence: CC BY-NC-SA 4.0
ID Serval
serval:BIB_31665
Type
Article: article d'un périodique ou d'un magazine.
Collection
Publications
Institution
Titre
Requirement of plakophilin 2 for heart morphogenesis and cardiac junction formation.
Périodique
Journal of Cell Biology
Auteur(s)
Grossmann K.S., Grund C., Huelsken J., Behrend M., Erdmann B., Franke W.W., Birchmeier W.
ISSN
0021-9525
Statut éditorial
Publié
Date de publication
2004
Volume
167
Numéro
1
Pages
149-160
Langue
anglais
Notes
Publication types: Journal Article
Résumé
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.
Mots-clé
Alleles, Animals, Blotting, Western, Crosses, Genetic, Detergents/pharmacology, Gene Expression Regulation, Developmental, Genetic Vectors, Heart/embryology, Heart/physiology, Immunohistochemistry, Mice, Mice, Transgenic, Microscopy, Fluorescence, Microscopy, Immunoelectron, Models, Genetic, Mutation, Octoxynol/pharmacology, Phenotype, Plakophilins, Proteins/genetics, Proteins/physiology, Time Factors
Pubmed
Web of science
Open Access
Oui
Création de la notice
19/11/2007 13:30
Dernière modification de la notice
20/08/2019 14:16
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