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Requirement of plakophilin 2 for heart morphogenesis and cardiac junction formation

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Official URL:https://doi.org/10.1083/jcb.200402096
PubMed:View item in PubMed
Creators Name:Grossmann, K.S. and Grund, C. and Huelsken, J. and Behrend, M. and Erdmann, B. and Franke, W.W. and Birchmeier, W.
Journal Title:Journal of Cell Biology
Journal Abbreviation:J Cell Biol
Volume:167
Number:1
Page Range:149-160
Date:11 October 2004
Keywords:Alleles, Western Blotting, Genetic Crosses, Detergents, Developmental Gene Expression Regulation, Genetic Vectors, Heart, Immunohistochemistry, Transgenic Mice, Fluorescence Microscopy, Immunoelectron Microscopy, Genetic Models, Mutation, Octoxynol, Phenotype, Plakophilins, Proteins, Time Factors, Animals, Mice
Abstract: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.
ISSN:0021-9525
Publisher:Rockefeller University Press (U.S.A.)
Additional Information:Copyright (c) 2004 by The Rockefeller University Press
Item Type:Article

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