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Targeted mutation of plakoglobin in mice reveals essential functions of desmosomes in the embryonic heart

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Item Type:Article
Title:Targeted mutation of plakoglobin in mice reveals essential functions of desmosomes in the embryonic heart
Creators Name:Ruiz, P. and Brinkmann, V. and Ledermann, B. and Behrend, M. and Grund, C. and Thalhammer, C. and Vogel, F. and Birchmeier, C. and Guenthert, U. and Franke, W.W. and Birchmeier, W.
Abstract:Plakoglobin (gamma-catenin), a member of the armadillo family of proteins, is a constituent of the cytoplasmic plaque of desmosomes as well as of other adhering cell junctions, and is involved in anchorage of cytoskeletal filaments to specific cadherins. We have generated a null mutation of the plakoglobin gene in mice. Homozygous -/- mutant animals die between days 12-16 of embryogenesis due to defects in heart function. Often, heart ventricles burst and blood floods the pericard. This tissue instability correlates with the absence of desmosomes in heart, but not in epithelia organs. Instead, extended adherens junctions are formed in the heart, which contain desmosomal proteins, i.e., desmoplakin. Thus, plakoglobin is an essential component of myocardiac desmosomes and seems to play a crucial role in the sorting out of desmosomal and adherens junction components, and consequently in the architecture of intercalated discs and the stabilization of heart tissue.
Keywords:beta Catenin, Cadherins, Cell Adhesion Molecules, Cytoskeletal Proteins, Desmoplakins, Desmosomes, Embryonic and Fetal Development, Epithelial Cells, Epithelium, gamma Catenin, Genetic Vectors, Heart, Intercellular Junctions, Messenger RNA, Mutation, Myocardium, Small Intestine, Stem Cells, Trans-Activators, Animals, Mice
Source:Journal of Cell Biology
Publisher:Rockefeller University Press
Page Range:215-225
Date:October 1996
Additional Information:Copyright (c) 1996 by The Rockefeller University Press
Official Publication:http://www.jcb.org/cgi/content/abstract/135/1/215
PubMed:View item in PubMed

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