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

Ruiz, P. and Brinkmann, V. and Ledermann, B. and Behrend, M. and Grund, C. and Thalhammer, C. and Vogel, F. and Birchmeier, C. and Günthert, U. and Franke, W. W. and Birchmeier, W.. (1996) Targeted mutation of plakoglobin in mice reveals essential functions of desmosomes in the embryonic heart. The Journal of cell biology, Vol. 135, H. 1. pp. 215-225.

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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.
Faculties and Departments:03 Faculty of Medicine > Bereich Querschnittsfächer (Klinik) > Pathologie USB > Neuro- und Muskelpathologie (Frank)
03 Faculty of Medicine > Departement Klinische Forschung > Bereich Querschnittsfächer (Klinik) > Pathologie USB > Neuro- und Muskelpathologie (Frank)
UniBasel Contributors:Günthert, Ursula
Item Type:Article, refereed
Article Subtype:Research Article
Publisher:Rockefeller University Press
ISSN:0021-9525
Note:Publication type according to Uni Basel Research Database: Journal article
Language:English
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Last Modified:31 Dec 2015 10:44
Deposited On:22 Mar 2012 13:35

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