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PMID: 8858175 Published · ppublish English Journal Article Research Support, Non-U.S. Gov't

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

The Journal of cell biology ·Vol. 135 ·No. 1 ·1996-10-00 ·Pages 215-25

Ruiz P, Brinkmann V, Ledermann B, Behrend M, Grund C, Thalhammer C, Vogel F, Birchmeier C, Günthert U, Franke WW, 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.

MeSH Terms
Animals Cadherins Cell Adhesion Molecules/analysis Cytoskeletal Proteins/analysis,genetics,physiology Desmoplakins Desmosomes/chemistry,physiology,ultrastructure Embryonic and Fetal Development Epithelial Cells Epithelium/chemistry Genetic Vectors/genetics Heart/embryology,physiology Intercellular Junctions/chemistry Intestine, Small/chemistry,cytology,ultrastructure Mice Mice, Inbred C57BL Mice, Mutant Strains Mutation Myocardium/chemistry,cytology RNA, Messenger/analysis Stem Cells Trans-Activators beta Catenin gamma Catenin
Chemicals
CTNNB1 protein, mouse Cadherins Cdh2 protein, mouse Cell Adhesion Molecules Cytoskeletal Proteins Desmoplakins Dsp protein, mouse Jup protein, mouse RNA, Messenger Trans-Activators beta Catenin gamma Catenin
Authors & Affiliations
11 authors, click to expand affiliations / ORCID
Ruiz P
Max-Delbruck-Center for Molecular Medicine, Berlin, Germany.
Brinkmann V
Ledermann B
Behrend M
Grund C
Thalhammer C
Vogel F
Birchmeier C
Günthert U
Franke W W
Birchmeier W
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Article Info
Journal
The Journal of cell biology
Abbr.
J Cell Biol
ISSN
0021-9525
DOI
10.1083/jcb.135.1.215
Published
1996-10-00
Pages
215-25
Language
English
Region
United States
NLM ID
0375356
PMCID
PMC2121015
Subset
IM
External Links
PubMed source DOI: 10.1083/jcb.135.1.215 PMC Full Text View journal details
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