plakoglobin


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plakoglobin

A protein encoded by JUP on chromosome 17q21, which is the core molecule in the submembranous plaques of desmosomes and intermediate junctions, and forms complexes with cadherins. It is a member of the catenin protein family as it contains a so-called armadillo repeat.

Interactions
Beta-catenin, MUC1, CDH1, CDH2, CDH3, E-cadherin, desmoglein 2, desmoplakin.

Abnormal in 
JUP mutations are associated with arrhythmogenic right ventricular cardiomyopathy and Naxos syndrome.
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References in periodicals archive ?
VE-cadherin and the armadillo family protein plakoglobin regulate vascular endothelial permeability and growth.
Naxos syndrome is correlated with a homozygous deletion of two base pairs found in the plakoglobin gene located in the 17q21.2 locus (Table 1) [11, 23].
Ben-Zeev, "Differential mechanisms of LEF/TCF family-dependent transcriptional activation by [beta]-catenin and plakoglobin," Molecular and Cellular Biology, vol.
Usefulness of immunostaining for plakoglobin as a diagnostic marker of arrhythmogenic right ventricular cardiomyopathy.
The plakoglobin, desmoplakin and desmocollin-2 are family members of desmosomal proteins that function as cell-cell adhesion junction, and are expressed in skin, heart and hair.5
The probable molecular mechanism hypothesized was that by mutating desmosomal protein impairs desmosomal assembly releasing free plakoglobin from the desmosomes, which could translocate into the nucleus and through competition with [beta]-catenin suppress signaling through the canonical Wnt/ [beta]-catenin-Tcf/Lef pathway.
Whereas the cadherins in most AJs are linked to the actin cytoskeleton through [alpha]-and [beta]-catenin, desmosomal cadherins recruit the armadillo proteins plakoglobin and plakophilin-1, which are vertebrate-specific gene duplications related to [beta]-catenin and are linked to the intermediate filament cytoskeleton either directly or via the linker protein desmoplakin (Schneider et al., 2003).
Gumbiner, "A homolog of the armadillo protein in Drosophila (plakoglobin) associated with E-cadherin," Science, vol.
Differential nuclear translocation and transactivation potential of beta-catenin and plakoglobin. J Cell Biol.
In mouse models, knockdown of plakoglobin abrogates CTC cluster formation and suppresses lung metastases (18).
They went on to demonstrate that overexpression of DSP led to increased plakoglobin ([gamma]-catenin).