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 ?
Regulation of endothelial barrier function and growth by the armadillo family proteins plakoglobin and beta-catenin.
Suppression of canonical Wnt/beta-catenin signaling by nuclear plakoglobin recapitulates phenotype of arrhythmogenic right ventricular cardiomyopathy.
Usefulness of immunostaining for plakoglobin as a diagnostic marker of arrhythmogenic right ventricular cardiomyopathy.
Identification of a deletion in plakoglobin in arrhthmogenic right ventricular cardiomyopathy with palmoplanter keratoderma and woolly hair (Naxos disease).
For example, plakoglobin, when freed from desmosomal complexes, translocates to the nucleus where it competes and opposes the action of [beta]-catenin and downregulates the canonical Wnt/-[beta]-catenin signaling pathway.
Mutations at the inner dense plaque, particularly affecting the desmin-binding site of desmoplakin may result in ARVD/C with predominantly left ventricular involvement and clinical overlapping with dilated cardiomyopathy, while the mutations affecting the outer dense plaque of desmosome (desmoglein2, plakoglobin, plakophilin2 and the N-terminal of desmoplakin) result in ARVD/C with the ordinary described phenotype indicating that mutations in the same gene at different locations might lead to different phenotypes either ARVD/C or ALVC (50).
Defining E-cadherin-associated protein complexes in epithelial cells: plakoglobin, [beta]- and [alpha]-catenin are distinct components.
Plakoglobin domains that define its association with the desmosomal cadherins and the classical cadherins: identification of unique and shared domains.
Co-immunoprecipitation of myc-tagged proteins from L cell lines revealed that plakoglobin binds with an unusual 6:1 stoichiometry to desmoglein, whereas it binds with a 1:1 stoichiometry to desmocollin (Kowalczyk et al.
p120, a novel substrate of protein tyrosine kinase receptors and of p60v-src, is related to cadherin-binding factors beta-catenin, plakoglobin and armadillo.
McKay et al[53] report a homozygous 2-bp deletion in the plakoglobin gene in 19 individuals with Naxos disease or autosomal recessive arrhythmogenic right ventricular cardiomyopathy.
Pemphigus vulgaris is an autoimmune skin disease characterized by acantholytic skin lesions with autoantibodies directed against desmoglen, plakoglobins, and cadherin polypeptides of desmosomes and other cell-cell adhesion molecules.