plakophilin-2 (PKP2)

Function: - may play a role in junctional plaques - phosphorylated upon DNA damage, probably by ATM or ATR Structure: - belongs to the beta-catenin family - contains 8 ARM repeats Compartment: - nucleus, cell junction, desmosome - nuclear & associated with desmosomes Alternative splicing: named isoforms=2 Expression: - widely expressed - found at desmosomal plaques in simple & stratified epithelia & in non-epithelial tissues, including myocardium & lymph node follicles - in most stratified epithelia found in the desmosomes of the basal cell layer - seems to be absent from suprabasal strata of epithelia Pathology: - defects in PKP2 are the cause of familial arrhythmogenic right ventricular dysplasia type 9

General

phosphoprotein plakophilin

Properties

SIZE: entity length = 881 aa MW = 97 kD COMPARTMENT: cell nucleus MOTIF: Tyr phosphorylation site {Y12} Tyr phosphorylation site {Y86} Ser phosphorylation site {S132} Ser phosphorylation site {S151} Ser phosphorylation site {S154} Ser phosphorylation site {S155} Tyr phosphorylation site {Y161} Tyr phosphorylation site {Y166} Tyr phosphorylation site {Y168} Ser phosphorylation site {S169} Ser phosphorylation site {S197} Tyr phosphorylation site {Y217} Ser phosphorylation site {S329} ARM repeat {341-383} ARM repeat {385-424} ARM repeat {427-467} ARM repeat {571-616} ARM repeat {671-711} ARM repeat {719-758} ARM repeat {763-804} ARM repeat {807-849}

Database Correlations

OMIM correlations MORBIDMAP 602861 UniProt Q99959 Pfam PF00514 Entrez Gene 5318