Gene Symbol: Pkp2
Description: plakophilin 2
Alias: plakophilin-2
Species: rat
Products:     Pkp2

Top Publications

  1. Gallicano G, Kouklis P, Bauer C, Yin M, Vasioukhin V, Degenstein L, et al. Desmoplakin is required early in development for assembly of desmosomes and cytoskeletal linkage. J Cell Biol. 1998;143:2009-22 pubmed
  2. Hofmann I, Mertens C, Brettel M, Nimmrich V, Schnolzer M, Herrmann H. Interaction of plakophilins with desmoplakin and intermediate filament proteins: an in vitro analysis. J Cell Sci. 2000;113 ( Pt 13):2471-83 pubmed
    Plakophilin 1 and 2 (PKP1, PKP2) are members of the arm-repeat protein family...
  3. Grossmann K, Grund C, Huelsken J, Behrend M, Erdmann B, Franke W, et al. Requirement of plakophilin 2 for heart morphogenesis and cardiac junction formation. J Cell Biol. 2004;167:149-60 pubmed
    ..We have ablated the plakophilin 2 gene in mice...
  4. Gerull B, Heuser A, Wichter T, Paul M, Basson C, McDermott D, et al. Mutations in the desmosomal protein plakophilin-2 are common in arrhythmogenic right ventricular cardiomyopathy. Nat Genet. 2004;36:1162-4 pubmed
    ..In 32 of 120 unrelated individuals with ARVC, we identified heterozygous mutations in PKP2, which encodes plakophilin-2, an essential armadillo-repeat protein of the cardiac desmosome...
  5. Franke W, Borrmann C, Grund C, Pieperhoff S. The area composita of adhering junctions connecting heart muscle cells of vertebrates. I. Molecular definition in intercalated disks of cardiomyocytes by immunoelectron microscopy of desmosomal proteins. Eur J Cell Biol. 2006;85:69-82 pubmed
  6. Li M, Mruk D, Lee W, Cheng C. Connexin 43 and plakophilin-2 as a protein complex that regulates blood-testis barrier dynamics. Proc Natl Acad Sci U S A. 2009;106:10213-8 pubmed publisher
    ..43 (Cx43), a gap junction integral membrane protein, structurally interacts with desmosomal protein plakophilin-2 (PKP2), basal ES proteins N-cadherin and beta-catenin, and signaling molecule c-Src, but not with the TJ proteins ..
  7. Dubash A, Kam C, Aguado B, Patel D, Delmar M, Shea L, et al. Plakophilin-2 loss promotes TGF-β1/p38 MAPK-dependent fibrotic gene expression in cardiomyocytes. J Cell Biol. 2016;212:425-38 pubmed publisher
    ..In this study, we provide evidence that loss of the desmosomal armadillo protein Plakophilin-2 (PKP2) in cardiomyocytes elevates transforming growth factor β1 (TGF-β1) and p38 mitogen-activated protein kinase (MAPK)..
  8. Gerull B, Kirchner F, Chong J, Tagoe J, Chandrasekharan K, Strohm O, et al. Homozygous founder mutation in desmocollin-2 (DSC2) causes arrhythmogenic cardiomyopathy in the Hutterite population. Circ Cardiovasc Genet. 2013;6:327-36 pubmed publisher
    ..The truncated protein remains partially stable and localized at the intercalated discs. These data suggest that the processed DSC2 protein plays a role in maintaining desmosome integrity and function. ..
  9. van Hengel J, Calore M, Bauce B, Dazzo E, Mazzotti E, De Bortoli M, et al. Mutations in the area composita protein ?T-catenin are associated with arrhythmogenic right ventricular cardiomyopathy. Eur Heart J. 2013;34:201-10 pubmed publisher
    ..Since the frequency of CTNNA3 mutations in ARVC patients is not rare, systematic screening for this gene should be considered to improve the clinical management of ARVC families. ..

More Information


  1. Sato P, Coombs W, Lin X, Nekrasova O, Green K, Isom L, et al. Interactions between ankyrin-G, Plakophilin-2, and Connexin43 at the cardiac intercalated disc. Circ Res. 2011;109:193-201 pubmed publisher
    ..that loss of AnkG expression caused significant changes in subcellular distribution and/or abundance of PKP2 and Connexin43 as well as a decrease in intercellular adhesion strength and electric coupling...
  2. Sato P, Musa H, Coombs W, Guerrero Serna G, Patino G, Taffet S, et al. Loss of plakophilin-2 expression leads to decreased sodium current and slower conduction velocity in cultured cardiac myocytes. Circ Res. 2009;105:523-6 pubmed publisher
    Plakophilin-2 (PKP2) is an essential component of the cardiac desmosome. Recent data show that it interacts with other molecules of the intercalated disc...
  3. Oxford E, Musa H, Maass K, Coombs W, Taffet S, Delmar M. Connexin43 remodeling caused by inhibition of plakophilin-2 expression in cardiac cells. Circ Res. 2007;101:703-11 pubmed
    ..We used RNA silencing technology to decrease the expression of PKP2 in cardiac cells (ventricular myocytes, as well as epicardium-derived cells) obtained from neonatal rat hearts...
  4. Gosavi P, Kundu S, Khapare N, Sehgal L, Karkhanis M, Dalal S. E-cadherin and plakoglobin recruit plakophilin3 to the cell border to initiate desmosome assembly. Cell Mol Life Sci. 2011;68:1439-54 pubmed publisher
    ..The results reported here are consistent with the model that PG and E-cadherin recruit PKP3 to the cell border to initiate desmosome formation. ..
  5. Thomas N, Jasmin J, Lisanti M, Iacobas D. Sex differences in expression and subcellular localization of heart rhythm determinant proteins. Biochem Biophys Res Commun. 2011;406:117-22 pubmed publisher
    ..These studies extend our previous findings in microarray studies to demonstrate that sex differences in gene expression are likely to confer distinct functional properties on male and female myocardium. ..
  6. Hong M, Bao L, Kefaloyianni E, Agullo Pascual E, Chkourko H, Foster M, et al. Heterogeneity of ATP-sensitive K+ channels in cardiac myocytes: enrichment at the intercalated disk. J Biol Chem. 2012;287:41258-67 pubmed publisher
    ..Our recent proteomics experiments identified plakoglobin and plakophilin-2 (PKP2) as putative K(ATP) channel-associated proteins...
  7. Bass Zubek A, Hobbs R, Amargo E, Garcia N, Hsieh S, Chen X, et al. Plakophilin 2: a critical scaffold for PKC alpha that regulates intercellular junction assembly. J Cell Biol. 2008;181:605-13 pubmed publisher
    ..In response to cell-cell contact, PKP2 associates with DP in plaque precursors that form in the cytoplasm and translocate to nascent desmosomes...
  8. Sen Chowdhry S, Syrris P, McKenna W. Role of genetic analysis in the management of patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy. J Am Coll Cardiol. 2007;50:1813-21 pubmed
  9. Roberts J, Herkert J, Rutberg J, Nikkel S, Wiesfeld A, Dooijes D, et al. Detection of genomic deletions of PKP2 in arrhythmogenic right ventricular cardiomyopathy. Clin Genet. 2013;83:452-6 pubmed publisher
    ..We describe two cases of ARVC possessing large deletions involving plakophilin-2 (PKP2) identified with microarray analysis and/or multiplex ligation-dependent probe amplification (MLPA) that would have ..
  10. van Tintelen J, Entius M, Bhuiyan Z, Jongbloed R, Wiesfeld A, Wilde A, et al. Plakophilin-2 mutations are the major determinant of familial arrhythmogenic right ventricular dysplasia/cardiomyopathy. Circulation. 2006;113:1650-8 pubmed
    Mutations in the plakophilin-2 gene (PKP2) have been found in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVC)...
  11. Kirchner F, Schuetz A, Boldt L, Martens K, Dittmar G, Haverkamp W, et al. Molecular insights into arrhythmogenic right ventricular cardiomyopathy caused by plakophilin-2 missense mutations. Circ Cardiovasc Genet. 2012;5:400-11 pubmed publisher
    ..mainly caused by dominant mutations in several components of the cardiac desmosome including plakophilin-2 (PKP2), the most prevalent disease gene...
  12. Goossens S, Janssens B, Bonne S, De Rycke R, Braet F, van Hengel J, et al. A unique and specific interaction between alphaT-catenin and plakophilin-2 in the area composita, the mixed-type junctional structure of cardiac intercalated discs. J Cell Sci. 2007;120:2126-36 pubmed
    ..This could explain the devastating effect of plakophilin-2 mutations on cell junction stability in intercalated discs, which lead to cardiac muscle malfunction. ..
  13. Fischer Kešo R, Breuninger S, Hofmann S, Henn M, Röhrig T, Ströbel P, et al. Plakophilins 1 and 3 bind to FXR1 and thereby influence the mRNA stability of desmosomal proteins. Mol Cell Biol. 2014;34:4244-56 pubmed publisher
    ..the RNA-binding proteins FXR1, G3BP, PABPC1, and UPF1, and these PKP1/3 complexes also comprised desmoplakin and PKP2 mRNAs...
  14. Yang Z, Bowles N, Scherer S, Taylor M, Kearney D, Ge S, et al. Desmosomal dysfunction due to mutations in desmoplakin causes arrhythmogenic right ventricular dysplasia/cardiomyopathy. Circ Res. 2006;99:646-55 pubmed 6 genes, including 4 encoding desmosomal proteins (Junctional plakoglobin (JUP), Desmoplakin (DSP), Plakophilin 2, and Desmoglein 2), have been identified in patients with ARVD/C...
  15. Pieperhoff S, Schumacher H, Franke W. The area composita of adhering junctions connecting heart muscle cells of vertebrates. V. The importance of plakophilin-2 demonstrated by small interference RNA-mediated knockdown in cultured rat cardiomyocytes. Eur J Cell Biol. 2008;87:399-411 pubmed publisher
  16. Matthes S, Taffet S, Delmar M. Plakophilin-2 and the migration, differentiation and transformation of cells derived from the epicardium of neonatal rat hearts. Cell Commun Adhes. 2011;18:73-84 pubmed publisher
    ..the expression of desmosomal molecules, and the functional importance of the desmosomal protein plakophilin-2 (PKP2), in epicardial and epicardium-derived cells. Epicardial explants were obtained from neonatal rat hearts...
  17. Tang E, Xiao X, Mruk D, Qian X, Mok K, Jenardhanan P, et al. Microtubule affinity-regulating kinase 4 (MARK4) is a component of the ectoplasmic specialization in the rat testis. Spermatogenesis. 2012;2:117-126 pubmed
    ..These findings thus illustrate that the integrity of apical ES, an actin-based and testis-specific AJ, is dependent not only on the actin filament network, but also on the tubulin-based cytoskeleton. ..