Plakophilin-2 mutations as a cause of arrhythmogenic right ventricular cardiomyopathy: Progress toward linking structural with functional changes

Heart Rhythm

Volumn 5, Issue 12, 2008, Pages 1724-1725

  Ram, Rashmi ^a   Van Wagoner, David R ^a  

^a LERNER RESEARCH INSTITUTE   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

DESMIN; DESMOCOLLIN; DESMOGLEIN; DESMOPLAKIN; GAP JUNCTION PROTEIN; PLAKOGLOBIN; PLAKOPHILIN; PLAKOPHILIN 2; UNCLASSIFIED DRUG;

ARRHYTHMOGENESIS; EDITORIAL; GAP JUNCTION; HEART CONDUCTION; HEART RIGHT VENTRICLE DYSPLASIA; HEART RIGHT VENTRICLE FUNCTION; HUMAN; MECHANICAL STRESS; MUTATION; NONHUMAN; PHENOTYPE; PRIORITY JOURNAL; PROTEIN EXPRESSION; PROTEIN PROTEIN INTERACTION;

ANIMALS; APOPTOSIS; ARRHYTHMOGENIC RIGHT VENTRICULAR DYSPLASIA; DISEASE MODELS, ANIMAL; DNA; HUMANS; MUTATION; MYOCYTES, CARDIAC; PLAKOPHILINS; RATS;

EID: 59749097968     PISSN: 15475271     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.hrthm.2008.10.040     Document Type: Editorial

References (15)

1. Duffy H.S., Delmar M., and Spray D.C. Formation of the gap junction nexus: binding partners for connexins. J Physiol Paris 96 (2002) 243-249
2. Angst B.D., Khan L.U., Severs N.J., et al. Dissociated spatial patterning of gap junctions and cell adhesion junctions during postnatal differentiation of ventricular myocardium. Circ Res 80 (1997) 88-94
3. Peters N.S., Severs N.J., Rothery S.M., et al. Spatiotemporal relation between gap junctions and fascia adherens junctions during postnatal development of human ventricular myocardium. Circ 90 (1994) 713-725
4. Calkins H., and Marcus F. Arrhythmogenic right ventricular cardiomyopathy/dysplasia: an update. Curr Cardiol Rep 10 (2008) 367-375
5. Oxford E.M., Musa H., Maass K., et al. Connexin43 remodeling caused by inhibition of plakophilin-2 expression in cardiac cells. Circ Res 101 (2007) 703-711
6. Joshi-Mukherjee R., Coombs W., Musa H., et al. Characterization of the molecular phenotype of two arrhythmogenic right ventricular cardiomyopathy (ARVC)-related plakophilin-2 (PKP2) mutations. Heart Rhythm 5 (2008) 1715-1723
7. Gutstein D.E., Liu F.Y., Meyers M.B., et al. The organization of adherens junctions and desmosomes at the cardiac intercalated disc is independent of gap junctions. J Cell Sci 116 (2003) 875-885
8. Zhurinsky J., Shtutman M., and Ben-Ze'ev A. Plakoglobin and beta-catenin: protein interactions, regulation and biological roles. J Cell Sci 113 Pt 18 (2000) 3127-3139
9. Miller J.R., Hocking A.M., Brown J.D., et al. Mechanism and function of signal transduction by the Wnt/beta-catenin and Wnt/Ca2+ pathways. Oncogene 18 (1999) 7860-7872
10. Garcia-Gras E., Lombardi R., Giocondo M.J., et al. Suppression of canonical Wnt/beta-catenin signaling by nuclear plakoglobin recapitulates phenotype of arrhythmogenic right ventricular cardiomyopathy. J Clin Invest 116 (2006) 2012-2021
11. Ai Z., Fischer A., Spray D.C., et al. Wnt-1 regulation of connexin43 in cardiac myocytes. J Clin Invest 105 (2000) 161-171
12. Ross S.E., Hemati N., Longo K.A., et al. Inhibition of adipogenesis by Wnt signaling. Science 289 (2000) 950-953
13. Hakimelahi S., Parker H.R., Gilchrist A.J., et al. Plakoglobin regulates the expression of the anti-apoptotic protein BCL-2. J Biol Chem 275 (2000) 10905-10911
14. Longo K.A., Kennell J.A., Ochocinska M.J., et al. Wnt signaling protects 3T3-L1 preadipocytes from apoptosis through induction of insulin-like growth factors. J Biol Chem 277 (2002) 38239-38244
15. Rodriguez-Sinovas A., Boengler K., Cabestrero A., et al. Translocation of connexin 43 to the inner mitochondrial membrane of cardiomyocytes through the heat shock protein 90-dependent TOM pathway and its importance for cardioprotection. Circ Res 99 (2006) 93-101