Molecular composition of the intercalated disc in a spontaneous canine animal model of arrhythmogenic right ventricular dysplasia/cardiomyopathy

Heart Rhythm

Volumn 4, Issue 9, 2007, Pages 1196-1205

  Oxford, Eva M ^a   Everitt, Melanie ^b   Coombs, Wanda ^a   Fox, Philip R ^c   Kraus, Marc ^d   Gelzer, Anna R M ^d   Saffitz, Jeffrey ^e   Taffet, Steven M ^a   Moïse, N Sydney ^d   Delmar, Mario ^a  

^a State University of New York Upstate Medical University: College of Medicine   (United States)

^b UNIVERSITY OF UTAH SCHOOL OF MEDICINE   (United States)

^c ANIMAL MEDICAL CENTER   (United States)

^d Department of Obstetrics Gynecology   (United States)

^e HARVARD MEDICAL SCHOOL   (United States)

Author keywords

ARVD C; CX43; Inherited cardiomyopathy; Intercalated disc

Indexed keywords

CONNEXIN 43; DESMIN; DESMOPLAKIN; NERVE CELL ADHESION MOLECULE; PLAKOGLOBIN; PLAKOPHILIN; PLAKOPHILIN 2; UNCLASSIFIED DRUG;

ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; CELL COMMUNICATION; CELL INTERACTION; CHEMICAL STRUCTURE; CONTROLLED STUDY; DOG; FEMALE; GAP JUNCTION; GENE MUTATION; GENE SEQUENCE; HEART RIGHT VENTRICLE DYSPLASIA; HEART VENTRICLE ARRHYTHMIA; HISTOPATHOLOGY; IMMUNOFLUORESCENCE MICROSCOPY; IMMUNOLOCALIZATION; INTERCALATION COMPLEX; MALE; NONHUMAN; PRIORITY JOURNAL; SEQUENCE ANALYSIS; SIGNAL TRANSDUCTION; WESTERN BLOTTING;

ANIMALS; ARRHYTHMOGENIC RIGHT VENTRICULAR DYSPLASIA; BLOTTING, WESTERN; CADHERINS; CONNEXIN 43; CYTOSKELETAL PROTEINS; DISEASE MODELS, ANIMAL; DOGS; FEMALE; GAP JUNCTIONS; MALE; MUTATION; MYOCYTES, CARDIAC; SEQUENCE ANALYSIS, DNA;

EID: 34548253988     PISSN: 15475271     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.hrthm.2007.05.025     Document Type: Article

References (36)

1. Corrado D., Basso C., Thiene G., McKenna W.J., Davies M.J., Fontaliran F., Nava A., Silvestri F., Blomstrom-Lundqvist C., Wlodarska E.K., Fontaine G., and Camerini F. Spectrum of clinicopathologic manifestations of arrhythmogenic right ventricular cardiomyopathy/dysplasia: a multicenter study. J Am Coll Cardiol 30 (1997) 1512-1520
2. Thiene G., Nava A., Corrado D., Rossi L., and Pennelli N. Right ventricular cardiomyopathy and sudden death in young people. N Engl J Med 318 (1988) 129-133
3. Maron B.J., Shirani J., Poliac L.C., Mathenge R., Roberts W.C., and Mueller F.O. Sudden death in young competitive athletes. Clinical, demographic, and pathological profiles. JAMA 276 (1996) 199-204
4. Tabib A., Loire R., Chalabreysse L., Meyronnet D., Miras A., Malicier D., Thivolet F., Chevalier P., and Bouvagnet P. Circumstances of death and gross and microscopic observations in a series of 200 cases of sudden death associated with arrhythmogenic right ventricular cardiomyopathy and/or dysplasia. Circulation 108 (2003) 3000-3005
5. Sen-Chowdhry S., Syrris P., and McKenna W.J. Genetics of right ventricular cardiomyopathy. J Cardiovasc Electrophysiol 16 (2005) 927-935
6. Hatsell S., and Cowin P. Deconstructing desmoplakin. Nat Cell Biol 3 (2001) E270-E272
7. Kaplan S.R., Gard J.J., Carvajal-Huerta L., Ruiz-Cabezas J.C., Thiene G., and Saffitz J.E. Structural and molecular pathology of the heart in carvajal syndrome. Cardiovasc Pathol 13 (2004) 26-32
8. Kaplan S.R., Gard J.J., Protonotarios N., Tsatsopoulou A., Spiliopoulou C., Anastasakis A., Squarcioni C.P., McKenna W.J., Thiene G., Basso C., Brousse N., Fontaine G., and Saffitz J.E. Remodeling of myocyte gap junctions in arrhythmogenic right ventricular cardiomyopathy due to a deletion in plakoglobin (Naxos disease). Heart Rhythm 1 (2004) 3-11
9. Basso C., Fox P.R., Meurs K.M., Towbin J.A., Spier A.W., Calabrese F., Maron B.J., and Thiene G. Arrhythmogenic right ventricular cardiomyopathy causing sudden cardiac death in boxer dogs: A new animal model of human disease. Circulation 109 (2004) 1180-1185
10. Fox P.R., Maron B.J., Basso C., Liu S.K., and Thiene G. Spontaneously occurring arrhythmogenic right ventricular cardiomyopathy in the domestic cat: a new animal model similar to the human disease. Circulation 102 (2000) 1863-1870
11. Kraus M.S., Moise N.S., Rishniw M., Dykes N., and Erb H.N. Morphology of ventricular arrhythmias in the boxer as measured by 12-lead electrocardiography with pace-mapping comparison. J Vet Intern Med 16 (2002) 153-158
12. Fontaine G., Fontaliran F., and Frank R. Arrhythmogenic right ventricular cardiomyopathies: clinical forms and main differential diagnoses. Circulation 97 (1998) 1532-1535
13. Basso C., Thiene G., Corrado D., Angelini A., Nava A., and Valente M. Arrhythmogenic right ventricular cardiomyopathy. Dysplasia, dystrophy, or myocarditis?. Circulation 94 (1996) 983-991
14. Li W.E., and Nagy J.I. Connexin 43 phosphorylation state and intercellular communication in cultured astrocytes following hypoxia and protein phosphatase inhibition. Eur J Neurosci 12 (2000) 2644-2650
15. Lehnart S.E., Wehrens X.H., Kushnir A., and Marks A.R. Cardiac ryanodine receptor function and regulation in heart disease. Ann N Y Acad Sci 1015 (2004) 144-159
16. Marx S.O., Reiken S., Hisamatsu Y., Jayaraman T., Burkhoff D., Rosemblit N., and Marks A.R. PKA phosphorylation dissociates FKBP12.6 from the calcium release channel (ryanodine receptor): defective regulation in failing hearts. Cell 101 (2000) 365-376
17. Saffitz J.E. Dependence of electrical coupling on mechanical coupling in cardiac myocytes. In: Thiene G., and Pessina A.C. (Eds). Advances in cardiovascular medicine (2003), Universita degli Studi di Padova, Padova, Italy 15-28
18. Giepmans B.N., and Moolenaar W.H. The gap junction protein connexin 43 interacts with the second PDZ domain of the zona occludens-1 protein. Curr Biol 8 (1998) 931-934
19. Toyofuku T., Yabuki M., Otsu K., Kuzuya T., Hori M., and Tada M. Direct association of the gap junction protein connexin-43 with ZO-1 in cardiac myocytes. J Biol Chem 273 (1998) 12725-12731
20. Hunter A.W., Barker R.J., Zhu C., and Gourdie R.G. Zonula occludens-1 alters connexin 43 gap junction size and organization by influencing channel accretion. Mol Biol Cell 16 (2005) 5686-5698
21. Ai Z., Fischer A., Spray D.C., Brown A.M., and Fishman G.I. Wnt-1 regulation of connexin 43 in cardiac myocytes. J Clin Invest 105 (2000) 161-171
22. Xu X., Li W.E., Huang G.Y., Meyer R., Chen T., Luo Y., Thomas M.P., Radice G.L., and Lo C.W. Modulation of mouse neural crest cell motility by N-cadherin and connexin 43 gap junctions. J Cell Biol 154 (2001) 217-230
23. Malhotra J.D., Thyagarajan V., Chen C., and Isom L.L. Tyrosine-phosphorylated and nonphosphorylated sodium channel beta1 subunits are differentially localized in cardiac myocytes. J Biol Chem 279 (2004) 40748-40754
24. Kostin S., Hein S., Bauer E.P., and Schaper J. Spatiotemporal development and distribution of intercellular junctions in adult rat cardiomyocytes in culture. Circ Res 85 (1999) 154-167
25. Zuppinger C., Schaub M.C., and Eppenberger H.M. Dynamics of early contact formation in cultured adult rat cardiomyocytes studied by N-cadherin fused to green fluorescent protein. J Mol Cell Cardiol 32 (2000) 539-555
26. Wei C.J., Xu X., and Lo C.W. Connexins and cell signaling in development and disease. Annu Rev Cell Dev Biol 20 (2004) 811-838
27. Giepmans B.N. Gap junctions and connexin-interacting proteins. Cardiovasc Res 62 (2004) 233-245
28. Wei C.J., Francis R., Xu X., and Lo C.W. Connexin 43 associated with an N-cadherin-containing multiprotein complex is required for gap junction formation in NIH3T3 cells. J Biol Chem 280 (2005) 19925-19936
29. Li J., Patel V.V., Kostetskii I., Xiong Y., Chu A.F., Jacobson J.T., Yu C., Morley G.E., Molkentin J.D., and Radice G.L. Cardiac-specific loss of N-cadherin leads to alteration in connexins with conduction slowing and arrhythmogenesis. Circ Res 97 (2005) 474-481
30. Pilichou K., Nava A., Basso C., Beffagna G., Bauce B., Lorenzon A., Frigo G., Vettori A., Valente M., Towbin J., Thiene G., Danieli G.A., and Rampazzo A. Mutations in desmoglein-2 gene are associated with arrhythmogenic right ventricular cardiomyopathy. Circulation 113 (2006) 1171-1179
31. Meurs K.M., Spier A.W., Miller M.W., Lehmkuhl L., and Towbin J.A. Familial ventricular arrhythmias in boxers. J Vet Intern Med 13 (1999) 437-439
32. van Tintelen J.P., Entius M.M., Bhuiyan Z.A., Jongbloed R., Wiesfeld A.C., Wilde A.A., van der Smagt J., Boven L.G., Mannens M.M., van Langen I.M., Hofstra R.M., Otterspoor L.C., Doevendans P.A., Rodriguez L.M., van Gelder I.C., and Hauer R.N. Plakophilin-2 mutations are the major determinant of familial arrhythmogenic right ventricular dysplasia/cardiomyopathy. Circulation 113 (2006) 1650-1658
33. Chen X., Bonne S., Hatzfeld M., van Roy F., and Green K.J. Protein binding and functional characterization of plakophilin 2. Evidence for its diverse roles in desmosomes and beta-catenin signaling. J Biol Chem 277 (2002) 10512-10522
34. Morley G.E., Vaidya D., Samie F.H., Lo C., Delmar M., and Jalife J. Characterization of conduction in the ventricles of normal and heterozygous Cx43 knockout mice using optical mapping. J Cardiovasc Electrophysiol 10 (1999) 1361-1375
35. Lerner D.L., Yamada K.A., Schuessler R.B., and Saffitz J.E. Accelerated onset and increased incidence of ventricular arrhythmias induced by ischemia in Cx43-deficient mice. Circulation 101 (2000) 547-552
36. Danik S.B., Liu F., Zhang J., Suk H.J., Morley G.E., Fishman G.I., and Gutstein D.E. Modulation of cardiac gap junction expression and arrhythmic susceptibility. Circ Res 95 (2004) 1035-1041