A novel mechanism for LQT3 with 2:1 block: A pore-lining mutation in Nav1.5 significantly affects voltage-dependence of activation

Heart Rhythm

Volumn 8, Issue 5, 2011, Pages 770-777

  Horne, Andrew J ^a   Eldstrom, Jodene ^a   Sanatani, Shubhayan ^a   Fedida, David ^a  

^a UNIVERSITY OF BRITISH COLUMBIA   (Canada)

Author keywords

Cardiac Sodium Channel; Flecainide; Lidocaine; LQT3; Mexiletine; V411M

Indexed keywords

ANTIARRHYTHMIC AGENT; LIDOCAINE; MEXILETINE; VOLTAGE GATED SODIUM CHANNEL;

ACTION POTENTIAL; ARTICLE; ATRIOVENTRICULAR BLOCK; CONGENITAL DISORDER; ECHOCARDIOGRAPHY; EMBRYO CELL; GENE; GENE MUTATION; GENETIC VARIABILITY; HEART ELECTROPHYSIOLOGY; HEART RIGHT VENTRICLE FUNCTION; HUMAN; HUMAN CELL; HYPERPOLARIZATION; IN VIVO STUDY; KIDNEY CELL; LONG QT SYNDROME; LQT3 GENE; PATCH CLAMP; PRIORITY JOURNAL; QT INTERVAL; REPOLARIZATION; SODIUM CURRENT;

ATRIOVENTRICULAR BLOCK; FEMALE; HUMANS; INFANT, NEWBORN; LONG QT SYNDROME; SODIUM CHANNELS;

EID: 79955454223     PISSN: 15475271     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.hrthm.2010.12.041     Document Type: Article

References (25)

1. P.J. Schwartz, M. Stramba-Badiale, and L. Crotti Prevalence of the congenital long-QT syndrome Circ 120 2009 1761 1767
2. C. Napolitano, S.G. Priori, and P.J. Schwartz Genetic testing in the long QT syndrome: development and validation of an efficient approach to genotyping in clinical practice JAMA 294 2005 2975 2980 (Pubitemid 41817658)
3. P.J. Schwartz, S.G. Priori, and C. Spazzolini Genotype-phenotype correlation in the long-QT syndrome: gene-specific triggers for life-threatening arrhythmias Circ 103 2001 89 95 (Pubitemid 32050441)
4. W. Zareba, A.J. Moss, and P.J. Schwartz Influence of genotype on the clinical course of the long-QT syndrome International Long-QT Syndrome Registry Research Group N Engl J Med 339 1998 960 965 (Pubitemid 28455098)
5. P.J. Schwartz, S.G. Priori, and E.H. Locati Long QT syndrome patients with mutations of the SCN5A and HERG genes have differential responses to Na+ channel blockade and to increases in heart rate Implications for gene-specific therapy Circ 92 1995 3381 3386
6. J. Benhorin, R. Taub, and M. Goldmit Effects of flecainide in patients with new SCN5A mutation: mutation-specific therapy for long-QT syndrome? Circ 101 2000 1698 1706 (Pubitemid 30196631)
7. A.J. Moss, W. Zareba, K.Q. Schwarz, S. Rosero, S. McNitt, and J.L. Robinson Ranolazine shortens repolarization in patients with sustained inward sodium current due to type-3 long-QT syndrome J Cardiovasc Electrophysiol 19 2008 1289 1293
8. T. Zimmer, and R. Surber SCN5A channelopathies - an update on mutations and mechanisms Prog Biophys Mol Biol 98 2008 120 136
9. P.B. Bennett, K. Yazawa, N. Makita, and A.L. George Jr Molecular mechanism for an inherited cardiac arrhythmia Nature 376 1995 683 685
10. D.W. Wang, K. Yazawa, A.L. George Jr., and P.B. Bennett Characterization of human cardiac Na+ channel mutations in the congenital long QT syndrome Proc Natl Acad Sci U S A 93 1996 13200 13205 (Pubitemid 26382793)
11. C.E. Clancy, M. Tateyama, H. Liu, X.H. Wehrens, and R.S. Kass Non-equilibrium gating in cardiac Na+ channels: an original mechanism of arrhythmia Circ 107 2003 2233 2237 (Pubitemid 36547218)
12. D.J. Tester, M.L. Will, C.M. Haglund, and M.J. Ackerman Compendium of cardiac channel mutations in 541 consecutive unrelated patients referred for long QT syndrome genetic testing Heart Rhythm 2 2005 507 517 (Pubitemid 40521365)
13. C. Luo, and Y. Rudy A model of the ventricular cardiac action potential: Depolarization, repolarization, and their interaction Circ Res 68 1991 1501 1526
14. M. Courtemanche, R.J. Ramirez, and S. Nattel Ionic mechanisms underlying human atrial action potential properties: insights from a mathematical model Am J Physiol 275 1998 H301 H321
15. L. Belardinelli, J.C. Shryock, and H. Fraser Inhibition of the late sodium current as a potential cardioprotective principle: effects of the late sodium current inhibitor ranolazine Heart 92 Suppl 4 2006 iv6 iv14
16. P.E. Fenster, and K.A. Comess Pharmacology and clinical use of mexiletine Pharmacotherapy 6 1986 1 9 (Pubitemid 16103456)
17. D. Fedida, P.M. Orth, J.C. Hesketh, and A.M. Ezrin The role of late INa and antiarrhythmic drugs in EAD formation and termination of Purkinje fibers J Cardiovasc Electrophysiol 17 Suppl 1 2006 S71 S78
18. G.J. Amos, E. Wettwer, F. Metzger, Q. Li, H.M. Himmel, and U. Ravens Differences between outward currents of human atrial and subepicardial ventricular myocytes J Physiol (Lond ) 491 1996 31 50 (Pubitemid 26059547)
19. R.H. An, R. Bangalore, S.Z. Rosero, and R.S. Kass Lidocaine block of LQT-3 mutant human Na+ channels Circ Res 79 1996 103 108 (Pubitemid 26196323)
20. D.S. Ragsdale, J.C. McPhee, T. Scheuer, and W.A. Catterall Molecular determinants of state-dependent block of Na+ channels by local anesthetics Science 265 1994 1724 1728 (Pubitemid 24314907)
21. H.B. Nuss, N.G. Kambouris, E. Marban, G.F. Tomaselli, and J.R. Balser Isoform-specific lidocaine block of sodium channels explained by differences in gating Biophys J 78 2000 200 210 (Pubitemid 30207131)
22. E. Ramos, and M.E. O'Leary State-dependent trapping of flecainide in the cardiac sodium channel J Physiol 560 2004 37 49 (Pubitemid 39406015)
23. L. Belardinelli, A. Dhalla, and J. Shryock Abnormal left ventricular relaxation in patients with long QT syndrome Eur Heart J 30 2009 2813 2814
24. R.N. Subbiah, M.H. Gollob, and L.J. Gula Torsades de pointes during complete atrioventricular block: Genetic factors and electrocardiogram correlates Can J Cardiol 26 2010 208 212
25. M. Yamaguchi, M. Shimizu, and H. Ino T wave peak-to-end interval and QT dispersion in acquired long QT syndrome: a new index for arrhythmogenicity Clin Sci (Lond) 105 2003 671 676 (Pubitemid 38002357)