Non-equilibrium gating in cardiac Na+ channels: An original mechanism of arrhythmia

Circulation

Volumn 107, Issue 17, 2003, Pages 2233-2237

  Clancy, Colleen E ^b   Tateyama, Michihiro ^b   Liu, Huajun ^b   Wehrens, Xander H T ^b   Kass, Robert S ^a  

^a Columbia Univ Coll of Phys/Surgs ^*  (United States)

^b NONE

Author keywords

Arrhythmia; Long QT syndrome; Remodeling; Sodium

Indexed keywords

SODIUM CHANNEL;

ARTICLE; BINDING KINETICS; CELL FUNCTION; CHANNEL GATING; DIAGNOSTIC PROCEDURE; DISEASE ASSOCIATION; GENE MUTATION; HEART MUSCLE POTENTIAL; HUMAN; HUMAN CELL; LONG QT SYNDROME; MATHEMATICAL ANALYSIS; MEMBRANE CURRENT; MEMBRANE DEPOLARIZATION; MEMBRANE POTENTIAL; MOLECULAR DYNAMICS; MOLECULAR MODEL; NON EQUILIBRIUM GATING; PATCH CLAMP; PRIORITY JOURNAL; REGULATORY MECHANISM; STANDARD; STEADY STATE; THEORY; WILD TYPE;

ACTION POTENTIALS; CELL LINE; COMPUTATIONAL BIOLOGY; COMPUTER SIMULATION; ELECTRIC CONDUCTIVITY; HEART; HUMANS; ION CHANNEL GATING; LONG QT SYNDROME; MODELS, CARDIOVASCULAR; MUTATION; PATCH-CLAMP TECHNIQUES; SODIUM CHANNELS;

EID: 0038372754     PISSN: 00097322     EISSN: None     Source Type: Journal    
DOI: 10.1161/01.CIR.0000069273.51375.BD     Document Type: Article

References (29)

1. Marban E. Cardiac channelopathies. Nature. 2002;415:213-218.
2. Jentsch TJ. Neuronal KCNQ potassium channels: physiology and role in disease. Nat Rev Neurosci. 2000;1:21-30.
3. Jentsch TJ, Schroeder BC, Kubisch C, et al. Pathophysiology of KCNQ channels: neonatal epilepsy and progressive deafness. Epilepsia. 2000; 41:1068-1069.
4. Schwake M, Friedrich T, Jentsch TJ. An internalization signal in C1C-5, an endosomal Cl-channel mutated in Dent's disease. J Biol Chem. 2001; 276:12049-12054.
5. Lossin C, Wang DW, Rhodes TH, et al. Molecular basis of an inherited epilepsy. Neuron. 2002;34:877-884.
6. Jurkat-Rott K, Lehmann-Horn F. Human muscle voltage-gated ion channels and hereditary disease. Curr Opin Pharmacol. 2001;1:280-287.
7. Kullmann DM. The neuronal channelopathies, Brain. 2002;125: 1177-1195.
8. Wang Q, Shen J, Splawski I, et al. SCN5A mutations associated with an inherited cardiac arrhythmia, long QT syndrome. Cell. 1995;80:805-811.
9. Bennett PB, Yazawa K, Makita N, et al. Molecular mechanism for an inherited cardiac arrhythmia. Nature (Lond). 1995;376:683-685.
10. Clancy CE, Rudy Y. Linking a genetic defect to its cellular phenotype in a cardiac arrhythmia. Nature (Lond). 1999;400:566-569.
11. Nuyens D, Stengl M, Dugarmaa S, et al. Abrupt rate accelerations or premature beats cause life-threatening arrhythmias in mice with long-QT3 syndrome. Nat Med. 2001;7:1021-1027.
12. An RH, Wang XL, Kerem B, et al. Novel LQT-3 mutation affects Na+ channel activity through interactions between alpha- and beta1-subunits. Circ Res. 1998;83:141-146.
13. Rivolta I, Clancy CE, Tateyama M, et al. A novel SCN5A mutation associated with long QT-3: altered inactivation kinetics and channel dysfunction. Physiol Genomics. 2002;10:191-197.
14. Abriel H, Cabo C, Wehrens XH, et al. Novel arrhythmogenic mechanism revealed by a long-QT syndrome mutation in the cardiac Na(+) channel. Circ Res. 2001;88:740-745.
15. Clancy CE, Rudy Y. Na(+) channel mutation that causes both Brugada and long-QT syndrome phenotypes, a simulation study of mechanism. Circulation. 2002;105:1208-1213.
16. Noble D. Modeling the heart: from genes to cells to the whole organ. Science. 2002;295:1678-1682.
17. Noble D. The rise of computational biology. Nat Rev Mol Cell Biol. 2002;3:459-463.
18. Noble D. Unraveling the genetics and mechanisms of cardiac arrhythmia. Proc Natl Acad Sci USA. 2002;99:5755-5756.
19. Chandra R, Starmer CF, Grant AO. Multiple effects of KPQ deletion mutation on gating of human cardiac Na+ channels expressed in mammalian cells. Am J Physiol. 1998;274:H1643-H1654.
20. Wang DW, Yazawa K, George ALJ, et al. Characterization of human cardiac Na+ channel mutations in the congenital long QT syndrome. Proc Natl Acad Sci USA. 1996;93:13200-13205.
21. Luo CH, Rudy Y. A dynamic model of the cardiac ventricular action potential: I: simulations of ionic currents and concentration changes. Circ Res. 1994;74:1071-1096.
22. Patton C. WEBMAXCLITE vl.15. Available at: http://www. stanford.edu/-cpatton/webmaxelite115.htm. Accessed March 27, 2003.
23. Cardiac Bioelectricity Research and Training Center. Research. Available at: http://www.cwru.edu/med/CBRTC/Research.html. Accessed March 27, 2003.
24. Schwartz PJ, Priori SG, Spazzolini C, et al. Genotype-phenotype correlation in the long-QT syndrome: gene-specific triggers for life-threatening arrhythmias. Circulation. 2001;103:89-95.
25. Wang DW, VanDeCarr D, Ruben PC, et al. Functional consequences of a domain 1/S6 segment sodium channel mutation associated with painful congenital myotonia. FEBS Lett. 1999;448:231-234.
26. Jurkat-Rott K, Mitrovic N, Hang C, et al. Voltage-sensor sodium channel mutations cause hypokalemic periodic paralysis type 2 by enhanced inactivation and reduced current. Proc Natl Acad Sci USA. 2000;97: 9549-9554
27. Desaphy JF, De Luca A, Tortorella P, et al. Gating of myotonic Na channel mutants defines the response to mexiletine and a potent derivative. Neurology. 2001;57:1849-1857.
28. Green DS, George AL Jr, Cannon SC. Human sodium channel gating defects caused by missense mutations in S6 segments associated with myotonia: S804F and V1293I. J Physiol. 1998;510:685-694.
29. Wang DW, Viswanathan PC, Balser JR, et al. Clinical, genetic, and biophysical characterization of SCN5A mutations associated with atrioventricular conduction block. Circulation. 2002;105:341-346.