Novel mechanism of HERG current suppression in LQT2: Shift in voltage dependence of HERG inactivation

Circulation Research

Volumn 83, Issue 4, 1998, Pages 415-422

  Nakajima, Tadashi ^a,c   Furukawa, Tetsushi ^a   Tanaka, Toshihiro ^b   Katayama, Yoshifumi ^a   Nagai, Ryozo ^c   Nakamura, Yusuke ^b   Hiraoka, Masayasu ^a  

^a TOKYO MEDICAL AND DENTAL UNIVERSITY   (Japan)

^b UNIVERSITY OF TOKYO   (Japan)

^c GUNMA UNIVERSITY   (Japan)

Author keywords

Cardiac arrhythmia; HERG mutation; Long QT syndrome

Indexed keywords

RNA;

ANIMAL CELL; ARTICLE; CONCENTRATION RESPONSE; ION PERMEABILITY; LONG QT SYNDROME; MEMBRANE CURRENT; MEMBRANE ELECTROPHYSIOLOGY; MEMBRANE POTENTIAL; MISSENSE MUTATION; NONHUMAN; PRIORITY JOURNAL; XENOPUS;

EID: 0032563717     PISSN: 00097330     EISSN: None     Source Type: Journal    
DOI: 10.1161/01.RES.83.4.415     Document Type: Article

References (31)

1. Ward OC. A new familial cardiac syndrome in children. J Ir Med Assoc. 1964;54:103-106.
2. Romano C. Congenital cardiac arrhythmia. Lancet. 1965;1:658-659.
3. Schwartz PJ, Periti M, Malliani A. The long QT syndrome. Am Heart J. 1975;109:378-390.
4. Keating M, Atkinson D, Dunn C, Timothy K, Vincent GM, Leppert M. Linkage of a cardiac arrhythmia, the long QT syndrome, and the Harvey ras-1 gene. Science. 1991;252:704-706.
5. Wang Q, Curran ME, Splawski I, Burn TC, Millholland JM, VanRaay TJ, Shen J, Timothy KW, Vincent GM, de Jager T, Schwartz PJ, Towbin JA, Moss AJ, Atkinson DL, Landes GM, Connors TD, Keating MT. Positional cloning of a novel potassium channel gene: KVLQT1 mutations cause cardiac arrhythmias. Nat Genet. 1996;12:17-23.
6. Jiang C, Atkinson D, Towbin JA, Splawski I, Lehmann MH, Li H, Timothy K, Taggart RT, Schwartz PJ, Vincent GM, Moss AJ, Keating MT. Two long QT syndrome loci map to chromosomes 3 and 7 with evidence for further heterogeneity. Nat Genet. 1994;8:141-147.
7. Curran ME, Splawski I, Timothy KW, Vincent GM, Green ED, Keating MT. A molecular basis for cardiac arrhythmia: HERG mutations cause long QT syndrome. Cell. 1995;80:795-803.
8. Wang Q, Shen J, Splawski I, Atkinson D, Li Z, Robinson LJ, Moss AJ, Towbin JA, Keating MT. SCN5A mutations associated with an inherited cardiac arrythmia, long QT syndrome. Cell. 1995;80:805-811.
9. Schott JJ, Charpentier F, Peltier S, Foley P, Drouin E, Bouhour JB, Donnelly P, Vergnaud G, Bachner L, Moisan JP, Le Marec H, Pascal O. Mapping of a gene for long QT syndrome to chromosome 4q25-27. Am J Hum Genet. 1995;57:1114-1122.
10. Ks function. Nat Genet. 1997;17:338-340.
11. Warmke JW, Ganetzky B. A family of potassium channel genes related to eag in Drosophila and mammals. Proc Natl Acad Sci U S A. 1994;91: 3438-3442.
12. + current: differential sensitivity to block by class III antiarrhythmic agents. J Gen Physiol. 1990;96:195-215.
13. Kr potassium channel. Cell. 1995;81:299-307.
14. Benson DW, MacRae CA, Vesely MR, Walsh EP, Seidman JG, Seidman CE, Satler CA. Missense mutation in the pore region of HERG causes familial long QT syndrome. Circulation. 1996;93:1791-1795.
15. Satler CA, Walsh EP, Vesely MR, Plummer MH, Ginsburg GS, Jacob HJ. Novel missense mutation in the cyclic nucleotide-binding domain of HERG causes long QT syndrome. Am J Med Genet. 1996;65:27-35.
16. Schulze-Bahr E, Haverkamp W, Funke H. The long QT syndrome. N Engl J Med. 1995;333:1783-1784.
17. Dausse E, Berthet M, Denjoy I, Andre-Fouet X, Cruaud C, Bennaceur M, Faure S, Coumel P, Schwartz K, Guicheney P. A mutation in HERG associated with notched T waves in long QT syndrome. J Mol Cell Cardiol. 1996;28:1609-1613.
18. +-channel dysfunction in an inherited cardiac arrhythmia (long QT syndrome). Proc Natl Acad Sci U S A. 1996;93:2208-2212.
19. MacKinnon R. Determination of the subunit stoichiometry of a voltage-activated potassium channel. Nature. 1991;350:232-235.
20. Li X, Xu J, Li M. The human Δ1261 mutation of the HERG potassium channel results in a truncated protein that contains a subunit interaction domain and decreases the channel expression. J Biol Chem. 1997;272: 705-708.
21. Tanaka T, Nagai R, Tomoike H, Takata S, Yano K, Yabuta K, Haneda N, Nakano O, Shibata A, Sawayama T, Kasai H, Yazaki Y, Nakamura Y. Four novel KVLQT1 and four novel HERG mutations in familial long QT syndrome. Circulation. 1997;95:565-567.
22. Furukawa T, Ogura T, Katayama Y, Hiraoka M. Characteristics of rabbit C1C-2 current expressed in Xenopus oocytes and its contribution to volume regulation. Am J Physiol. 1998;274:C500-C512.
23. Smith PL, Baukrowitz T, Yellen G. The inward rectification mechanism of the HERG cardiac potassium channel. Nature. 1996; 379:833-836.
24. Wang S, Liu S, Morales MJ, Strauss HC, Rasmusson RL. A quantitative analysis of the activation and inactivation kinetics of HERG expressed in Xenopus oocytes. J Physiol (Lond). 1997;502:45-60.
25. Trudeau MC, Warmke JW, Ganetzky B, Robertson GA. HERG, a human inward rectifier in the voltage-gated potassium channel family. Science. 1995;269:92-95.
26. Schönherr R, Heinemann SH. Molecular determinants for activation and inactivation of HERG, a human inward rectifier potassium channel. J Physiol (Lond). 1996;493:635-642.
27. Zhou Z, Gong Q, Ye B, Fan Z, Makielski JC, Robertson GA, January CT. Properties of HERG channels stably expressed in HEK 293 cells studied at physiological temperature. Biophys J. 1998;74:230-241.
28. + channels: effects of alterations in the carboxy-terminal region. Neuron. 1991;7:547-556.
29. Lopex-Barneo J, Hoshi T, Heinemann SH, Aldrich RW. Effects of external cations and mutations in the pore region on C-type inactivation of Shaker potassium channels. Receptors Channels. 1993;1:61-71.
30. Wang S, Morales MJ, Liu S, Strauss HC, Rasmusson RL. Time, voltage and ionic concentration dependence of rectification of h-erg expressed in Xenopus oocytes. FEBS Lett. 1996;389:167-173.
31. + channels. Circ Res. 1998; 82:386-395.