Selective knockout of mouse ERG1 B potassium channel eliminates IKr in adult ventricular myocytes and elicits episodes of abrupt sinus bradycardia

Molecular and Cellular Biology

Volumn 23, Issue 6, 2003, Pages 1856-1862

  Lees Miller, James P ^a   Guo, Jiqing ^a   Somers, Julie R ^a   Roach, Dan E ^a   Sheldon, Robert S ^a   Rancourt, Derrick E ^a   Duff, Henry J ^a  

^a UNIVERSITY OF CALGARY   (Canada)

Author keywords

[No Author keywords available]

Indexed keywords

POTASSIUM CHANNEL;

AGE DISTRIBUTION; ANIMAL CELL; ARTICLE; ELECTROCARDIOGRAM; EMBRYO; EMBRYO CELL; ERG1 GENE; FEMALE; FETUS; GENE; GENE MUTATION; HEART MUSCLE CELL; HEART REPOLARIZATION; HEART VENTRICLE; HOMOLOGOUS RECOMBINATION; KNOCKOUT GENE; LONG QT SYNDROME; MALE; MOUSE; NONHUMAN; POTASSIUM CURRENT; PRIORITY JOURNAL; PROTEIN EXPRESSION; SINUS BRADYCARDIA; TIME;

AGE FACTORS; ANIMALS; ARRHYTHMIA, SINUS; BRADYCARDIA; CATION TRANSPORT PROTEINS; DISEASE MODELS, ANIMAL; DNA-BINDING PROTEINS; ELECTROCARDIOGRAPHY; ETHER-A-GO-GO POTASSIUM CHANNELS; GENE TARGETING; HEART VENTRICLES; ION TRANSPORT; LONG QT SYNDROME; MICE; MICE, KNOCKOUT; MYOCARDIUM; PATCH-CLAMP TECHNIQUES; PHENETHYLAMINES; POTASSIUM; POTASSIUM CHANNEL BLOCKERS; POTASSIUM CHANNELS; POTASSIUM CHANNELS, VOLTAGE-GATED; PROTEIN ISOFORMS; RNA, MESSENGER; SULFONAMIDES; TRANS-ACTIVATORS;

ANIMALIA;

EID: 0037371841     PISSN: 02707306     EISSN: None     Source Type: Journal    
DOI: 10.1128/MCB.23.6.1856-1862.2003     Document Type: Article

References (46)

1. Ackerman, M. J. 1998. The long QT syndrome: ion channel diseases of the heart. Mayo Clin. Proc. 73:250-269.
2. Babij, P., G. R. Askew, B. Nieuwenhuijsen, C. M. Su, T. R. Bridal, B. Jow, T. M. Argentieri, J. Kulik, L. J. DeGennaro, W. Spinelli, and T. J. Colatsky. 1998. Inhibition of cardiac delayed rectifier K+ current by overexpression of the long QT syndrome HERG G628S mutation in transgenic mice. Circ. Res. 83:668-678.
3. Brahmajothi, M. V., M. J. Morales, K. A. Reimer, and H. C. Strauss. 1997. Regional localization of ERG, the channel protein responsible for the rapid component of the delayed rectifier, K+ current in the ferret heart. Circ. Res. 81:128-135.
4. Chen, J., A. Zou, I. Splawski, M. T. Keating, and M. C. Sanguinetti. 1999. Long QT syndrome-associated mutations in the Per-Arnt-Sim (PAS) domain of HERG potassium channels accelerate channel deactivation. J. Biol. Chem. 274:10113-10118.
5. Curran, M. E., I. Splawski, K. W. Timothy, G. M. Vincent, E. D. Green, and M. T. Keating. 1995. A molecular basis for cardiac arrhythmia: HERG mutations cause long QT syndrome. Cell 80:795-803.
6. Danielsson, B. R., A. C. Skold, and F. Azarbayjani. 2001. Class III antiarrhythmics and phenytoin: teratogenicity due to embryonic cardiac dysrhythmia and reoxygenation damage. Curr. Pharm. Des. 7:787-802.
7. Deng, C., and M. R. Capecchi. 1992. Reexamination of gene targeting frequency as a function of the extent of homology between the targeting vector and the target locus. Mol. Cell. Biol. 12:3365-3371.
8. Dong, D., Y. Duan, D. Roach, S. L. Swirp, L. Wang, J. P. Lees-Miller, R. S. Sheldon, J. D. Molkentin, and H. J. Duff. Overexpression of calcineurin in mouse causes sudden cardiac death associated with decreased density of K+ channels. Cardiovasc. Res., in press.
9. Furukawa, Y., Y. Miyashita, K. Nakajima, M. Hirose, F. Kurogouchi, and S. Chiba. 1999. Effects of verapamil, zatebradine, and E-4031 on the pacemaker location and rate in response to sympathetic stimulation in dog hearts. J. Pharmacol. Exp. Ther. 289:1334-1342.
10. Hoorntje, T., M. Alders, P. van Tintelen, K. van der Lip, N. Sreeram, A. van der Wal, M. Mannens, and A. Wilde. 1999. Homozygous premature truncation of the HERG protein: the human HERG knockout. Circulation 100:1264-1267.
11. Kato, H., and K. Moriwaki. 1972. Factors involved in the production of banded structures in mammalian chromosomes. Chromosoma 38:105-120.
12. Lees-Miller, J. P., C. Kondo, L. Wang, and H. J. Duff. 1997. Electrophysiological characterization of an alternatively processed ERG K+ channel in mouse and human hearts. Circ. Res. 81:719-726.
13. London, B., E. Aydar, C. M. Lewarchik, J. S. Seibel, C. T. January, and G. A. Robertson. 1998. N- and C-terminal isoforms of HERG in the human heart. Biophys. J. 74:A26.
14. London, B., and X. Pan. 1998. QT interval prolongation and arrhythmias in heterozygous MERG1-targeted mice. Circulation 98:279.
15. London, B., M. C. Trudeau, K. P. Newton, A. K. Beyer, N. G. Copeland, D. J. Gilbert, N. A. Jenkins, C. A. Satler, and G. A. Robertson. 1997. Two isoforms of the mouse ether-a-go-go- related gene coassemble to form channels with properties similar to the rapidly activating component of the cardiac delayed rectifier K+ current. Circ. Res. 81:870-878.
16. Manley, N. R., J. R. Barrow, T. Zhang, and M. R. Capecchi. 2001. Hoxb2 and Hoxb4 act together to specify ventral body wall formation. Dev. Biol. 237:130-144.
17. Marban, E. 1999. Heart failure: the electrophysiologic connection. J. Cardiovasc. Electrophysiol. 10:1425-1428.
18. Marban, E. 2002. Cardiac channelopathies. Nature 415:213-218.
19. McLenachan, J. M., E. Henderson, K. I. Morris, and H. J. Dargie. 1987. Ventricular arrhythmias in patients with hypertensive left ventricular hypertrophy. N. Engl. J. Med. 317:787-792.
20. Mitchell, G. F., A. Jeron, and G. Koren. 1998. Measurement of heart rate and Q-T interval in the conscious mouse. Am. J. Physiol. 274:H747-H751.
21. Molkenton, J. D., J. R. Lu, C. L. Antos, B. Markham, J. Richardson, J. Robbins, S. R. Grant, and E. N. Olson. 1998. A calcineurin-dependent transcriptional pathway for cardiac hypertrophy. Cell 93:215-228.
22. Morais Cabral, J. H., A. Lee, S. L. Cohen, B. T. Chait, M. Li, and R. Mackinnon. 1998. Crystal structure and functional analysis of the HERG potassium channel N terminus: a eukaryotic PAS domain. Cell 95:649-655.
23. Nagy, A., J. Rossant, R. Nagy, W. Abramow-Newerly, and J. C. Roder. 1993. Derivation of completely cell culture-derived mice from early-passage embryonic stem cells. Proc. Natl. Acad. Sci. USA 90:8424-8428.
24. Nerbonne, J. M., C. G. Nichols, T. L. Schwarz, and D. Escande. 2001. Genetic manipulation of cardiac K(+) channel function in mice: what have we learned, and where do we go from here? Circ. Res. 89:944-956.
25. Pond, A. L., B. K. Scheve, A. T. Benedict, K. Petrecca, D. R. Van Wagoner, A. Shrier, and J. M. Nerbonne. 2000. Expression of distinct ERG proteins in rat, mouse, and human heart. Relation to functional I(Kr) channels. J. Biol. Chem. 275:5997-6006.
26. Priori, S. G., C. Napolitano, and P. J. Schwartz. 1999. Low penetrance in the long QT syndrome: clinical impact. Circulation 99:529-533.
27. Sanguinetti, M. C., M. E. Curran, P. S. Spector, and M. T. Keating. 1996. Spectrum of HERG K+ -channel dysfunction in an inherited cardiac arrhythmia. Proc. Natl. Acad. Sci. USA 93:2208-2212.
28. Sanguinetti, M. C., C. Jiang, M. E. Curran, and M. T. Keating. 1995. A mechanistic link between an inherited and an acquired cardiac arrhythmia: HERG encodes the IKr potassium channel. Cell 81:299-307.
29. Schwartz, P. J., S. G. Priori, C. Spazzolini, A. J. Moss, G. M. Vincent, C. Napolitano, I. Denjoy, P. Guicheney, G. Breithardt, M. T. Keating, J. A. Towbin, A. H. Beggs, P. Brink, A. A. Wilde, L. Toivonen, W. Zareba, J. L. Robinson, K. W. Timothy, V. Corfield, D. Wattanasirichaigoon, C. Corbett, W. Haverkamp, E. Schulze-Bahr, M. H. Lehmann, K. Schwartz, P. Coumel, and R. Bloise. 2001. Genotype-phenotype correlation in the long-QT syndrome: gene-specific triggers for life-threatening arrhythmias. Circulation 103:89-95.
30. Shen, C. T., Y. C. Wu, S. S. Yu, and N. K. Wang. 1997. Multi-undulant T-U-wave, sinus bradycardia and long QT syndrome: a possible phenotype of mutant genes controlling the inward potassium rectifiers. Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi 38:267-275.
31. Skold, A. C., and B. R. Danielsson. 2000. Developmental toxicity of the class III antiarrhythmic agent almokalant in mice. Adverse effects mediated via induction of embryonic heart rhythm abnormalities. Arzneimittelforschung 50:520-525.
32. Smith, P. L., T. Baukrowitz, and G. Yellen. 1996. The inward rectification mechanism of the HERG cardiac potassium channel. Nature 379:833-836.
33. Kr, in rabbit sinoatrial node cells by external divalent cations. Pflugers Arch. 438:147-153.
34. Splawski, I., J. Shen, K. W. Timothy, M. H. Lehmann, S. Priori, J. L. Robinson, A. J. Moss, P. J. Schwartz, J. A. Towbin, G. M. Vincent, and M. T. Keating. 2000. Protein spectrum of mutations in long QT syndrome genes. KVLQT1, HERG, SCN5A, KCNE1, and KCNE2. Circulation 102:1178-1185.
35. Stevenson, W. G., L. W. Stevenson, H. R. Middlekauff, and L. A. Saxon. 1993. Sudden death prevention in patients with advanced ventricular dysfunction. Circulation 88:2953-2961.
36. Tsuzuki, T., and D. E. Rancourt. 1998. ES cell gene targeting using phage vectors generated by plasmid-phage recombination. Nucleic Acids Res. 26:988-994.
37. Vandenberg, J. I., B. D. Walker, and T. J. Campbell. 2001. HERG K+ channels: friend and foe. Trends Pharmacol. Sci. 22:240-246.
38. Vincent, G. M. 1998. The molecular genetics of the long QT syndrome: genes causing fainting and sudden death. Annu. Rev. Med. 49:263-274.
39. Wang, L., and H. J. Duff. 1997. Developmental changes in transient outward current in mouse ventricle. Circ. Res. 81:120-127.
40. Wang, L., Z. P. Feng, C. S. Kondo, R. S. Sheldon, and H. J. Duff. 1996. Developmental changes in the delayed rectifier K+ channels in mouse heart. Circ. Res. 79:79-85.
41. Wang, L., S. Swirp, and H. J. Duff. 2000. Age-dependent response of the electrocardiogram to K(+) channel blockers in mice. Am. J. Physiol. 278: C73-C80.
42. Wang, Z., B. Fermini, and S. Nattel. 1994. Rapid and slow components of delayed rectifier current in human atrial myocytes. Cardiovasc. Res. 28:1540-1546.
43. Wymore, R. S., G. A. Gintant, R. T. Wymore, J. E. Dixon, D. McKinnon, and I. S. Cohen. 1997. Tissue and species distribution of mRNA for the IKr-like K+ channel, erg. Circ. Res. 80:261-268.
44. Yoshida, H., M. Horie, H. Otani, T. Kawashima, Y. Onishi, and S. Sasayama. 2001. Bradycardia-induced long QT syndrome caused by a de novo missense mutation in the S2-S3 inner loop of HERG. Am. J. Med. Genet. 98:348-352.
45. Zhou, Z., Q. Gong, B. Ye, Z. Fan, J. C. Makielski, G. A. Robertson, and C. T. January. 1998. Properties of HERG channels stably expressed in HEK 293 cells studied at physiological temperature. Biophys. J. 74:230-241.
46. Zhou, Z., Q. Gong, and C. T. January. 1999. Correction of defective protein trafficking of a mutant HERG potassium channel in human long QT syndrome. Pharmacological and temperature effects. J. Biol. Chem. 274:31123-31126.