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hERG

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354: 331: 228: 253: 612: 619: 3115: 3130: 605: 1501:(known as 'the turret') and 'the pore loop', which begins and ends extracellularly but loops into the plasma membrane; the pore loop for each of the hERG subunits in one channel faces into the ion-conducting pore and is adjacent to the corresponding loops of the three other subunits, and together they form the selectivity filter region of the channel pore. The selectivity sequence, SVGFG, is very similar to that contained in bacterial 360: 259: 49: 1500:
amino acid residue at every 3rd position and is thought to act as a voltage-sensitive sensor, which allows the channel to respond to voltage changes by changing conformations between conducting and non-conducting states (called 'gating'). Between the S5 and S6 helices, there is an extracellular loop
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are used interchangeably, but, in the technical sense, "hERG channels" can be made only by scientists in the laboratory; in formal terms, the naturally occurring channels in the body that include hERG are referred to by the name of the electrical current that has been measured in that cell type, so,
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Due to the documented potential of QT-interval-prolonging drugs, the United States Food and Drug Administration issued recommendations for the establishment of a cardiac safety profile during pre-clinical drug development: ICH S7B. The nonclinical evaluation of the potential for delayed ventricular
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Although there exist other potential targets for cardiac adverse effects, the vast majority of drugs associated with acquired QT prolongation are known to interact with the hERG potassium channel. One of the main reasons for this phenomenon is the larger inner vestibule of the hERG channel, thus
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Moss AJ, Zareba W, Kaufman ES, Gartman E, Peterson DR, Benhorin J, et al. (February 2002). "Increased risk of arrhythmic events in long-QT syndrome with mutations in the pore region of the human ether-a-go-go-related gene potassium channel".
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When this channel's ability to conduct electrical current across the cell membrane is inhibited or compromised, either by application of drugs or by rare mutations in some families, it can result in a potentially fatal disorder called
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Gutman GA, Chandy KG, Grissmer S, Lazdunski M, McKinnon D, Pardo LA, et al. (December 2005). "International Union of Pharmacology. LIII. Nomenclature and molecular relationships of voltage-gated potassium channels".
1551:. Among the drugs that can cause QT prolongation, the more common ones include antiarrhythmics (especially Class 1A and Class III), anti-psychotic agents, and certain antibiotics (including quinolones and macrolides). 2923:
Taglialatela M, Castaldo P, Pannaccione A, Giorgio G, Annunziato L (June 1998). "Human ether-a-gogo related gene (HERG) K+ channels as pharmacological targets: present and future implications".
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repolarization (QT interval prolongation) by human pharmaceuticals, issued as CHMP/ICH/423/02, adopted by CHMP in May 2005. Preclinical hERG studies should be accomplished in
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hERG has also been associated with modulating the functions of some cells of the nervous system and with establishing and maintaining cancer-like features in leukemic cells.
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This channel is also sensitive to drug binding, as well as decreased extracellular potassium levels, both of which can result in decreased channel function and drug-induced
367: 266: 1539:), due to repolarization disturbances of the cardiac action potential. There are far more hERG mutations described for long QT syndrome than for short QT syndrome. 3054: 3114: 2072:"A comparison of currents carried by HERG, with and without coexpression of MiRP1, and the native rapid delayed rectifier current. Is MiRP1 the missing link?" 1416:) of the cell membrane during the cardiac action potential. Sometimes, when referring to the pharmacological effects of drugs, the terms "hERG channels" and 3129: 1505:. Although a full crystal structure for hERG is not yet available, a structure has been found for the cytoplasmic N-terminus, which was shown to contain a 2121:"Disease-associated mutations in KCNE potassium channel subunits (MiRPs) reveal promiscuous disruption of multiple currents and conservation of mechanism" 1012: 993: 1348: 189: 1374:. A number of clinically successful drugs in the market have had the tendency to inhibit hERG, lengthening the QT and potentially leading to a fatal 5411: 4795: 4778: 4773: 4768: 4763: 4800: 3898: 3170: 1460:'s inward rectification that results in the channel producing a 'paradoxical resurgent current' in response to repolarization of the membrane. 1446:
expression), a potassium ion channel is formed, and this channel has many signature features of the cardiac 'rapid' delayed rectifier current (
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Trudeau MC, Warmke JW, Ganetzky B, Robertson GA (July 1995). "HERG, a human inward rectifier in the voltage-gated potassium channel family".
2005: 1768: 1750: 5401: 4852: 4033: 1608: 1590: 1370:. Conversely, genetic mutations that increase the current through these channels can lead to the related inherited heart rhythm disorder 4750: 3771: 3187: 2735:"S7B Nonclinical Evaluation of the Potential for Delayed Ventricular Repolarization (QT Interval Prolongation) by Human Pharmaceuticals" 1442:) of hERG, but, when the originally-discovered form of hERG is experimentally transferred into cells that previously lacked hERG (i.e., 1244: 353: 4536: 2734: 2295: 2046: 1562:, and it does prolong the QT interval, but its multiple other antiarrhythmic effects prevent this from causing torsades de pointes. 1251: 330: 3062: 5129: 4128: 3906: 3222: 1737: 1716: 1689: 5151: 1733: 252: 227: 72: 5360: 3271: 1712: 4726: 3638: 3066: 2742: 169: 5001: 1640: 1379: 366: 265: 2434:"A mechanistic link between an inherited and an acquired cardiac arrhythmia: HERG encodes the IKr potassium channel" 706:
voltage-gated potassium channel activity involved in ventricular cardiac muscle cell action potential repolarization
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causes peculiarly severe QTc prolongation by blocking hERG and was withdrawn by the manufacturer for this reason.
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Vincent GM (1998). "The molecular genetics of the long QT syndrome: genes causing fainting and sudden death".
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is not yet available, but structures have recently been solved by electron microscopy. In the laboratory the
2573:"Crystal structure and functional analysis of the HERG potassium channel N terminus: a eukaryotic PAS domain" 5350: 2666:"Blockade of HERG potassium currents by fluvoxamine: incomplete attenuation by S6 mutations at F656 or Y652" 1988:
Overholt JL, Ficker E, Yang T, Shams H, Bright GR, Prabhakar NR (2002). "Chemosensing at the Carotid Body".
1431:. This difference in nomenclature becomes clearer in the controversy as to whether the channels conducting 1038: 1589:
The hERG gene was first named and described in a paper by Jeff Warmke and Barry Ganetzky, then both at the
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include other subunits (e.g., beta subunits) or whether the channels include a mixture of different types (
160:, ERG-1, ERG1, H-ERG, HERG, HERG1, Kv11.1, LQT2, SQT1, potassium voltage-gated channel subfamily H member 2 5381: 3373: 3360: 3347: 3327: 3314: 3279: 2273: 1789:
Hedley PL, Jørgensen P, Schlamowitz S, Wangari R, Moolman-Smook J, Brink PA, et al. (November 2009).
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hERG potassium channel comprises four identical alpha subunits, which form the channel's pore through the
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Robertson GA, Jones EM, Wang J (2005). "Gating and Assembly of Heteromeric hERG1a/1b Channels Underlying
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hERG forms the major portion of one of the ion channel proteins (the 'rapid' delayed rectifier current (
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voltage-gated potassium channel activity involved in cardiac muscle cell action potential repolarization
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Bjerregaard P, Gussak I (February 2005). "Short QT syndrome: mechanisms, diagnosis and treatment".
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Sanguinetti MC, Tristani-Firouzi M (March 2006). "hERG potassium channels and cardiac arrhythmia".
1535: 1383: 1223: 1194: 1160: 1131: 1347:. This ion channel (sometimes simply denoted as 'hERG') is best known for its contribution to the 4721: 3597: 3244: 3025: 2987: 2646: 2602: 2463: 2199: 2150: 1921: 1863: 1820: 1612: 201: 2483:"LQT2 : amplitude reduction and loss of selectivity in the tail that wags the HERG channel" 1227: 1202: 1198: 1190: 1164: 1139: 1135: 1127: 611: 1555:
providing more space for many different drug classes to bind and block this potassium channel.
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Abbott GW, Sesti F, Splawski I, Buck ME, Lehmann MH, Timothy KW, et al. (April 1999).
604: 197: 5064: 3204: 1469: 1340: 421: 33: 2397: 2339: 2170:"MiRP1 forms IKr potassium channels with HERG and is associated with cardiac arrhythmia" 1901: 48: 4860: 3547: 2783: 2758: 2690: 2665: 2548: 2523: 2096: 2071: 1965: 1940: 1624: 1484:, numbered S1-S6, a pore helix situated between S5 and S6, and cytoplasmically located 1409: 2936: 2832: 2807: 2589: 2572: 2186: 2169: 927: 922: 917: 912: 907: 902: 897: 892: 887: 882: 877: 872: 867: 862: 857: 852: 847: 842: 837: 832: 827: 822: 817: 812: 807: 802: 797: 781: 776: 771: 766: 761: 756: 751: 746: 730: 725: 720: 715: 710: 705: 700: 695: 690: 685: 680: 675: 670: 665: 660: 5395: 4814: 4487: 3029: 2450: 2433: 2358: 2323: 2272:. Novartis Foundation Symposia. Vol. 266. pp. 4–15, discussion 15–8, 44–5. 1956: 1620: 1526: 1477: 1412:), and this current is critical in correctly timing the return to the resting state ( 647: 2991: 2606: 2154: 1824: 1607:
was named in the 1960s by William D. Kaplan and William E. Trout, III, while at the
181: 5167: 4558: 2650: 2203: 1867: 1565: 1473: 1443: 439: 218: 2808:"14-3-3 amplifies and prolongs adrenergic stimulation of HERG K+ channel activity" 2467: 2033:. Novartis Foundation Symposia. Vol. 266. pp. 225–32, discussion 232–4. 1992:. Advances in Experimental Medicine and Biology. Vol. 475. pp. 241–248. 1925: 3063:
Special K: Primate-specific Potassium Channel Variant Implicated in Schizophrenia
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Morais Cabral JH, Lee A, Cohen SL, Chait BT, Li M, Mackinnon R (November 1998).
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National Center for Biotechnology Information, U.S. National Library of Medicine
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National Center for Biotechnology Information, U.S. National Library of Medicine
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Proceedings of the National Academy of Sciences of the United States of America
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membrane repolarization during ventricular cardiac muscle cell action potential
522: 5077: 4443: 3811: 3779: 2823: 2324:"A family of potassium channel genes related to eag in Drosophila and mammals" 1838:
Bjerregaard P (August 2018). "Diagnosis and management of short QT syndrome".
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The hERG Cardiac Potassium Channel: Structure, Function and Long QT Syndrome
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The hERG Cardiac Potassium Channel: Structure, Function and Long QT Syndrome
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Arcangeli A (2005). "Expression and Role of hERG Channels in Cancer Cells".
957: 582: 460: 405: 392: 304: 291: 193: 3021: 2983: 2841: 2699: 2681: 2642: 2557: 2508: 2348: 2305: 2287: 2244: 2235: 2218: 2195: 2146: 2105: 2056: 2015: 1917: 1859: 1816: 2975: 2954: 2915: 2880: 2792: 2634: 2598: 2524:"Cryo-EM Structure of the Open Human Ether-à-go-go-Related K Channel hERG" 2459: 2413: 2367: 2217:
Jones EM, Roti Roti EC, Wang J, Delfosse SA, Robertson GA (October 2004).
2137: 1974: 1408:)) that conducts potassium (K) ions out of the muscle cells of the heart ( 1291: 1286: 5175: 4523: 3502: 3497: 3492: 3487: 3419: 3414: 1493: 1275: 1102: 1083: 2713: 2070:
Weerapura M, Nattel S, Chartier D, Caballero R, Hébert TE (April 2002).
1909: 1791:"The genetic basis of long QT and short QT syndromes: a mutation update" 5302: 5059: 4895: 3396: 3391: 3386: 3123:: STRUCTURE OF THE N-TERMINAL DOMAIN OF THE HUMAN-ERG POTASSIUM CHANNEL 3013: 2945: 2664:
Milnes JT, Crociani O, Arcangeli A, Hancox JC, Witchel HJ (July 2003).
1807: 1790: 1525:. Both clinical disorders stem from ion channel dysfunction (so-called 1439: 1069: 1024: 126: 122: 118: 114: 110: 106: 102: 98: 94: 4979: 4974: 4708: 4703: 4649: 4644: 4460: 4448: 4438: 4433: 4421: 4416: 4411: 4298: 4293: 4288: 4180: 4116: 4111: 4106: 4101: 4096: 4091: 4086: 4021: 4016: 4006: 4001: 3996: 3947: 3942: 3932: 3927: 3804: 3799: 3794: 3789: 3695: 3690: 3685: 3675: 3670: 3660: 3655: 3585: 3580: 3575: 3570: 3473: 3468: 3463: 3458: 3446: 3441: 3436: 3431: 3340: 3307: 3302: 3297: 3292: 3138:: Solution Structure of the HERG K+ channel S5-P extracellular linker 2906: 2889: 1497: 1259: 979: 903:
regulation of ventricular cardiac muscle cell membrane repolarization
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membrane repolarization during cardiac muscle cell action potential
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Chiesa N, Rosati B, Arcangeli A, Olivotto M, Wanke E (June 1997).
1644: 1623:, their legs start to shake, like the dancing at the then popular 918:
negative regulation of potassium ion export across plasma membrane
2219:"Cardiac IKr channels minimally comprise hERG 1a and 1b subunits" 942: 938: 5293: 5288: 5283: 5273: 5268: 5263: 5253: 5248: 5243: 5238: 5233: 5228: 5223: 5208: 5203: 5198: 5193: 5188: 5183: 5122: 5117: 5112: 5107: 5102: 5008: 4996: 4991: 4986: 4917: 4870: 4834: 4829: 4824: 4819: 4546: 3922: 3259: 3249: 173: 5323: 4508: 3754: 3542: 3199: 3152: 3076: 1941:"A novel role for HERG K+ channels: spike-frequency adaptation" 627: 4847: 4745: 3866: 3826: 3072: 2432:
Sanguinetti MC, Jiang C, Curran ME, Keating MT (April 1995).
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negative regulation of potassium ion transmembrane transport
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positive regulation of potassium ion transmembrane transport
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Kagan A, Melman YF, Krumerman A, McDonald TV (April 2002).
2890:"The long QT syndrome: ion channel diseases of the heart" 2759:"The behavior of four neurological mutants of Drosophila" 1529:) that can lead to the risk of potentially fatal cardiac 1509:(aminoacid 26–135) that slows the rate of deactivation. 429: 1517:
Loss-of-function mutations in this channel may lead to
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GeneReviews/NIH/NCBI/UW entry on Romano-Ward Syndrome
1521:(LQT2), while gain-of-function mutations may lead to 594: 5336: 5160: 5049: 4845: 4743: 4719: 4521: 4127: 4032: 3897: 3770: 3637: 3596: 3558: 3270: 3215: 2427: 2425: 2423: 1216: 1183: 1153: 1120: 898:
regulation of potassium ion transmembrane transport
1883: 1881: 1879: 1877: 1729: 1727: 1725: 1708: 1706: 1704: 2964:Nature Clinical Practice. Cardiovascular Medicine 2256: 2254: 1480:. Each hERG subunit consists of 6 transmembrane 376: 275: 823:ventricular cardiac muscle cell action potential 32:"IKR" redirects here. For the airline code, see 2379: 2377: 1424:for example, in the heart, the correct name is 843:membrane repolarization during action potential 798:membrane depolarization during action potential 2317: 2315: 1734:GRCm38: Ensembl release 89: ENSMUSG00000038319 1468:A detailed atomic structure for hERG based on 1390:that must be avoided during drug development. 1386:). This has made hERG inhibition an important 1362:, which helps coordinate the heart's beating. 893:regulation of heart rate by cardiac conduction 3164: 3088: 1492:. The S4 helix contains a positively charged 1351:: the hERG channel mediates the repolarizing 8: 1643:with the 14-3-3 epsilon protein, encoded by 1593:. The hERG gene is the human homolog of the 726:delayed rectifier potassium channel activity 1713:GRCh38: Ensembl release 89: ENSG00000055118 923:potassium ion import across plasma membrane 833:potassium ion export across plasma membrane 731:inward rectifier potassium channel activity 5333: 5320: 4518: 4505: 3767: 3751: 3555: 3539: 3212: 3196: 3171: 3157: 3149: 3095: 3081: 3073: 953: 777:inward rectifier potassium channel complex 643: 417: 316: 213: 83: 3050:International Conference on Harmonization 2944: 2905: 2831: 2782: 2689: 2588: 2547: 2498: 2449: 2357: 2347: 2277: 2234: 2185: 2136: 2095: 1964: 1806: 818:regulation of ion transmembrane transport 1784: 1782: 1558:hERG containing channels are blocked by 711:voltage-gated potassium channel activity 3110: 1700: 873:phosphorelay signal transduction system 757:voltage-gated potassium channel complex 2119:Abbott GW, Goldstein SA (March 2002). 38: 2757:Kaplan WD, Trout WE (February 1969). 878:regulation of membrane repolarization 863:potassium ion transmembrane transport 782:integral component of plasma membrane 681:C3HC4-type RING finger domain binding 381: 342: 337: 280: 239: 234: 7: 2322:Warmke JW, Ganetzky B (April 1994). 1331:) that codes for a protein known as 4537:Calcium-activated chloride channels 2223:The Journal of Biological Chemistry 1615:. When flies with mutations in the 853:regulation of heart rate by hormone 716:phosphorelay sensor kinase activity 3607:Amiloride-sensitive cation channel 2522:Wang W, MacKinnon R (April 2017). 1213: 1180: 1150: 1117: 1093: 1074: 1048: 1029: 1003: 984: 686:voltage-gated ion channel activity 599: 549:central gray substance of midbrain 533:choroid plexus of fourth ventricle 517: 455: 434: 25: 666:protein homodimerization activity 29:Mammalian protein found in humans 3128: 3113: 1957:10.1111/j.1469-7793.1997.313bn.x 1349:electrical activity of the heart 813:regulation of membrane potential 721:ubiquitin protein ligase binding 617: 610: 603: 365: 358: 352: 329: 264: 257: 251: 226: 47: 5130:Voltage-dependent anion channel 3223:Inositol trisphosphate receptor 2670:British Journal of Pharmacology 1690:Voltage-gated potassium channel 1591:University of Wisconsin–Madison 1572:Drug development considerations 772:perinuclear region of cytoplasm 5412:PAS-domain-containing proteins 5152:General bacterial porin family 747:integral component of membrane 628:More reference expression data 583:More reference expression data 487:right hemisphere of cerebellum 1: 3055:Drosophila flybase entry for 2937:10.1016/S0006-2952(98)00002-1 2590:10.1016/S0092-8674(00)81635-9 2187:10.1016/S0092-8674(00)80728-X 1376:irregularity of the heartbeat 483:muscle layer of sigmoid colon 350: 249: 3067:Schizophrenia Research Forum 2873:10.1146/annurev.med.49.1.263 2743:Food and Drug Administration 2451:10.1016/0092-8674(95)90340-2 2088:10.1113/jphysiol.2001.013296 1619:gene are anaesthetised with 495:myocardium of left ventricle 5402:Genes on human chromosome 7 2481:Robertson GA (March 2000). 1852:10.1016/j.hrthm.2018.02.034 1549:(acquired) long QT syndrome 1380:ventricular tachyarrhythmia 5428: 4571:Chloride Channel Accessory 3180:Membrane transport protein 2888:Ackerman MJ (March 1998). 2540:10.1016/j.cell.2017.03.048 1629:West Hollywood, California 803:cardiac muscle contraction 661:potassium channel activity 31: 5377: 5332: 5319: 4517: 4504: 3766: 3750: 3566:Epithelial sodium channel 3554: 3538: 3211: 3195: 3108: 2861:Annual Review of Medicine 2775:10.1093/genetics/61.2.399 2076:The Journal of Physiology 1945:The Journal of Physiology 1769:"Mouse PubMed Reference:" 1751:"Human PubMed Reference:" 1290: 1285: 1281: 1274: 1258: 1245:Chr 7: 150.94 – 150.98 Mb 1239: 1220: 1187: 1176: 1157: 1124: 1113: 1100: 1096: 1081: 1077: 1068: 1055: 1051: 1036: 1032: 1023: 1010: 1006: 991: 987: 978: 963: 956: 952: 936: 848:potassium ion homeostasis 701:identical protein binding 646: 642: 625: 602: 593: 580: 529: 520: 467: 458: 428: 420: 416: 399: 386: 349: 328: 319: 315: 298: 285: 248: 225: 216: 212: 167: 164: 154: 147: 142: 91: 86: 69: 64: 59: 55: 46: 41: 3483:Cation channels of sperm 2925:Biochemical Pharmacology 1998:10.1007/0-306-46825-5_22 1474:heterologously expressed 1360:cardiac action potential 671:scaffold protein binding 3002:Pharmacological Reviews 2894:Mayo Clinic Proceedings 2824:10.1093/emboj/21.8.1889 2500:10.1161/01.res.86.5.492 2406:10.1126/science.7604285 2039:10.1002/047002142X.ch17 1639:HERG has been shown to 1252:Chr 5: 24.52 – 24.56 Mb 913:membrane repolarization 858:transmembrane transport 838:potassium ion transport 565:interventricular septum 2682:10.1038/sj.bjp.0705335 2349:10.1073/pnas.91.8.3438 2288:10.1002/047002142X.ch2 2236:10.1074/jbc.M408344200 537:neural layer of retina 3559:Constitutively active 2976:10.1038/ncpcardio0097 2635:10.1161/hc0702.105124 2138:10.1096/fj.01-0520hyp 1470:X-ray crystallography 1345:potassium ion channel 2487:Circulation Research 691:ion channel activity 383:5 A3|5 10.94 cM 344:Chromosome 5 (mouse) 242:Chromosome 7 (human) 87:List of PDB id codes 60:Available structures 5326:By gating mechanism 2398:1995Sci...269...92T 2340:1994PNAS...91.3438W 2229:(43): 44690–44694. 1910:10.1038/nature04710 1902:2006Natur.440..463S 1536:torsades de pointes 1384:torsades de pointes 808:signal transduction 4853:TRP cation channel 4751:CNG cation channel 4034:Tandem pore domain 3245:Ryanodine receptor 3014:10.1124/pr.57.4.10 2534:(3): 422–430.e10. 1808:10.1002/humu.21106 1613:Duarte, California 1597:gene found in the 1058:ENSMUSG00000038319 928:cardiac conduction 791:Biological process 740:Cellular component 654:Molecular function 553:epithelium of lens 499:anterior pituitary 5389: 5388: 5373: 5372: 5369: 5368: 5361:Stretch-activated 5338:Ion channel class 5315: 5314: 5311: 5310: 4500: 4499: 4496: 4495: 3772:Calcium-activated 3760:Potassium channel 3746: 3745: 3742: 3741: 3534: 3533: 3530: 3529: 3146: 3145: 2931:(11): 1741–1746. 2007:978-0-306-46367-9 1951:(Pt 2): 313–318. 1896:(7083): 463–469. 1801:(11): 1486–1511. 1543:Drug interactions 1523:short QT syndrome 1372:short QT syndrome 1301: 1300: 1297: 1296: 1270: 1269: 1235: 1234: 1210: 1209: 1172: 1171: 1147: 1146: 1109: 1108: 1090: 1089: 1064: 1063: 1045: 1044: 1019: 1018: 1000: 999: 948: 947: 638: 637: 634: 633: 589: 588: 576: 575: 569:cerebellar cortex 557:medulla oblongata 514: 513: 412: 411: 311: 310: 206:KCNH2 - orthologs 138: 137: 134: 133: 70:Ortholog search: 16:(Redirected from 5419: 5334: 5321: 4529:Chloride channel 4519: 4506: 3899:Inward-rectifier 3768: 3752: 3556: 3540: 3510:Two-pore channel 3213: 3197: 3173: 3166: 3159: 3150: 3132: 3117: 3097: 3090: 3083: 3074: 3033: 2995: 2958: 2948: 2919: 2909: 2907:10.4065/73.3.250 2884: 2846: 2845: 2835: 2818:(8): 1889–1898. 2812:The EMBO Journal 2803: 2797: 2796: 2786: 2754: 2748: 2747: 2739: 2731: 2725: 2724: 2722: 2721: 2710: 2704: 2703: 2693: 2661: 2655: 2654: 2617: 2611: 2610: 2592: 2568: 2562: 2561: 2551: 2519: 2513: 2512: 2502: 2478: 2472: 2471: 2453: 2429: 2418: 2417: 2381: 2372: 2371: 2361: 2351: 2334:(8): 3438–3442. 2319: 2310: 2309: 2281: 2258: 2249: 2248: 2238: 2214: 2208: 2207: 2189: 2165: 2159: 2158: 2140: 2116: 2110: 2109: 2099: 2067: 2061: 2060: 2026: 2020: 2019: 1985: 1979: 1978: 1968: 1936: 1930: 1929: 1885: 1872: 1871: 1846:(8): 1261–1267. 1835: 1829: 1828: 1810: 1786: 1777: 1776: 1765: 1759: 1758: 1747: 1741: 1731: 1720: 1710: 1519:long QT syndrome 1410:cardiac myocytes 1368:long QT syndrome 1325:ene) is a gene ( 1283: 1282: 1254: 1247: 1230: 1214: 1205: 1181: 1177:RefSeq (protein) 1167: 1151: 1142: 1118: 1094: 1075: 1049: 1030: 1004: 985: 954: 644: 630: 621: 614: 607: 600: 585: 525: 523:Top expressed in 518: 463: 461:Top expressed in 456: 435: 418: 408: 395: 384: 369: 362: 356: 345: 333: 317: 307: 294: 283: 268: 261: 255: 244: 230: 214: 208: 159: 152: 129: 84: 78: 57: 56: 51: 39: 21: 5427: 5426: 5422: 5421: 5420: 5418: 5417: 5416: 5392: 5391: 5390: 5385: 5365: 5328: 5307: 5156: 5055: 5045: 4841: 4739: 4715: 4513: 4492: 4406: 4283: 4140: 4123: 4028: 3992: 3962: 3918: 3910: 3893: 3847: 3762: 3738: 3711: 3650: 3633: 3592: 3550: 3526: 3409: 3381: 3368: 3355: 3335: 3322: 3287: 3266: 3207: 3205:Calcium channel 3191: 3177: 3147: 3142: 3139: 3133: 3124: 3118: 3104: 3101: 3041: 3036: 2998: 2961: 2922: 2887: 2858: 2854: 2852:Further reading 2849: 2805: 2804: 2800: 2756: 2755: 2751: 2737: 2733: 2732: 2728: 2719: 2717: 2712: 2711: 2707: 2663: 2662: 2658: 2619: 2618: 2614: 2570: 2569: 2565: 2521: 2520: 2516: 2480: 2479: 2475: 2431: 2430: 2421: 2392:(5220): 92–95. 2383: 2382: 2375: 2321: 2320: 2313: 2298: 2279:10.1.1.512.5443 2268:in the Heart". 2267: 2260: 2259: 2252: 2216: 2215: 2211: 2167: 2166: 2162: 2118: 2117: 2113: 2082:(Pt 1): 15–27. 2069: 2068: 2064: 2049: 2028: 2027: 2023: 2008: 1987: 1986: 1982: 1938: 1937: 1933: 1887: 1886: 1875: 1837: 1836: 1832: 1788: 1787: 1780: 1767: 1766: 1762: 1749: 1748: 1744: 1732: 1723: 1711: 1702: 1698: 1686: 1653: 1637: 1587: 1574: 1545: 1527:channelopathies 1515: 1478:plasma membrane 1466: 1459: 1452: 1437: 1430: 1422: 1407: 1399: 1358:current in the 1357: 1336: 1292:View/Edit Mouse 1287:View/Edit Human 1250: 1243: 1240:Location (UCSC) 1226: 1222: 1201: 1197: 1193: 1189: 1163: 1159: 1138: 1134: 1130: 1126: 1039:ENSG00000055118 932: 786: 762:plasma membrane 735: 696:protein binding 626: 616: 615: 609: 608: 581: 572: 567: 563: 559: 555: 551: 547: 543: 541:right ventricle 539: 535: 521: 510: 505: 501: 497: 493: 491:pituitary gland 489: 485: 481: 477: 473: 459: 403: 390: 382: 372: 371: 370: 363: 343: 320:Gene location ( 302: 289: 281: 271: 270: 269: 262: 240: 217:Gene location ( 168: 155: 148: 93: 71: 37: 34:IKR (ICAO code) 30: 23: 22: 15: 12: 11: 5: 5425: 5423: 5415: 5414: 5409: 5404: 5394: 5393: 5387: 5386: 5378: 5375: 5374: 5371: 5370: 5367: 5366: 5364: 5363: 5358: 5353: 5348: 5342: 5340: 5330: 5329: 5324: 5317: 5316: 5313: 5312: 5309: 5308: 5306: 5305: 5300: 5299: 5298: 5297: 5296: 5291: 5286: 5278: 5277: 5276: 5271: 5266: 5258: 5257: 5256: 5251: 5246: 5241: 5236: 5231: 5226: 5218: 5217: 5216: 5211: 5206: 5201: 5196: 5191: 5186: 5172: 5170: 5158: 5157: 5155: 5154: 5149: 5148: 5147: 5142: 5137: 5127: 5126: 5125: 5120: 5115: 5110: 5105: 5100: 5095: 5090: 5085: 5080: 5069: 5067: 5053: 5047: 5046: 5044: 5043: 5042: 5041: 5036: 5031: 5026: 5021: 5016: 5006: 5005: 5004: 4999: 4989: 4984: 4983: 4982: 4977: 4972: 4962: 4961: 4960: 4955: 4950: 4945: 4940: 4935: 4930: 4925: 4915: 4914: 4913: 4908: 4903: 4898: 4893: 4888: 4883: 4878: 4868: 4857: 4855: 4843: 4842: 4840: 4839: 4838: 4837: 4832: 4827: 4822: 4817: 4805: 4804: 4803: 4798: 4793: 4783: 4782: 4781: 4776: 4771: 4766: 4755: 4753: 4741: 4740: 4738: 4737: 4731: 4729: 4727:Proton channel 4717: 4716: 4714: 4713: 4712: 4711: 4706: 4696: 4695: 4694: 4689: 4684: 4679: 4674: 4669: 4664: 4654: 4653: 4652: 4647: 4642: 4637: 4632: 4627: 4622: 4617: 4612: 4602: 4597: 4596: 4595: 4594: 4593: 4588: 4583: 4578: 4568: 4567: 4566: 4561: 4551: 4550: 4549: 4533: 4531: 4515: 4514: 4509: 4502: 4501: 4498: 4497: 4494: 4493: 4491: 4490: 4485: 4484: 4483: 4478: 4473: 4463: 4458: 4453: 4452: 4451: 4446: 4441: 4436: 4426: 4425: 4424: 4419: 4414: 4404: 4399: 4398: 4397: 4396: 4391: 4386: 4379: 4378: 4377: 4372: 4367: 4360: 4359: 4358: 4353: 4346: 4345: 4344: 4339: 4334: 4327: 4326: 4325: 4320: 4313: 4312: 4311: 4306: 4301: 4296: 4291: 4281: 4276: 4275: 4274: 4273: 4268: 4263: 4258: 4251: 4250: 4249: 4242: 4241: 4240: 4235: 4230: 4223: 4222: 4221: 4216: 4211: 4206: 4199: 4198: 4197: 4192: 4185: 4184: 4183: 4178: 4173: 4168: 4163: 4158: 4153: 4148: 4138: 4133: 4131: 4125: 4124: 4122: 4121: 4120: 4119: 4114: 4109: 4104: 4099: 4094: 4089: 4084: 4079: 4074: 4069: 4064: 4059: 4054: 4049: 4038: 4036: 4030: 4029: 4027: 4026: 4025: 4024: 4019: 4014: 4009: 4004: 3999: 3990: 3986: 3985: 3984: 3979: 3974: 3969: 3960: 3952: 3951: 3950: 3945: 3940: 3935: 3930: 3925: 3916: 3912: 3908: 3903: 3901: 3895: 3894: 3892: 3891: 3890: 3889: 3884: 3879: 3874: 3869: 3864: 3859: 3854: 3845: 3841: 3840: 3839: 3831: 3830: 3829: 3824: 3819: 3809: 3808: 3807: 3802: 3797: 3792: 3787: 3776: 3774: 3764: 3763: 3755: 3748: 3747: 3744: 3743: 3740: 3739: 3737: 3736: 3735: 3734: 3729: 3724: 3719: 3709: 3705: 3704: 3703: 3698: 3693: 3688: 3683: 3678: 3673: 3668: 3663: 3658: 3648: 3643: 3641: 3635: 3634: 3632: 3631: 3630: 3629: 3624: 3619: 3614: 3603: 3601: 3594: 3593: 3591: 3590: 3589: 3588: 3583: 3578: 3573: 3562: 3560: 3552: 3551: 3548:Sodium channel 3543: 3536: 3535: 3532: 3531: 3528: 3527: 3525: 3524: 3523: 3522: 3517: 3507: 3506: 3505: 3500: 3495: 3490: 3479: 3478: 3477: 3476: 3471: 3466: 3461: 3451: 3450: 3449: 3444: 3439: 3434: 3424: 3423: 3422: 3417: 3407: 3402: 3401: 3400: 3399: 3394: 3389: 3379: 3371: 3366: 3358: 3353: 3345: 3344: 3343: 3333: 3325: 3320: 3312: 3311: 3310: 3305: 3300: 3295: 3285: 3276: 3274: 3268: 3267: 3265: 3264: 3263: 3262: 3257: 3252: 3242: 3241: 3240: 3235: 3230: 3219: 3217: 3209: 3208: 3200: 3193: 3192: 3178: 3176: 3175: 3168: 3161: 3153: 3144: 3143: 3141: 3140: 3134: 3127: 3125: 3119: 3112: 3109: 3106: 3105: 3102: 3100: 3099: 3092: 3085: 3077: 3071: 3070: 3060: 3052: 3047: 3040: 3039:External links 3037: 3035: 3034: 3008:(4): 473–508. 2996: 2959: 2920: 2900:(3): 250–269. 2885: 2855: 2853: 2850: 2848: 2847: 2798: 2769:(2): 399–409. 2749: 2726: 2705: 2676:(5): 887–898. 2656: 2629:(7): 794–799. 2612: 2583:(5): 649–655. 2563: 2514: 2493:(5): 492–493. 2473: 2444:(2): 299–307. 2419: 2373: 2311: 2296: 2265: 2250: 2209: 2180:(2): 175–187. 2160: 2131:(3): 390–400. 2111: 2062: 2047: 2021: 2006: 1990:Oxygen Sensing 1980: 1931: 1873: 1830: 1795:Human Mutation 1778: 1760: 1742: 1721: 1699: 1697: 1694: 1693: 1692: 1685: 1682: 1681: 1680: 1674: 1669: 1664: 1659: 1652: 1649: 1636: 1633: 1625:Whisky a Go Go 1586: 1583: 1573: 1570: 1544: 1541: 1514: 1511: 1465: 1462: 1457: 1450: 1435: 1428: 1420: 1414:repolarization 1405: 1398: 1395: 1355: 1334: 1299: 1298: 1295: 1294: 1289: 1279: 1278: 1272: 1271: 1268: 1267: 1265: 1263: 1256: 1255: 1248: 1241: 1237: 1236: 1233: 1232: 1218: 1217: 1211: 1208: 1207: 1185: 1184: 1178: 1174: 1173: 1170: 1169: 1155: 1154: 1148: 1145: 1144: 1122: 1121: 1115: 1111: 1110: 1107: 1106: 1098: 1097: 1091: 1088: 1087: 1079: 1078: 1072: 1066: 1065: 1062: 1061: 1053: 1052: 1046: 1043: 1042: 1034: 1033: 1027: 1021: 1020: 1017: 1016: 1008: 1007: 1001: 998: 997: 989: 988: 982: 976: 975: 970: 965: 961: 960: 950: 949: 946: 945: 934: 933: 931: 930: 925: 920: 915: 910: 905: 900: 895: 890: 885: 880: 875: 870: 865: 860: 855: 850: 845: 840: 835: 830: 825: 820: 815: 810: 805: 800: 794: 792: 788: 787: 785: 784: 779: 774: 769: 764: 759: 754: 749: 743: 741: 737: 736: 734: 733: 728: 723: 718: 713: 708: 703: 698: 693: 688: 683: 678: 673: 668: 663: 657: 655: 651: 650: 640: 639: 636: 635: 632: 631: 623: 622: 597: 591: 590: 587: 586: 578: 577: 574: 573: 571: 570: 566: 562: 558: 554: 550: 546: 542: 538: 534: 530: 527: 526: 515: 512: 511: 509: 508: 504: 500: 496: 492: 488: 484: 480: 479:left ventricle 476: 472: 468: 465: 464: 452: 451: 443: 432: 426: 425: 422:RNA expression 414: 413: 410: 409: 401: 397: 396: 388: 385: 380: 374: 373: 364: 357: 351: 347: 346: 341: 335: 334: 326: 325: 313: 312: 309: 308: 300: 296: 295: 287: 284: 279: 273: 272: 263: 256: 250: 246: 245: 238: 232: 231: 223: 222: 210: 209: 166: 162: 161: 153: 145: 144: 140: 139: 136: 135: 132: 131: 89: 88: 80: 79: 68: 62: 61: 53: 52: 44: 43: 28: 24: 14: 13: 10: 9: 6: 4: 3: 2: 5424: 5413: 5410: 5408: 5405: 5403: 5400: 5399: 5397: 5384: 5383: 5376: 5362: 5359: 5357: 5356:Voltage-gated 5354: 5352: 5349: 5347: 5344: 5343: 5341: 5339: 5335: 5331: 5327: 5322: 5318: 5304: 5301: 5295: 5292: 5290: 5287: 5285: 5282: 5281: 5279: 5275: 5272: 5270: 5267: 5265: 5262: 5261: 5259: 5255: 5252: 5250: 5247: 5245: 5242: 5240: 5237: 5235: 5232: 5230: 5227: 5225: 5222: 5221: 5219: 5215: 5212: 5210: 5207: 5205: 5202: 5200: 5197: 5195: 5192: 5190: 5187: 5185: 5182: 5181: 5179: 5178: 5177: 5174: 5173: 5171: 5169: 5166: 5164: 5159: 5153: 5150: 5146: 5143: 5141: 5138: 5136: 5133: 5132: 5131: 5128: 5124: 5121: 5119: 5116: 5114: 5111: 5109: 5106: 5104: 5101: 5099: 5096: 5094: 5091: 5089: 5086: 5084: 5081: 5079: 5076: 5075: 5074: 5071: 5070: 5068: 5066: 5063: 5061: 5057: 5048: 5040: 5037: 5035: 5032: 5030: 5027: 5025: 5022: 5020: 5017: 5015: 5012: 5011: 5010: 5007: 5003: 5000: 4998: 4995: 4994: 4993: 4990: 4988: 4985: 4981: 4978: 4976: 4973: 4971: 4968: 4967: 4966: 4963: 4959: 4956: 4954: 4951: 4949: 4946: 4944: 4941: 4939: 4936: 4934: 4931: 4929: 4926: 4924: 4921: 4920: 4919: 4916: 4912: 4909: 4907: 4904: 4902: 4899: 4897: 4894: 4892: 4889: 4887: 4884: 4882: 4879: 4877: 4874: 4873: 4872: 4869: 4866: 4862: 4859: 4858: 4856: 4854: 4851: 4849: 4844: 4836: 4833: 4831: 4828: 4826: 4823: 4821: 4818: 4816: 4813: 4812: 4811: 4810: 4806: 4802: 4799: 4797: 4794: 4792: 4789: 4788: 4787: 4784: 4780: 4777: 4775: 4772: 4770: 4767: 4765: 4762: 4761: 4760: 4757: 4756: 4754: 4752: 4749: 4747: 4742: 4736: 4733: 4732: 4730: 4728: 4725: 4723: 4718: 4710: 4707: 4705: 4702: 4701: 4700: 4697: 4693: 4690: 4688: 4685: 4683: 4680: 4678: 4675: 4673: 4670: 4668: 4665: 4663: 4660: 4659: 4658: 4655: 4651: 4648: 4646: 4643: 4641: 4638: 4636: 4633: 4631: 4628: 4626: 4623: 4621: 4618: 4616: 4613: 4611: 4608: 4607: 4606: 4603: 4601: 4598: 4592: 4589: 4587: 4584: 4582: 4579: 4577: 4574: 4573: 4572: 4569: 4565: 4562: 4560: 4557: 4556: 4555: 4552: 4548: 4545: 4544: 4543: 4540: 4539: 4538: 4535: 4534: 4532: 4530: 4527: 4525: 4520: 4516: 4512: 4511:Miscellaneous 4507: 4503: 4489: 4488:Shaker (gene) 4486: 4482: 4479: 4477: 4474: 4472: 4469: 4468: 4467: 4464: 4462: 4461:minK/ISK-like 4459: 4457: 4454: 4450: 4447: 4445: 4442: 4440: 4437: 4435: 4432: 4431: 4430: 4427: 4423: 4420: 4418: 4415: 4413: 4410: 4409: 4408: 4401: 4400: 4395: 4392: 4390: 4387: 4385: 4382: 4381: 4380: 4376: 4373: 4371: 4368: 4366: 4363: 4362: 4361: 4357: 4354: 4352: 4349: 4348: 4347: 4343: 4340: 4338: 4335: 4333: 4330: 4329: 4328: 4324: 4321: 4319: 4316: 4315: 4314: 4310: 4307: 4305: 4302: 4300: 4297: 4295: 4292: 4290: 4287: 4286: 4285: 4278: 4277: 4272: 4269: 4267: 4264: 4262: 4259: 4257: 4254: 4253: 4252: 4248: 4245: 4244: 4243: 4239: 4236: 4234: 4231: 4229: 4226: 4225: 4224: 4220: 4217: 4215: 4212: 4210: 4207: 4205: 4202: 4201: 4200: 4196: 4193: 4191: 4188: 4187: 4186: 4182: 4179: 4177: 4174: 4172: 4169: 4167: 4164: 4162: 4159: 4157: 4154: 4152: 4149: 4147: 4144: 4143: 4142: 4135: 4134: 4132: 4130: 4129:Voltage-gated 4126: 4118: 4115: 4113: 4110: 4108: 4105: 4103: 4100: 4098: 4095: 4093: 4090: 4088: 4085: 4083: 4080: 4078: 4075: 4073: 4070: 4068: 4065: 4063: 4060: 4058: 4055: 4053: 4050: 4048: 4045: 4044: 4043: 4040: 4039: 4037: 4035: 4031: 4023: 4020: 4018: 4015: 4013: 4010: 4008: 4005: 4003: 4000: 3998: 3995: 3994: 3993: 3987: 3983: 3980: 3978: 3975: 3973: 3970: 3968: 3965: 3964: 3963: 3956: 3953: 3949: 3946: 3944: 3941: 3939: 3936: 3934: 3931: 3929: 3926: 3924: 3921: 3920: 3919: 3913: 3911: 3905: 3904: 3902: 3900: 3896: 3888: 3885: 3883: 3880: 3878: 3875: 3873: 3870: 3868: 3865: 3863: 3860: 3858: 3855: 3853: 3850: 3849: 3848: 3842: 3838: 3835: 3834: 3832: 3828: 3825: 3823: 3820: 3818: 3815: 3814: 3813: 3810: 3806: 3803: 3801: 3798: 3796: 3793: 3791: 3788: 3786: 3783: 3782: 3781: 3778: 3777: 3775: 3773: 3769: 3765: 3761: 3758: 3753: 3749: 3733: 3730: 3728: 3725: 3723: 3720: 3718: 3715: 3714: 3713: 3706: 3702: 3699: 3697: 3694: 3692: 3689: 3687: 3684: 3682: 3679: 3677: 3674: 3672: 3669: 3667: 3664: 3662: 3659: 3657: 3654: 3653: 3652: 3645: 3644: 3642: 3640: 3639:Voltage-gated 3636: 3628: 3625: 3623: 3620: 3618: 3615: 3613: 3610: 3609: 3608: 3605: 3604: 3602: 3599: 3595: 3587: 3584: 3582: 3579: 3577: 3574: 3572: 3569: 3568: 3567: 3564: 3563: 3561: 3557: 3553: 3549: 3546: 3541: 3537: 3521: 3518: 3516: 3513: 3512: 3511: 3508: 3504: 3501: 3499: 3496: 3494: 3491: 3489: 3486: 3485: 3484: 3481: 3480: 3475: 3472: 3470: 3467: 3465: 3462: 3460: 3457: 3456: 3455: 3452: 3448: 3445: 3443: 3440: 3438: 3435: 3433: 3430: 3429: 3428: 3425: 3421: 3418: 3416: 3413: 3412: 3411: 3404: 3403: 3398: 3395: 3393: 3390: 3388: 3385: 3384: 3383: 3375: 3372: 3370: 3362: 3359: 3357: 3349: 3346: 3342: 3339: 3338: 3337: 3329: 3326: 3324: 3316: 3313: 3309: 3306: 3304: 3301: 3299: 3296: 3294: 3291: 3290: 3289: 3281: 3278: 3277: 3275: 3273: 3272:Voltage-gated 3269: 3261: 3258: 3256: 3253: 3251: 3248: 3247: 3246: 3243: 3239: 3236: 3234: 3231: 3229: 3226: 3225: 3224: 3221: 3220: 3218: 3214: 3210: 3206: 3203: 3198: 3194: 3189: 3185: 3181: 3174: 3169: 3167: 3162: 3160: 3155: 3154: 3151: 3137: 3131: 3126: 3122: 3116: 3111: 3107: 3098: 3093: 3091: 3086: 3084: 3079: 3078: 3075: 3068: 3064: 3061: 3059: 3058: 3053: 3051: 3048: 3046: 3043: 3042: 3038: 3031: 3027: 3023: 3019: 3015: 3011: 3007: 3003: 2997: 2993: 2989: 2985: 2981: 2977: 2973: 2969: 2965: 2960: 2956: 2952: 2947: 2942: 2938: 2934: 2930: 2926: 2921: 2917: 2913: 2908: 2903: 2899: 2895: 2891: 2886: 2882: 2878: 2874: 2870: 2866: 2862: 2857: 2856: 2851: 2843: 2839: 2834: 2829: 2825: 2821: 2817: 2813: 2809: 2802: 2799: 2794: 2790: 2785: 2780: 2776: 2772: 2768: 2764: 2760: 2753: 2750: 2746:. 6 May 2020. 2745: 2744: 2736: 2730: 2727: 2715: 2709: 2706: 2701: 2697: 2692: 2687: 2683: 2679: 2675: 2671: 2667: 2660: 2657: 2652: 2648: 2644: 2640: 2636: 2632: 2628: 2624: 2616: 2613: 2608: 2604: 2600: 2596: 2591: 2586: 2582: 2578: 2574: 2567: 2564: 2559: 2555: 2550: 2545: 2541: 2537: 2533: 2529: 2525: 2518: 2515: 2510: 2506: 2501: 2496: 2492: 2488: 2484: 2477: 2474: 2469: 2465: 2461: 2457: 2452: 2447: 2443: 2439: 2435: 2428: 2426: 2424: 2420: 2415: 2411: 2407: 2403: 2399: 2395: 2391: 2387: 2380: 2378: 2374: 2369: 2365: 2360: 2355: 2350: 2345: 2341: 2337: 2333: 2329: 2325: 2318: 2316: 2312: 2307: 2303: 2299: 2297:9780470021408 2293: 2289: 2285: 2280: 2275: 2271: 2264: 2257: 2255: 2251: 2246: 2242: 2237: 2232: 2228: 2224: 2220: 2213: 2210: 2205: 2201: 2197: 2193: 2188: 2183: 2179: 2175: 2171: 2164: 2161: 2156: 2152: 2148: 2144: 2139: 2134: 2130: 2126: 2125:FASEB Journal 2122: 2115: 2112: 2107: 2103: 2098: 2093: 2089: 2085: 2081: 2077: 2073: 2066: 2063: 2058: 2054: 2050: 2048:9780470021408 2044: 2040: 2036: 2032: 2025: 2022: 2017: 2013: 2009: 2003: 1999: 1995: 1991: 1984: 1981: 1976: 1972: 1967: 1962: 1958: 1954: 1950: 1946: 1942: 1935: 1932: 1927: 1923: 1919: 1915: 1911: 1907: 1903: 1899: 1895: 1891: 1884: 1882: 1880: 1878: 1874: 1869: 1865: 1861: 1857: 1853: 1849: 1845: 1841: 1834: 1831: 1826: 1822: 1818: 1814: 1809: 1804: 1800: 1796: 1792: 1785: 1783: 1779: 1774: 1770: 1764: 1761: 1756: 1752: 1746: 1743: 1739: 1735: 1730: 1728: 1726: 1722: 1718: 1714: 1709: 1707: 1705: 1701: 1695: 1691: 1688: 1687: 1683: 1678: 1675: 1673: 1670: 1668: 1665: 1663: 1660: 1658: 1655: 1654: 1650: 1648: 1646: 1642: 1634: 1632: 1630: 1627:nightclub in 1626: 1622: 1618: 1617:Ether-à-go-go 1614: 1610: 1606: 1605:Ether-à-go-go 1602: 1601: 1596: 1595:Ether-à-go-go 1592: 1584: 1582: 1581:environment. 1580: 1571: 1569: 1567: 1563: 1561: 1556: 1552: 1550: 1542: 1540: 1538: 1537: 1532: 1528: 1524: 1520: 1512: 1510: 1508: 1504: 1503:KcsA channels 1499: 1495: 1491: 1487: 1483: 1482:alpha helices 1479: 1475: 1471: 1463: 1461: 1456: 1453:), including 1449: 1445: 1441: 1434: 1427: 1419: 1415: 1411: 1404: 1396: 1394: 1391: 1389: 1385: 1381: 1377: 1373: 1369: 1363: 1361: 1354: 1350: 1346: 1342: 1338: 1330: 1329: 1324: 1320: 1316: 1314: 1309: 1305: 1293: 1288: 1284: 1280: 1277: 1273: 1266: 1264: 1261: 1257: 1253: 1249: 1246: 1242: 1238: 1231: 1229: 1225: 1219: 1215: 1212: 1206: 1204: 1200: 1196: 1192: 1186: 1182: 1179: 1175: 1168: 1166: 1162: 1156: 1152: 1149: 1143: 1141: 1137: 1133: 1129: 1123: 1119: 1116: 1114:RefSeq (mRNA) 1112: 1105: 1104: 1099: 1095: 1092: 1086: 1085: 1080: 1076: 1073: 1071: 1067: 1060: 1059: 1054: 1050: 1047: 1041: 1040: 1035: 1031: 1028: 1026: 1022: 1015: 1014: 1009: 1005: 1002: 996: 995: 990: 986: 983: 981: 977: 974: 971: 969: 966: 962: 959: 955: 951: 944: 940: 935: 929: 926: 924: 921: 919: 916: 914: 911: 909: 906: 904: 901: 899: 896: 894: 891: 889: 886: 884: 881: 879: 876: 874: 871: 869: 866: 864: 861: 859: 856: 854: 851: 849: 846: 844: 841: 839: 836: 834: 831: 829: 828:ion transport 826: 824: 821: 819: 816: 814: 811: 809: 806: 804: 801: 799: 796: 795: 793: 790: 789: 783: 780: 778: 775: 773: 770: 768: 765: 763: 760: 758: 755: 753: 750: 748: 745: 744: 742: 739: 738: 732: 729: 727: 724: 722: 719: 717: 714: 712: 709: 707: 704: 702: 699: 697: 694: 692: 689: 687: 684: 682: 679: 677: 674: 672: 669: 667: 664: 662: 659: 658: 656: 653: 652: 649: 648:Gene ontology 645: 641: 629: 624: 620: 613: 606: 601: 598: 596: 592: 584: 579: 568: 564: 560: 556: 552: 548: 544: 540: 536: 532: 531: 528: 524: 519: 516: 506: 502: 498: 494: 490: 486: 482: 478: 475:right auricle 474: 471:apex of heart 470: 469: 466: 462: 457: 454: 453: 450: 448: 444: 442: 441: 437: 436: 433: 431: 427: 423: 419: 415: 407: 402: 398: 394: 389: 379: 375: 368: 361: 355: 348: 340: 336: 332: 327: 323: 318: 314: 306: 301: 297: 293: 288: 278: 274: 267: 260: 254: 247: 243: 237: 233: 229: 224: 220: 215: 211: 207: 203: 199: 195: 191: 187: 183: 179: 175: 171: 163: 158: 151: 146: 141: 130: 128: 124: 120: 116: 112: 108: 104: 100: 96: 90: 85: 82: 81: 77: 74: 67: 63: 58: 54: 50: 45: 40: 35: 27: 19: 5407:Ion channels 5379: 5346:Ligand-gated 5337: 5325: 5168:Gap junction 5161: 5050: 4846: 4807: 4785: 4758: 4744: 4720: 4698: 4656: 4604: 4570: 4553: 4541: 4522: 4510: 4465: 4428: 4402: 4364: 4279: 4136: 4041: 3988: 3958: 3914: 3843: 3756: 3707: 3646: 3544: 3453: 3426: 3405: 3377: 3364: 3351: 3331: 3318: 3283: 3216:Ligand-gated 3201: 3184:ion channels 3135: 3120: 3056: 3005: 3001: 2970:(2): 84–87. 2967: 2963: 2928: 2924: 2897: 2893: 2864: 2860: 2815: 2811: 2801: 2766: 2762: 2752: 2741: 2729: 2718:. Retrieved 2714:"Amiodarone" 2708: 2673: 2669: 2659: 2626: 2622: 2615: 2580: 2576: 2566: 2531: 2527: 2517: 2490: 2486: 2476: 2441: 2437: 2389: 2385: 2331: 2327: 2269: 2262: 2226: 2222: 2212: 2177: 2173: 2163: 2128: 2124: 2114: 2079: 2075: 2065: 2030: 2024: 1989: 1983: 1948: 1944: 1934: 1893: 1889: 1843: 1840:Heart Rhythm 1839: 1833: 1798: 1794: 1772: 1763: 1754: 1745: 1638: 1635:Interactions 1616: 1611:Hospital in 1609:City of Hope 1604: 1598: 1594: 1588: 1575: 1566:Thioridazine 1564: 1557: 1553: 1546: 1534: 1516: 1467: 1454: 1447: 1444:heterologous 1432: 1425: 1417: 1402: 1400: 1392: 1364: 1352: 1339:, the alpha 1332: 1326: 1322: 1318: 1315:ther-à-go-go 1312: 1311: 1307: 1303: 1302: 1224:NP_001281091 1221: 1195:NP_001191727 1188: 1161:NM_001294162 1158: 1132:NM_001204798 1125: 1101: 1082: 1056: 1037: 1011: 992: 972: 967: 767:cell surface 503:right testis 445: 438: 303:150,978,321 290:150,944,961 165:External IDs 92: 26: 5351:Light-gated 3833:IK channel 3103:PDB gallery 2946:11566/37313 2867:: 263–274. 2623:Circulation 1679:(GBR-12909) 1672:Terfenadine 1662:Domperidone 1531:arrhythmias 507:left testis 404:24,556,602 391:24,524,587 143:Identifiers 5396:Categories 4554:Bestrophin 3812:SK channel 3780:BK channel 3454:γ-subunits 3427:β-subunits 3410:δ-subunits 2720:2019-05-28 2716:. Drugbank 1740:, May 2017 1719:, May 2017 1696:References 1677:Vanoxerine 1667:Sertindole 1657:Astemizole 1651:Inhibitors 1600:Drosophila 1560:amiodarone 1507:PAS domain 1388:antitarget 449:(ortholog) 186:HomoloGene 5382:disorders 5380:see also 5163:Cytoplasm 5073:Aquaporin 4542:Anoctamin 4365:11.1/hERG 3030:219195192 2274:CiteSeerX 1490:C-termini 1464:Structure 1228:NP_038597 1203:NP_742054 1199:NP_742053 1191:NP_000229 1165:NM_013569 1140:NM_172057 1136:NM_172056 1128:NM_000238 958:Orthologs 194:GeneCards 5176:Connexin 4456:minK/ISK 3022:16382104 2992:10125533 2984:16265378 2842:11953308 2763:Genetics 2700:12839862 2643:11854117 2607:15090987 2558:28431243 2509:10720408 2306:16050259 2245:15304481 2196:10219239 2155:26205057 2147:11874988 2106:11927665 2057:16050271 2016:10849664 1918:16554806 1860:29501667 1825:19122696 1817:19862833 1736:– 1715:– 1684:See also 1641:interact 1513:Genetics 1494:arginine 1440:isoforms 1397:Function 1276:Wikidata 937:Sources: 752:membrane 5303:Innexin 5060:solutes 2955:9714291 2916:9511785 2881:9509262 2793:5807804 2784:1212165 2691:1573929 2651:2616052 2599:9845367 2549:5484391 2460:7736582 2414:7604285 2394:Bibcode 2386:Science 2368:8159766 2336:Bibcode 2204:8507168 2097:2290231 1975:9192303 1966:1159479 1898:Bibcode 1868:4519580 1738:Ensembl 1717:Ensembl 1533:(e.g., 1382:called 1341:subunit 1321:elated 1070:UniProt 1025:Ensembl 964:Species 943:QuickGO 424:pattern 182:1341722 150:Aliases 3600:-gated 3598:Proton 3374:T-type 3361:R-type 3348:Q-type 3328:P-type 3315:N-type 3280:L-type 3028:  3020:  2990:  2982:  2953:  2914:  2879:  2840:  2833:125975 2830:  2791:  2781:  2698:  2688:  2649:  2641:  2605:  2597:  2556:  2546:  2507:  2468:240044 2466:  2458:  2412:  2366:  2356:  2304:  2294:  2276:  2243:  2202:  2194:  2153:  2145:  2104:  2094:  2055:  2045:  2014:  2004:  1973:  1963:  1926:929837 1924:  1916:  1890:Nature 1866:  1858:  1823:  1815:  1603:fly; 1585:Naming 1498:lysine 1262:search 1260:PubMed 1103:O35219 1084:Q12809 980:Entrez 595:BioGPS 561:atrium 282:7q36.1 174:152427 5214:GJA10 5065:Porin 4965:TRPML 4735:HVCN1 4429:KCNIP 4284:α7-12 3188:TC 1A 3026:S2CID 2988:S2CID 2738:(PDF) 2647:S2CID 2603:S2CID 2464:S2CID 2359:43592 2200:S2CID 2151:S2CID 1922:S2CID 1864:S2CID 1821:S2CID 1645:YWHAE 1621:ether 1343:of a 1328:KCNH2 1310:uman 1306:(the 1013:16511 973:Mouse 968:Human 939:Amigo 447:Mouse 440:Human 387:Start 322:Mouse 286:Start 219:Human 198:KCNH2 157:KCNH2 42:KCNH2 18:KCNH2 5294:GJD4 5289:GJD3 5284:GJD2 5274:GJC3 5269:GJC2 5264:GJC1 5254:GJB7 5249:GJB6 5244:GJB5 5239:GJB4 5234:GJB3 5229:GJB2 5224:GJB1 5209:GJA9 5204:GJA8 5199:GJA5 5194:GJA4 5189:GJA3 5184:GJA1 5009:TRPV 4992:TRPP 4987:TRPN 4918:TRPM 4871:TRPC 4861:TRPA 4699:CLNS 4657:CLIC 4605:CLCN 4600:CFTR 4547:ANO1 4466:MiRP 4394:12.3 4389:12.2 4384:12.1 4375:11.3 4370:11.2 4356:10.2 4351:10.1 4141:α1-6 3955:GIRK 3369:α2.3 3356:α2.1 3323:α2.2 3136:1ujl 3121:1byw 3018:PMID 2980:PMID 2951:PMID 2912:PMID 2877:PMID 2838:PMID 2789:PMID 2696:PMID 2639:PMID 2595:PMID 2577:Cell 2554:PMID 2528:Cell 2505:PMID 2456:PMID 2438:Cell 2410:PMID 2364:PMID 2302:PMID 2292:ISBN 2241:PMID 2192:PMID 2174:Cell 2143:PMID 2102:PMID 2053:PMID 2043:ISBN 2012:PMID 2002:ISBN 1971:PMID 1914:PMID 1856:PMID 1813:PMID 1488:and 1337:11.1 1304:hERG 994:3757 545:lens 430:Bgee 378:Band 339:Chr. 277:Band 236:Chr. 170:OMIM 127:2N7G 123:4HQA 119:4HP9 115:2LE7 111:2L4R 107:2L1M 103:2L0W 99:1UJL 95:1BYW 76:RCSB 73:PDBe 5058:(+ 4896:4AP 4809:HCN 4342:9.3 4337:9.2 4332:9.1 4323:8.2 4318:8.1 4309:7.5 4304:7.4 4299:7.3 4294:7.2 4289:7.1 4271:6.4 4266:6.3 4261:6.2 4256:6.1 4247:5.1 4238:4.3 4233:4.2 4228:4.1 4219:3.4 4214:3.3 4209:3.2 4204:3.1 4195:2.2 4190:2.1 4181:1.8 4176:1.7 4171:1.6 4166:1.5 4161:1.4 4156:1.3 4151:1.2 4146:1.1 4042:K2P 4022:7.1 4017:6.2 4012:6.1 4007:5.1 4002:4.2 3997:4.1 3982:3.4 3977:3.3 3972:3.2 3967:3.1 3948:2.6 3943:2.4 3938:2.3 3933:2.2 3928:2.1 3923:1.1 3909:ATP 3887:5.1 3882:4.2 3877:4.1 3872:3.1 3867:2.3 3862:2.2 3857:2.1 3852:1.1 3837:IK1 3827:SK3 3822:SK2 3817:SK1 3696:1.9 3691:1.8 3686:1.7 3681:1.6 3676:1.5 3671:1.4 3666:1.3 3661:1.2 3656:1.1 3545:Na: 3397:3.3 3392:3.2 3387:3.1 3341:2.1 3308:1.4 3303:1.3 3298:1.2 3293:1.1 3202:Ca: 3057:erg 3010:doi 2972:doi 2941:hdl 2933:doi 2902:doi 2869:doi 2828:PMC 2820:doi 2779:PMC 2771:doi 2686:PMC 2678:doi 2674:139 2631:doi 2627:105 2585:doi 2544:PMC 2536:doi 2532:169 2495:doi 2446:doi 2402:doi 2390:269 2354:PMC 2344:doi 2284:doi 2231:doi 2227:279 2182:doi 2133:doi 2092:PMC 2084:doi 2080:540 2035:doi 1994:doi 1961:PMC 1953:doi 1949:501 1906:doi 1894:440 1848:doi 1803:doi 1579:GLP 1496:or 1378:(a 400:End 299:End 202:OMA 190:201 178:MGI 66:PDB 5398:: 5280:D 5260:C 5220:B 5180:A 5062:): 4815:FC 4709:1B 4704:1A 4692:L1 4650:KB 4645:KA 4524:Cl 4117:18 4112:17 4107:16 4102:15 4097:13 4092:12 4087:10 3991:ir 3961:ir 3917:ir 3846:Ca 3805:β4 3800:β3 3795:β2 3790:β1 3785:α1 3757:K: 3708:Na 3701:7A 3647:Na 3474:γ4 3469:γ3 3464:γ2 3459:γ1 3447:β4 3442:β3 3437:β2 3432:β1 3378:Ca 3365:Ca 3352:Ca 3332:Ca 3319:Ca 3284:Ca 3182:: 3065:- 3024:. 3016:. 3006:57 3004:. 2986:. 2978:. 2966:. 2949:. 2939:. 2929:55 2927:. 2910:. 2898:73 2896:. 2892:. 2875:. 2865:49 2863:. 2836:. 2826:. 2816:21 2814:. 2810:. 2787:. 2777:. 2767:61 2765:. 2761:. 2740:. 2694:. 2684:. 2672:. 2668:. 2645:. 2637:. 2625:. 2601:. 2593:. 2581:95 2579:. 2575:. 2552:. 2542:. 2530:. 2526:. 2503:. 2491:86 2489:. 2485:. 2462:. 2454:. 2442:81 2440:. 2436:. 2422:^ 2408:. 2400:. 2388:. 2376:^ 2362:. 2352:. 2342:. 2332:91 2330:. 2326:. 2314:^ 2300:. 2290:. 2282:. 2266:Kr 2253:^ 2239:. 2225:. 2221:. 2198:. 2190:. 2178:97 2176:. 2172:. 2149:. 2141:. 2129:16 2127:. 2123:. 2100:. 2090:. 2078:. 2074:. 2051:. 2041:. 2010:. 2000:. 1969:. 1959:. 1947:. 1943:. 1920:. 1912:. 1904:. 1892:. 1876:^ 1862:. 1854:. 1844:15 1842:. 1819:. 1811:. 1799:30 1797:. 1793:. 1781:^ 1771:. 1753:. 1724:^ 1703:^ 1647:. 1631:. 1486:N- 1458:Kr 1451:Kr 1436:Kr 1429:Kr 1421:Kr 1406:Kr 1356:Kr 941:/ 406:bp 393:bp 305:bp 292:bp 200:; 196:: 192:; 188:: 184:; 180:: 176:; 172:: 125:, 121:, 117:, 113:, 109:, 105:, 101:, 97:, 5165:: 5145:3 5140:2 5135:1 5123:9 5118:8 5113:7 5108:6 5103:5 5098:4 5093:3 5088:2 5083:1 5078:0 5056:O 5054:2 5052:H 5039:6 5034:5 5029:4 5024:3 5019:2 5014:1 5002:2 4997:1 4980:3 4975:2 4970:1 4958:8 4953:7 4948:6 4943:5 4938:4 4933:3 4928:2 4923:1 4911:7 4906:6 4901:5 4891:4 4886:3 4881:2 4876:1 4867:) 4865:1 4863:( 4850:: 4848:M 4835:4 4830:3 4825:2 4820:1 4801:3 4796:2 4791:1 4786:β 4779:4 4774:3 4769:2 4764:1 4759:α 4748:: 4746:M 4724:: 4722:H 4687:6 4682:5 4677:4 4672:3 4667:2 4662:1 4640:7 4635:6 4630:5 4625:4 4620:3 4615:2 4610:1 4591:4 4586:3 4581:2 4576:1 4564:2 4559:1 4526:: 4481:3 4476:2 4471:1 4449:4 4444:3 4439:2 4434:1 4422:3 4417:2 4412:1 4407:β 4405:v 4403:K 4282:v 4280:K 4139:v 4137:K 4082:9 4077:7 4072:6 4067:5 4062:4 4057:3 4052:2 4047:1 3989:K 3959:K 3957:/ 3915:K 3907:K 3844:K 3732:4 3727:3 3722:2 3717:1 3712:β 3710:v 3651:α 3649:v 3627:4 3622:3 3617:2 3612:1 3586:δ 3581:γ 3576:β 3571:α 3520:2 3515:1 3503:4 3498:3 3493:2 3488:1 3420:2 3415:1 3408:2 3406:α 3382:α 3380:v 3376:/ 3367:v 3363:/ 3354:v 3350:/ 3336:α 3334:v 3330:/ 3321:v 3317:/ 3288:α 3286:v 3282:/ 3260:3 3255:2 3250:1 3238:3 3233:2 3228:1 3190:) 3186:( 3172:e 3165:t 3158:v 3096:e 3089:t 3082:v 3069:. 3032:. 3012:: 2994:. 2974:: 2968:2 2957:. 2943:: 2935:: 2918:. 2904:: 2883:. 2871:: 2844:. 2822:: 2795:. 2773:: 2723:. 2702:. 2680:: 2653:. 2633:: 2609:. 2587:: 2560:. 2538:: 2511:. 2497:: 2470:. 2448:: 2416:. 2404:: 2396:: 2370:. 2346:: 2338:: 2308:. 2286:: 2263:I 2247:. 2233:: 2206:. 2184:: 2157:. 2135:: 2108:. 2086:: 2059:. 2037:: 2018:. 1996:: 1977:. 1955:: 1928:. 1908:: 1900:: 1870:. 1850:: 1827:. 1805:: 1775:. 1757:. 1455:I 1448:I 1433:I 1426:I 1418:I 1403:I 1353:I 1335:v 1333:K 1323:G 1319:R 1317:- 1313:E 1308:h 324:) 221:) 204:: 36:. 20:)

Index

KCNH2
IKR (ICAO code)

PDB
PDBe
RCSB
1BYW
1UJL
2L0W
2L1M
2L4R
2LE7
4HP9
4HQA
2N7G
Aliases
KCNH2
OMIM
152427
MGI
1341722
HomoloGene
201
GeneCards
KCNH2
OMA
KCNH2 - orthologs
Human
Chromosome 7 (human)
Chr.

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