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Gene and Protein Information  |
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| Species | TM | P Loops | AA | Chromosomal Location | Gene Symbol | Gene Name | Reference |
| Human | 6 | 1 | 676 | 11p15.5-p15.4 | KCNQ1 | potassium voltage-gated channel subfamily Q member 1 | 47,52 |
| Mouse | 6 | 1 | 668 | 7 88.12 cM | Kcnq1 | potassium voltage-gated channel, subfamily Q, member 1 | 3 |
| Rat | 6 | 1 | 669 | 1q42 | Kcnq1 | potassium voltage-gated channel subfamily Q member 1 | 20 |
| Previous and Unofficial Names |
| JLNS1 | IKs producing slow voltage-gated potassium channel subunit alpha KvLQT1 | RWS1 | Romano-Ward syndrome 1 | KCNA8 | KCNA9 | LQT1 | slow delayed rectifier | potassium channel, voltage gated KQT-like subfamily Q, member 1 | potassium channel, voltage-gated KQT-like subfamily Q, member 1 | potassium voltage-gated channel |
| Database Links |
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| Alphafold | P51787 (Hs), P97414 (Mm), Q9Z0N7 (Rn) |
| ChEMBL Target | CHEMBL1866 (Hs) |
| DrugBank Target | P51787 (Hs) |
| Ensembl Gene | ENSG00000053918 (Hs), ENSMUSG00000009545 (Mm), ENSRNOG00000020532 (Rn) |
| Entrez Gene | 3784 (Hs), 16535 (Mm), 84020 (Rn) |
| Human Protein Atlas | ENSG00000053918 (Hs) |
| KEGG Gene | hsa:3784 (Hs), mmu:16535 (Mm), rno:84020 (Rn) |
| OMIM | 607542 (Hs) |
| Orphanet | ORPHA122800 (Hs) |
| Pharos | P51787 (Hs) |
| RefSeq Nucleotide | NM_181798 (Hs), NM_000218 (Hs), NM_008434 (Mm), NM_032073 (Rn) |
| RefSeq Protein | NP_000209 (Hs), NP_861463 (Hs), NP_032460 (Mm), NP_114462 (Rn) |
| UniProtKB | P51787 (Hs), P97414 (Mm), Q9Z0N7 (Rn) |
| Wikipedia | KCNQ1 (Hs) |
| Associated Proteins |
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| Associated Protein Comments | ||||||||||||||||||||||||||||||||||||||
| Yotiao – targets Protein kinase A, phosphodiesterase PDE4D3, adenylate cyclase AC9 and protein phosphatase 1 to KCNQ1 [9,24,26,43]. Calmodulin-is tethered to the KCNQ1 proximal C-terminus and is important for channel folding and gating [42]. Gating: allosteric gating, where KCNQ1 voltage sensors move independently. KCNQ1 can open after zero to four voltage sensor movements [31]; KCNE1 alters the voltage sensor movement [4,28,32]; PIP2 stabilizes the channel in the open state [8,13,25]. Modulation: The scaffolding A-kinase anchoring protein (AKAP) so-called yotiao or AKAP9 brings the IKS channel complex together with protein kinase PKA, protein phosphatase PP1, phosphodiesterase PDE4D3 and adenylate cyclase AC9 to achieve upregulation following β-adrenergic stimulation [24,26,43]. |
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| Functional Characteristics |
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| cardiac IK5 | |
| Ion Selectivity and Conductance |
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| Ion Selectivity and Conductance Comments | ||||||
| Voltage Dependence |
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Download all structure-activity data for this target as a CSV file 
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| Key to terms and symbols | View all chemical structures | Click column headers to sort | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
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| ML277 activates KCNQ1 selectively versus KCNQ2-5, and does not activate IKS [27]. | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Inhibitors | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
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| Key to terms and symbols | View all chemical structures | Click column headers to sort | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
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| Block is for KCNQ1+KCNE1 channels; block of KCNQ1 alone is ~10-x more potent for XE991, ~10-x less potent for 293B or azimilide | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
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| Phenotypes, Alleles and Disease Models
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| Clinically-Relevant Mutations and Pathophysiology
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| For a summary of missense mutations associated with Long QT syndrome, please see the OMIM entry for the gene and the reference Wang et al. 1996 [47]. | ||||||||||||||||||||||||||||||||||||||||||
| Gene Expression and Pathophysiology
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| Biologically Significant Variants
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| Variant 1 encodes the predominant isoform 1. Variant 2 differs in the 5' UTR and CDS,compared to variant 1. Isoform 2 has a shorter and distinct N-terminus compared to isoform 1 and causes dominant-negative suppression of KCNQ1 channel function. |
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22. Lewis A, Green K, Dawson C, Redrup L, Huynh KD, Lee JT, Hemberger M, Reik W. (2006) Epigenetic dynamics of the Kcnq1 imprinted domain in the early embryo. Development, 133 (21): 4203-10. [PMID:17021040]
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25. Loussouarn G, Park KH, Bellocq C, Baró I, Charpentier F, Escande D. (2003) Phosphatidylinositol-4,5-bisphosphate, PIP2, controls KCNQ1/KCNE1 voltage-gated potassium channels: a functional homology between voltage-gated and inward rectifier K+ channels. EMBO J, 22 (20): 5412-21. [PMID:14532114]
26. Marx SO, Kurokawa J, Reiken S, Motoike H, D'Armiento J, Marks AR, Kass RS. (2002) Requirement of a macromolecular signaling complex for beta adrenergic receptor modulation of the KCNQ1-KCNE1 potassium channel. Science, 295 (5554): 496-9. [PMID:11799244]
27. Mattmann ME, Yu H, Lin Z, Xu K, Huang X, Long S, Wu M, McManus OB, Engers DW, Le UM et al.. (2012) Identification of (R)-N-(4-(4-methoxyphenyl)thiazol-2-yl)-1-tosylpiperidine-2-carboxamide, ML277, as a novel, potent and selective K(v)7.1 (KCNQ1) potassium channel activator. Bioorg Med Chem Lett, 22 (18): 5936-41. [PMID:22910039]
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48. Werry D, Eldstrom J, Wang Z, Fedida D. (2013) Single-channel basis for the slow activation of the repolarizing cardiac potassium current, I(Ks). Proc Natl Acad Sci USA, 110 (11): E996-1005. [PMID:23431135]
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50. Wu DM, Jiang M, Zhang M, Liu XS, Korolkova YV, Tseng GN. (2006) KCNE2 is colocalized with KCNQ1 and KCNE1 in cardiac myocytes and may function as a negative modulator of I(Ks) current amplitude in the heart. Heart rhythm : the official journal of the Heart Rhythm Society, 3 (12): 1469-80. [PMID:17161791]
51. Xiong Q, Sun H, Li M. (2007) Zinc pyrithione-mediated activation of voltage-gated KCNQ potassium channels rescues epileptogenic mutants. Nat Chem Biol, 3 (5): 287-96. [PMID:17435769]
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53. Yang Y, Sigworth FJ. (1998) Single-channel properties of IKs potassium channels. J Gen Physiol, 112 (6): 665-78. [PMID:9834139]
