KCNH2 Antibody

1. Dominant-negative hERG1b G288S subunits suppress hERG1a currents. hERG1a G628S did not produce measurable currents and a mixture of hERG1a and hERG1a G628S markedly reduced hERG1a current PMID: 29089383
2. novel variants in SCN5A, KCNH2 and KCNQ1 are associated with congenital long QT syndrome in a Polish population PMID: 30244407
3. Data have defined the requirements for the susceptibility of LQT2 mutations to nonsense-mediated mRNA decay (NMD), and suggested that the majority of LQT2 nonsense and frameshift mutations are potential targets of NMD. Given that these specific mutations account for more than 30% of reported LQT2 mutations, the degradation of mutant mRNA by NMD is an important disease causing mechanism in the pathogenesis of LQT2. PMID: 29058182
4. Applying the computational approaches in this study, have helped to elucidate the possible binding patterns and time evaluation dynamics of this drug at hERG1 channel models (both in its open and open-inactivated states) together with the crucial amino acid residues that mostly contribute in binding processes via interaction binding energy decomposition analysis. PMID: 28963955
5. In a family afflicted by recurrent SIDS, post-mortem directed genetic testing was conducted. We demonstrate that despite the variant being present in both SIDS cases, KCNH2-p.Pro963Thr did not appreciably alter membrane surface expression of the hERG channel, or the biophysical properties, including deactivation gating. These findings suggest that KCNH2-p.Pro963Thr is not a monogenic disease-causing LQTS mutation PMID: 29331839
6. It was also found that drug ionization may play a crucial role in preferential targeting to the open-inactivated state of the pore domain. pH-dependent hERG blockade by dofetilie was studied with patch-clamp recordings. The results show that low pH increases the extent and speed of drug-induced block. PMID: 27731415
7. Fluconazole can prolong the QT interval and possess proarrhythmic activity due to its inhibition of hERG protein trafficking in experimental model. PMID: 27189953
8. Our results underscore the importance of careful characterization of the impact of epitope fusion tags and of confirming complete sequence accuracy prior to genotype-phenotype studies for ion channel proteins such as hERG. PMID: 28544109
9. Data show that ether-a-go-go-related 1 (hERG1) ion channel expression knockdown elicited a reversion of the epithelial to the mesenchymal state (EMT) profile in colorectal cancer HCT116 cells with a reacquisition of the epithelial-like profile. PMID: 28593575
10. TBX20 can be considered a KCNH2-modifying gene. PMID: 28049825
11. The KCNH2 intron 9 branch point is linked to the regulation of KCNH2 isoform expression in cardiomyocytes. PMID: 28433559
12. Our findings suggest that the K897T polymorphism of KCNH2 may contribute to the occurrence of syncope in Andersen-Tawil syndrome. PMID: 28336205
13. this study shows that hERG1 behaves as biomarker of progression to adenocarcinoma in Barrett's esophagus and can be exploited for a novel endoscopic surveillance PMID: 27517748
14. results reveal a novel mechanism by which stimulation of Kv11.1 channel leads to transcription of a potent tumor suppressor and suggest a potential therapeutic use for Kv11.1 channel activators PMID: 25945833
15. QTi derived from Holter recordings predicts the mutation carrier state in families with Long QT syndromes. Increased 24-hour QT-RR slope is a diagnostic hint pointing in the direction of LQT2. PMID: 28212739
16. The notorious ligand promiscuity of this channel earmarked hERG as one of the most important antitargets to be considered in early stages of drug development process. Herein we report on the development of an innovative and freely accessible web server for early identification of putative hERG blockers and non-blockers in chemical libraries. PMID: 27490970
17. structural basis and role of the fast component of gating charge PMID: 29117522
18. In HEK293 cells, CO inhibited wild-type, but not C723S mutant, Kv11.1 K(+) currents. Inhibition was prevented by an antioxidant, mitochondrial inhibitors, or inhibition of NO formation. PMID: 28743763
19. data provide novel information about hypoxia-mediated hERG dysfunction and may have biological and clinical implications in hypoxia-associated diseases. PMID: 28784631
20. Relative expression of Kv11.1 C-terminal isoforms can be regulated by modified U1 snRNA. PMID: 29066300
21. Expression and functions of hERG are regulated by specific miRNAs. PMID: 27558843
22. The present study shows clinical and genetic aspects of p.His492Tyr carriers and demonstrated two major findings; (1) KCNH2 p.His492Tyr variant presented symptomatic Romano-Ward syndrome in the presence of another LQTS-related gene mutation, in contrast, (2) heterozygous carriers had mild QT prolongation while additional triggers can be "latent" form of p.His492Tyr PMID: 27816319
23. Homozygous carriers of CYP2B6*6 allele may be at increased risk for EFV-induced QT interval prolongation via inhibition of hERG. PMID: 27333947
24. ER-located J-protein chaperones were identified as key regulators for the iogenesis and physiological function of ERG K+ channels. They regulate two distinct aspects of K+ channel biogenesis, the stabilization and assembly of channel subunits. PMID: 27916661
25. atypical sequence of the pore helix of hERG may play a key role in determining how anionic lipids influence its gating PMID: 28314880
26. Besides a prolonged cardiac repolarization phase, LQT2 patients display increased GLP-1, GIP, and insulin secretion and defective glucagon secretion, causing decreased plasma glucose and thus increased risk of hypoglycemia. PMID: 28235848
27. BIMU8 is a potent blocker of hERG, NaV1.5 and CaV1.2 cardiac ion channels, inducing cardiac arrhythmias. PMID: 28552773
28. The K897T polymorphism in the hERG1 gene has been reported as an important modifier of the IKr current, probably leading to QT interval prolongation-as it is observed in the LQT2 syndrome. PMID: 26109178
29. Stabilization of the Activated hERG Channel Voltage Sensor by Depolarization Involves the S4-S5 Linker PMID: 28122216
30. Study determined the structure of hERG using cryo-electron microscopy; the voltage sensors are depolarized, and the inner helical gate is open; an unusual geometry of the "central cavity" within hERG's ion conduction pathway; hERG contains a subtle difference in the structure of its selectivity filter compared to all other K+ channels so far analyzed. PMID: 28431243
31. S1 region mutations would reduce both the action potential repolarizing current passed by Kv11.1 channels in cardiac myocytes, as well as the current passed in response to premature depolarizations that normally helps protect against the formation of ectopic beats. PMID: 28280240
32. hERG mediated by proteases such as calpain may contribute to ischemia-associated QT prolongation and sudden cardiac death PMID: 27502273
33. the two tyrosine residues in the Kv11.1 S4S5 linker play critical but distinct roles in the slow deactivation phenotype, which is a hallmark of Kv11.1 channels. PMID: 27317659
34. s found that expression of HERG K(+) channels in MDS patients was significantly higher than controls and was lower than AML. PMID: 27077769
35. M3-mAChR activation leads to enhancement of hsp expression via PKC-dependent phosphorylation of HSF1, thereby stabilizing the mutant hERG-FLAG protein. Thus, M3-mAChR activators may have a therapeutic value for patients with LQT2. PMID: 27803431
36. The solution structure of hERG cyclic nucleotide-binding homology domain. PMID: 27025590
37. A novel heterozygous missense mutation in exon 7 of KCNH2 gene, causing a protein change p.F617V, was found in a family with life-threatening arrhythmias in women and clinical outcome typical for long QT2 syndrome. PMID: 25987402
38. Activation of the alpha1-AR reduces the amplitude of IhERG and IKr through a positive shift in the activation half voltage, which reduces the channel availability at physiological membrane potentials. PMID: 27997898
39. Monte Carlo method for predicting of cardiac toxicity of hERG blockers. PMID: 27067105
40. A561VhERG exerts a potent dominantnegative effect on WThERG channels, resulting in decreased hERG currents and impairment of hERG membrane localization. PMID: 26847485
41. Alternate mRNA transcripts encoding human ether-a-go-go-related gene (hERG) 1a and 1b subunits, which assemble to produce ion channels mediating cardiac repolarization, are physically associated during translation. This interaction occurred even when translation of 1b was prevented, indicating the transcripts associate independent of their encoded proteins. PMID: 27078096
42. KCNH2 gene was initially discovered in the hippocampus, and its expression was later demonstrated in many regions of the central nervous system. In a study that included 343 LQTS probands, a personal history of seizures was more common in LQT2 than all other subtypes of LQTS combined. PMID: 25819988
43. Result suggests that the mutation T613A in Kv 11.1 causes Long QT Syndrome type 2 due to a reduced repolarization reserve. PMID: 26173150
44. The human ether-a-go-go-related gene (hERG) encodes the pore-forming subunit of a delayed rectifier voltage gated K+ channel. PMID: 26519040
45. In patients with genotype-positive Long QT syndrome, significant differences exist in the degree of daytime and nocturnal QTc prolongation. PMID: 26334569
46. Mutational screening identified 104 mutations (44% novel), i.e. 46 KCNQ1, 54 KCNH2 and 4 SCN5A mutations for long QT syndrome in China. PMID: 26496715
47. we carried out an in silico screen for the naturally occurring hERG mutation, the M54T MiRP1 mutation, which has been implicated in drug-induced LQTS and arrhythmia. PMID: 26859003
48. that activation of calpains by reactive oxygen species-dependent elevation of [Ca(2+)]i mediates Human ether-a-go-go-related gene protein degradation by IH PMID: 26659724
49. The altered channel gating kinetics in combination with defective trafficking of mutated Kv11.1 channels is expected to result in reduced repolarizing current density and, thus, a long QT syndrome phenotype. PMID: 26403377
50. Propose that berberine reduces hERG membrane stability with multiple mechanisms. PMID: 26543354

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HGNC: 6251

OMIM: 152427

KEGG: hsa:3757

STRING: 9606.ENSP00000262186

UniGene: Hs.647099