Potassium Voltage-Gated Channel, Subfamily H (Eag-Related), Member 2 蛋白 (KCNH2)

KCNH2 encodes a voltage-activated potassium channel belonging to the eag family. 再加上,我们可以发Potassium Voltage-Gated Channel, Subfamily H (Eag-Related), Member 2 抗体 (101)和数多这个蛋白质的别的产品。

列出全部蛋白 基因 基因ID UniProt
KCNH2 3757 Q12809
KCNH2 16511  
大鼠 KCNH2 KCNH2 117018 O08962
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KCNH2 蛋白 by Origin and Source

Origin 在表达 标记
Human ,
, ,
Mouse (Murine)

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Human Potassium Voltage-Gated Channel, Subfamily H (Eag-Related), Member 2 (KCNH2) interaction partners

  1. KCNH2 c.453delC, L552S and R176W mutations associated with lower risk for cardiac events than other KCNH2 mutations.

  2. Synonymous nucleotide modification of the KCNH2 gene affects both mRNA characteristics and translation of the encoded hERG ion channel.

  3. small clockwise rotation of the inner (S6) helix of the hERG pore from its configuration in the cryo-EM structure may be required to optimize Phe-656 side chain orientations compatible with high-affinity block

  4. This study indicated that the K801T mutation caused the gain of function of human KCNH2 channels that may account for the clinical phenotype of early repolarization syndrome.

  5. 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

  6. novel variants in SCN5A, KCNH2 and KCNQ1 are associated with congenital long QT syndrome in a Polish population

  7. 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.

  8. 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.

  9. 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

  10. 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.

  11. Fluconazole can prolong the QT interval and possess proarrhythmic activity due to its inhibition of hERG protein trafficking in experimental model.

  12. 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.

  13. 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.

  14. TBX20 can be considered a KCNH2-modifying gene.

  15. The KCNH2 intron 9 branch point is linked to the regulation of KCNH2 isoform expression in cardiomyocytes.

  16. Our findings suggest that the K897T polymorphism of KCNH2 may contribute to the occurrence of syncope in Andersen-Tawil syndrome.

  17. 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

  18. 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

  19. 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.

  20. 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.

Guinea Pig Potassium Voltage-Gated Channel, Subfamily H (Eag-Related), Member 2 (KCNH2) interaction partners

  1. data suggest that CO induces arrhythmias in guinea pig cardiac myocytes via the ONOO(-)-mediated inhibition of Kv11.1 K(+) channels

Mouse (Murine) Potassium Voltage-Gated Channel, Subfamily H (Eag-Related), Member 2 (KCNH2) interaction partners

  1. PKD1 mediates the PKC effects on KV11.1 and we found that PKD targets S284 in the N-terminus of the channel.

  2. histone H4 hyperacetylation induced by Class I HDACs inhibitors promoted the expression profiles of potassium channels (Kcnj2, Kcnj3, Kcnj5, Kcnj11, and Kcnh2)

  3. Using a combination of mouse genetics, biochemistry, molecular and cell biology, we demonstrate that Nkx2-5 regulates the gene encoding Kcnh2 channel and others, shedding light on potential mechanisms generating electrical abnormalities observed in patients bearing NKX2-5 dysfunction and opening opportunities to the study of novel therapeutic targets for anti-arrhythmogenic therapies

  4. Report altered protein trafficking/function of mouse ERG K(+) channel clones.

  5. Enhancement of HERG protein expression through Hsp90 inhibition of CHIP binding might be a novel therapeutic strategy for long QT syndrome 2 caused by trafficking abnormalities of HERG proteins.

  6. The findings of this study suggested that MERG1a may not modulate Murf1 expression through the AKT/FOXO pathway.

  7. Data indicate presence of KCNH2 in left/right atria & indicate functional interaction between KCNH2 & KCNA5. Kinetic analyses confirm KCNH2 as "rapid" and KCNA5 as "ultra rapid" potassium channels.

  8. Knockdown of mERG1 transcripts with small interfering RNA (siRNA) dramatically reduced I (Kr) amplitude, confirming the molecular link of mERG1 and I (Kr) in HL-1 cells.

  9. The LQT2 missense mutations G601S and N470D hERG exhibited altered protein trafficking in mouse cardiomyocytes.

  10. ERG1 B is necessary for I(Kr) expression in the surface membrane of adult myocytes. Knockout of ERG1 B predisposes mice to episodic sinus bradycardia.

  11. erg1 gene expression pattern in the developing embryo.

  12. MERG1A is developmentally regulated, and it might play a role in early mouse embryogenic development.

  13. All erg genes were translated in neuronal tissue. ERG proteins were generally expressed in neuronal soma, but dendritic and/or white matter labeling could be detected in specific areas.

  14. Functional expression of the stria vascularis-specific MERG1a channel reveals a current that activates at negative potentials and shows rapid inactivation.

  15. Expression of Merg1a initiates atrophy by activating ubiquitin-proteasome proteolysis.

  16. During spinal cord development, excitability of GABAergic ventral interneurons depend on the function of the ERG current.

  17. Expression of ERG1 isoforms in the myometrium did not alter throughout gestation or upon delivery, but the expression of genes encoding auxillary subunits (KCNE) were up-regulated considerably.

  18. ERG1 K+ channels contribute to threshold excitability of mouse auditory brainstem neurons.

  19. Erg1 channels are new mediators of alpha- and beta-cell repolarization. However, antagonism of Erg1 has divergent effects in these cells; to augment glucose-dependent insulin secretion and inhibit low glucose stimulated glucagon secretion.

Rabbit Potassium Voltage-Gated Channel, Subfamily H (Eag-Related), Member 2 (KCNH2) interaction partners

  1. RNF207 is an important regulator of action potential duration, likely via effects on HERG trafficking and localization in a heat shock protein-dependent manner.

  2. In transgenic long QT syndrome rabbits, Kv11.1 channel activation shortens the cardiac activation potential duration.

蛋白简介Potassium Voltage-Gated Channel, Subfamily H (Eag-Related), Member 2 (KCNH2)


This gene encodes a voltage-activated potassium channel belonging to the eag family. It shares sequence similarity with the Drosophila ether-a-go-go (eag) gene. Mutations in this gene can cause long QT syndrome type 2 (LQT2). Transcript variants encoding distinct isoforms have been identified.

Gene names and symbols associated with KCNH2

  • potassium voltage-gated channel subfamily H member 2 (KCNH2)
  • potassium voltage-gated channel subfamily H member 2 (Kcnh2)
  • potassium voltage-gated channel, subfamily H (eag-related), member 2 (Kcnh2)
  • potassium voltage-gated channel, subfamily H (eag-related), member 2 (KCNH2)
  • AI326795 蛋白
  • cerg 蛋白
  • derg 蛋白
  • erg 蛋白
  • erg1 蛋白
  • gp-erg 蛋白
  • HERG 蛋白
  • HERG1 蛋白
  • KCNH2 蛋白
  • Kv11.1 蛋白
  • LQT 蛋白
  • Lqt2 蛋白
  • M-erg 蛋白
  • Merg1 蛋白
  • merg1a 蛋白
  • merg1b 蛋白
  • SQT1 蛋白

Protein level used designations for KCNH2

ERG-1 , H-ERG , eag homolog , eag-related protein 1 , ether-a-go-go-related gene potassium channel 1 , ether-a-go-go-related potassium channel protein , ether-a-go-go-related protein 1 , hERG-1 , potassium voltage-gated channel subfamily H member 2 , voltage-gated potassium channel subunit Kv11.1 , potassium voltage-gated channel, subfamily H (eag-related), member 2 , potassium channel protein ERG , potassium voltage-gated channel subfamily H member 2-like , c-ERG , eag related protein 1 , ether-a-go-go related gene potassium channel 1 , MERG , ether a go-go related , RERG , r-ERG , ERG1 , ra-erg , ventricular ERG K+ channel subunit , voltage-gated potassium channel 11.1 , voltage-gated potassium channel, subfamily H, member 2

3757 Homo sapiens
463894 Pan troglodytes
100064000 Equus caballus
100135563 Cavia porcellus
100413935 Callithrix jacchus
100447585 Pongo abelii
403761 Canis lupus familiaris
16511 Mus musculus
117018 Rattus norvegicus
100009242 Oryctolagus cuniculus
100858122 Gallus gallus
100523293 Sus scrofa
539971 Bos taurus
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