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ERG/KCNH2 Rabbit pAb
- 产品详情
- 实验流程
Application 
| IHC-P, IHC-F, IF |
|---|---|
| Primary Accession | Q12809 |
| Other Accession | Q12809 |
| Host | Rabbit |
| Clonality | Polyclonal |
| Calculated MW | 126655 Da |
| Physical State | Liquid |
| Immunogen | KLH conjugated synthetic peptide derived from human ERG/KCNH2 |
| Epitope Specificity | 541-640/1159 |
| Isotype | IgG |
| Purity | affinity purified by Protein A |
| Buffer | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| SIMILARITY | Belongs to the potassium channel family. H (Eag) (TC1.A.1.20) subfamily. Kv11.1/KCNH2 sub-subfamily. Contains 1 cyclic nucleotide-binding domain. Contains 1 PAC (PAS-associated C-terminal) domain. Contains 1 PAS (PER-ARNT-SIM) domain. |
| SUBUNIT | The potassium channel is probably composed of a homo- or heterotetrameric complex of pore-forming alpha subunits that can associate with modulating beta subunits. Heteromultimer with KCNH6/ERG2 and KCNH7/ERG3. Interacts with ALG10B (By similarity). Heteromultimer with KCNE1 and KCNE2. |
| Post-translational modifications | Phosphorylated on serine and threonine residues. Phosphorylation by PKA inhibits ion conduction. |
| DISEASE | Defects in KCNH2 are the cause of long QT syndrome type 2 (LQT2) [MIM:613688]. Long QT syndromes are heart disorders characterized by a prolonged QT interval on the ECG and polymorphic ventricular arrhythmias. They cause syncope and sudden death in response to exercise or emotional stress. Deafness is often associated with LQT2. Defects in KCNH2 are the cause of short QT syndrome type 1 (SQT1) [MIM:609620]. Short QT syndromes are heart disorders characterized by idiopathic persistently and uniformly short QT interval on ECG in the absence of structural heart disease in affected individuals. They cause syncope and sudden death. |
| Important Note | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| Background Descriptions | The potassium voltage gated channel, subfamily H (eag related), member 2 (KCNH2) gene encodes a voltage-gated potassium channel which has an important role in cardiac action potential repolarization in the mammalian heart. Mutations in KCNH2 have been shown to cause chromosome 7-linked congenital long QT syndrome, a disorder associated with delayed cardiac repolarization, prolonged electrocardiographic QT intervals, and the development of ventricular arrhythmias. KCNH2 channels are an important target for many drugs, and have emerged as a significant type of cardiac ion channel.Highly expressed in heart and brain. |
| Gene ID | 3757 |
|---|---|
| Other Names | ERG-1; ERG1; H-ERG; HERG; HERG1; Kv11.1; LQT2; SQT1; LQT; M-erg; Merg1; merg1a; merg1b; KCNH2_HUMAN; KCNH2; Eag homolog; Ether-a-go-go-related gene potassium channel 1 (ERG-1 | Eag-related protein 1 | Ether-a-go-go-related protein 1 | H-ERG | hERG-1 | hERG1); Potassium voltage-gated channel subfamily H member 2; Voltage-gated potassium channel subunit Kv11.1; ERG; KCNH2_MOUSE; Ether-a-go-go-related gene potassium channel 1 (ERG-1 | Eag-related protein 1 | Ether-a-go-go-related protein 1 | MERG); KCNH2_RAT; Ether-a-go-go-related gene potassium channel 1 (ERG-1 | Eag-related protein 1 | Ether-a-go-go-related protein 1 | RERG | r-ERG); potassium voltage-gated channel, subfamily H (eag-related), member 2; human ether-a-go-go-related gene; long QT syndrome type 2 |
| Dilution | IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500 |
| Storage | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
For Research Use Only. Not For Use In Diagnostic Procedures.
| Name | KCNH2 (HGNC:6251) |
|---|---|
| Function | Pore-forming (alpha) subunit of voltage-gated inwardly rectifying potassium channel (PubMed:10219239, PubMed:10753933, PubMed:10790218, PubMed:10837251, PubMed:11997281, PubMed:12063277, PubMed:18559421, PubMed:22314138, PubMed:22359612, PubMed:26363003, PubMed:27916661, PubMed:9230439, PubMed:9351446, PubMed:9765245). Channel properties are modulated by cAMP and subunit assembly (PubMed:10837251). Characterized by unusual gating kinetics by producing relatively small outward currents during membrane depolarization and large inward currents during subsequent repolarization which reflect a rapid inactivation during depolarization and quick recovery from inactivation but slow deactivation (closing) during repolarization (PubMed:10219239, PubMed:10753933, PubMed:10790218, PubMed:10837251, PubMed:11997281, PubMed:12063277, PubMed:18559421, PubMed:22314138, PubMed:22359612, PubMed:26363003, PubMed:27916661, PubMed:9230439, PubMed:9351446, PubMed:9765245). Forms a stable complex with KCNE1 or KCNE2, and that this heteromultimerization regulates inward rectifier potassium channel activity (PubMed:10219239, PubMed:9230439). |
| Cellular Location | Cell membrane; Multi-pass membrane protein |
| Tissue Location | Highly expressed in heart and brain. Isoforms USO are frequently overexpressed in cancer cells |
Research Areas
Application Protocols
Provided below are standard protocols that you may find useful for product applications.
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Cat# AP98068
