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特异性钾离子通道蛋白抗体
  • 产品货号:
    BN41751R
  • 中文名称:
    特异性钾离子通道蛋白抗体
  • 英文名称:
    Rabbit anti-ERG/KCNH2 Polyclonal antibody
  • 品牌:
    Biorigin
  • 货号

    产品规格

    售价

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  • BN41751R-50ul

    50ul

    ¥1486.00

    交叉反应:Human,Mouse,Rat(predicted:Dog,Pig,Horse,Rabbit) 推荐应用:ELISA

  • BN41751R-100ul

    100ul

    ¥2360.00

    交叉反应:Human,Mouse,Rat(predicted:Dog,Pig,Horse,Rabbit) 推荐应用:ELISA

  • BN41751R-200ul

    200ul

    ¥3490.00

    交叉反应:Human,Mouse,Rat(predicted:Dog,Pig,Horse,Rabbit) 推荐应用:ELISA

产品描述

英文名称ERG/KCNH2
中文名称特异性钾离子通道蛋白抗体
别    名ERG; ERG1; H ERG; HERG 1; HERG; HERG1; LQT 2; LQT2; Potassium channel HERG; SQT1; Voltagegated potassium channel, subfamily H, member 2; KCNH2_HUMAN.
研究领域肿瘤  免疫学  细胞周期蛋白  通道蛋白  
抗体来源Rabbit
克隆类型Polyclonal
交叉反应Human, Rat,  (predicted: Mouse, Dog, Pig, Horse, Rabbit, )
产品应用ELISA=1:5000-10000 Flow-Cyt=1μg /test ICC=1:100-500 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量127kDa
细胞定位细胞膜 
性    状Liquid
浓    度1mg/ml
免 疫 原KLH conjugated synthetic peptide derived from human HERG:1001-1159/1159 <Cytoplasmic>
亚    型IgG
纯化方法affinity purified by Protein A
储 存 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
PubMedPubMed
产品介绍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.

Function:
Pore-forming (alpha) subunit of voltage-gated inwardly rectifying potassium channel. Channel properties are modulated by cAMP and subunit assembly. Mediates the rapidly activating component of the delayed rectifying potassium current in heart (IKr). Isoform 3 has no channel activity by itself, but modulates channel characteristics when associated with isoform 1.

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.

Subcellular Location:
Membrane; Multi-pass membrane protein.

Tissue Specificity:
Highly expressed in heart and brain.

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.

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.

SWISS:
Q12809

Gene ID:
3757

Database links:

Entrez Gene: 3757 Human

Entrez Gene: 16511 Mouse

Entrez Gene: 117018 Rat

Omim: 152427 Human

SwissProt: Q12809 Human

SwissProt: O35219 Mouse

SwissProt: O08962 Rat

Unigene: 647099 Human

Unigene: 6539 Mouse

Unigene: 10970 Rat



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.





















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