ERG/KCNH2 Rabbit pAb (一抗) | Bioss

2026-05-01~2026-06-30,AB2605
货号:bs-1815R
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概述

产品编号
bs-1815R
产品类型
宠物抗体、农牧业/家禽抗体
英文名称
ERG/KCNH2 Rabbit pAb
中文名称
特异性钾离子通道蛋白抗体
英文别名
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
抗体来源
Rabbit
免疫原
KLH conjugated synthetic peptide derived from human HERG: 1001-1159/1159 <Cytoplasmic>
亚型
IgG
性状
Liquid
纯化方法
affinity purified by Protein A
克隆类型
Polyclonal
理论分子量
127 kDa
浓度
1mg/ml
储存液
0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
SWISS
Gene ID
保存条件
Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事项
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
背景资料
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.

产品应用

应用已检合格种属预测种属推荐稀释比例
ELISAHuman, Mouse, Rat, Rabbit, Pig, Dog, Horse1:5000-10000

交叉反应

交叉反应: (predicted: Human, Mouse, Rat, Rabbit, Pig, Dog, Horse)

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靶标

基因名
KCNH2
蛋白名
Potassium voltage-gated channel subfamily H member 2
亚基
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.
亚细胞定位
Membrane; Multi-pass membrane protein.
组织特异性
Highly expressed in heart and brain.
翻译后修饰
Phosphorylated on serine and threonine residues. Phosphorylation by PKA inhibits ion conduction.
疾病
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.
相似性
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.
功能
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.

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