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Rabbit Anti-ERG/KCNH2/APC Conjugated antibody (bs-1815R-APC)
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说 明 书: 100ul  
100ul/2980.00元
大包装/询价
产品编号 bs-1815R-APC
英文名称1 Rabbit Anti-ERG/KCNH2/APC Conjugated antibody
中文名称 APC标记的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.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
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 Flow-Cyt=1:50-200 ICC=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 127kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human HERG
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 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. 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.
产品介绍 background:
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.

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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