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货号： bs-23703R-AP 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-23703R-AP
- 英文名称
- Anti-ERG/KCNH2/AP
- 中文名称
- 碱性磷酸酶（AP）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, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep,
- 产品应用
- WB=1:50-200 IHC-P=1:50-200 IHC-F=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
- 2mg/1ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human ERG/KCNH2
- 亚 型
- IgG
- 纯化方法
- affinity purified by Protein A
- 储 存 液
- Constituents: 0.05M TBS, pH 8.0 with 10mg/ml BSA and 0.05% NaN3, 50% glycerol. Or Lyophilized. Buffer = 0.05M TBS, pH 8.0 with 10mg/ml BSA and 0.05% NaN3. Reconstitute with sterile distilled water.
- 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: 3757Human
Entrez Gene: 16511Mouse
Entrez Gene: 117018Rat
Omim: 152427Human
SwissProt: Q12809Human
SwissProt: O35219Mouse
SwissProt: O08962Rat
Unigene: 647099Human
Unigene: 6539Mouse
Unigene: 10970Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.