特异性钾离子通道蛋白抗体
规格：1mg/1ml
英文名： ERG/KCNH2
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.
分子量： 127kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human HERG
交叉反应：Human, Mouse, Rat, Dog, Pig, Horse, Rabbit,
细胞定位：细胞膜
特异性钾离子通道蛋白抗体产品介绍：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.特异性钾离子通道蛋白抗体 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: 特异性钾离子通道蛋白抗体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. 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.
特异性钾离子通道蛋白抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 Flow-Cyt=1:20-300 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：肿瘤 **学 细胞周期蛋白 通道蛋白
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid