钾离子通道蛋白家族成员2抗体
规格:1mg/1ml
英文名: KCNE2
别名: ATFB4; cardiac voltage gated potassium channel accessory subunit 2; Kcne2; KCNE2_HUMAN; LQT5; LQT6; minimum potassium ion channel related peptide 1; Minimum potassium ion channel-related peptide 1 ant
分子量: 14kDa
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 KCNE2
交叉反应:Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Sheep,
细胞定位:细胞膜
钾离子通道蛋白家族成员2抗体产品介绍:background: Ancillary protein that assembles as a beta subunit with a voltage-gated potassium channel complex of pore-forming alpha subunits. Modulates the gating kinetics and enhances stability of the channel complex. Associated with KCNH2/HERG is proposed to form the rapidly activating component of the delayed rectifying potassium current in heart (IKr). May associate with KCNQ2 and/or KCNQ3 and modulate the native M-type current. May associate with KCNQ1/KVLTQ1 and elicit a voltage-independent current. May associate with HCN1 and HCN2 and increase potassium current. Function: Ancillary protein that assembles as a beta subunit with a voltage-gated potassium channel complex of pore-forming alpha subunits. Modulates the gating kinetics and enhances stability of the channel complex. Associated with KCNH2/HERG is proposed to form the rapidly activating component of the delayed rectifying potassium current in heart (IKr). May associate with KCNQ2 and/or KCNQ3 and modulate the native M-type current. May associate with KCNQ1/KVLTQ1 and elicit a voltage-independent current. May associate with HCN1 and HCN2 and increase potassium current. Subunit: Associates with KCNH2/ERG1. May associate with KCNQ1/KVLQT1, KCNQ2 and KCNQ3. Associates with HCN1 and probably HCN2 (By similarity). Subcellular Location: Membrane. Tissue Specificity: Highly expressed in brain, heart, skeletal muscle, pancreas, placenta, kidney, colon and thymus. A small but significant expression is found in liver, ovary, testis, prostate, small intestine and leukocytes. Very low expression, nearly undetectable, in lung and spleen. DISEASE: Defects in KCNE2 are the cause of long QT 钾离子通道蛋白家族成员2抗体syndrome type 6 (LQT6) [MIM:613693]. 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. KCNE2 mutants form channels that open slowly and close rapidly, thereby diminishing potassium currents. Defects in KCNE2 are the cause of familial atrial fibrillation type 4 (ATFB4) [MIM:611493]. Atrial fibrillation is a common disorder of cardiac rhythm that is hereditary in a small subgroup of patients. It is characterized by disorganized atrial electrical activity and ineffective atrial contraction promoting blood stasis in the atria and reduces ventricular filling. It can result in palpitations, syncope, thromboembolic stroke, and congestive heart failure. Similarity: Belongs to the potassium channel KCNE family. Database links: Entrez Gene: 9992 Human Entrez Gene: 246133 Mouse Omim: 603796 Human SwissProt: Q9Y6J6 Human SwissProt: Q9D808 Mouse Unigene: 551521 Human Unigene: 679753 Human Unigene: 156736 Mouse Important Note: This product as supplied is intended for research 钾离子通道蛋白家族成员2抗体use only, not for use in human, therapeutic or diagnostic applications. Involvement in disease; Defects in KCNE2 are the cause of long QT syndrome type 6 (LQT6). 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. KCNE2 mutants form channels that open slowly and close rapidly, thereby diminishing potassium currents.
钾离子通道蛋白家族成员2抗体产品应用:WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:50-200 (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:心血管 神经生物学 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