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Rabbit Anti-KCNE2/BF488 Conjugated antibody (bs-9930R-BF488)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-9930R-BF488
英文名稱 Rabbit Anti-KCNE2/BF488 Conjugated antibody
中文名稱 BF488標記的鉀離子通道蛋白家族成員2抗體
別    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 antibody minK related peptide 1; MinK-related peptide 1; MIRP1; Potassium channel subunit beta MiRP1; potassium channel subunit, MiRP1; potassium voltage gated channel subfamily E member 2; potassium voltage gated channel, Isk related family, member 2; Potassium voltage-gated channel subfamily E member 2; voltage-gated K+ channel subunit MIRP1.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 心血管  神經生物學  通道蛋白  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Sheep, )
產品應用 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 14kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human KCNE2
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol
保存條件 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.
產品介紹 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 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 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.
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