FITC标记的钾离子通道蛋白家族KCNQ1抗体-抗体-抗体-生物在线
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FITC标记的钾离子通道蛋白家族KCNQ1抗体

FITC标记的钾离子通道蛋白家族KCNQ1抗体

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产品名称： FITC标记的钾离子通道蛋白家族KCNQ1抗体

英文名称： Anti-KCNQ1/FITC

产品编号： HZ-6760R-FITC

产品价格： null

产品产地： 中国/上海

品牌商标： HZbscience

更新时间： 2023-08-17T10:24:20

使用范围： IF=1:50-200

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 Rabbit Anti-KCNQ1/FITC Conjugated antibody

FITC标记的钾离子通道蛋白家族KCNQ1抗体

 

英文名称 Anti-KCNQ1/FITC
中文名称 FITC标记的钾离子通道蛋白家族KCNQ1抗体
别    名 KCNQ-1; ATFB1; ATFB3; IKs producing slow voltage-gated potassium channel subunit alpha; IKs producing slow voltage-gated potassium channel subunit alpha KvLQT1; Jervell and Lange-Nielsen syndrome 1; JLNS1; KCNA8; KCNA9; KCNQ1; KCNQ1_HUMAN; kidney and cardiac voltage dependend K+ channel; KQT-like 1; Kv1.9; Kv7.1; KVLQT1; long (electrocardiographic) QT syndrome, Ward-Romano syndrome 1; LQT; LQT1; Potassium voltage-gated channel subfamily KQT member 1; potassium voltage-gated channel, KQT-like subfamily, member 1; RWS; slow delayed rectifier channel subunit; SQT2; Voltage-gated potassium channel subunit Kv7.1; WRS.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 心血管  细胞生物  神经生物学  通道蛋白  细胞膜受体  细胞表面分子  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Chicken, Dog, Cow, 
产品应用 IF=1:50-200  
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 75kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human KCNQ-1
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:
Probably important in cardiac repolarization. Associates with KCNE1 (MinK) to form the I(Ks) cardiac potassium current. Elicits a rapidly activating, potassium-selective outward current. Muscarinic agonist oxotremorine-M strongly suppresses KCNQ1/KCNE1 current in CHO cells in which cloned KCNQ1/KCNE1 channels were coexpressed with M1 muscarinic receptors. May associate also with KCNE3 (MiRP2) to form the potassium channel that is important for cyclic AMP-stimulated intestinal secretion of chloride ions, which is reduced in cystic fibrosis and pathologically stimulated in cholera and other forms of secretory diarrhea. Involvement in disease: 

Function:
Probably important in cardiac repolarization. Associates with KCNE1 (MinK) to form the I(Ks) cardiac potassium current. Elicits a rapidly activating, potassium-selective outward current. Muscarinic agonist oxotremorine-M strongly suppresses KCNQ1/KCNE1 current in CHO cells in which cloned KCNQ1/KCNE1 channels were coexpressed with M1 muscarinic receptors. May associate also with KCNE3 (MiRP2) to form the potassium channel that is important for cyclic AMP-stimulated intestinal secretion of chloride ions, which is reduced in cystic fibrosis and pathologically stimulated in cholera and other forms of secretory diarrhea.

Subunit:
Heterotetramer with KCNE1 (MinK) or KCNE3 (MiRP2). Interacts with CALM.

Subcellular Location:
Cell membrane; Multi-pass membrane protein. Cytoplasmic vesicle membrane; Multi-pass membrane protein.

Tissue Specificity:
Abundantly expressed in heart, pancreas, prostate, kidney, small intestine and peripheral blood leukocytes. Less abundant in placenta, lung, spleen, colon, thymus, testis and ovaries. 

DISEASE:
Defects in KCNQ1 are the cause of long QT syndrome type 1 (LQT1) ; also known as Romano-Ward syndrome (RWS). 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. LQT1 inheritance is an autosomal dominant.
Defects in KCNQ1 are the cause of Jervell and Lange-Nielsen syndrome type 1 (JLNS1) . JLNS1 is an autosomal recessive disorder characterized by congenital deafness, prolongation of the QT interval, syncopal attacks due to ventricular arrhythmias, and a high risk of sudden death.
Defects in KCNQ1 are the cause of atrial fibrillation familial type 3 (ATFB3) . 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 family. KQT (TC 1.A.1.15) subfamily. Kv7.1/KCNQ1 sub-subfamily.

Database links:
 UniProtKB/Swiss-Prot: P51787.3


Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications
   

可能在心脏复极中很重要。与KCNE1（水貂）缔合形成I（Ks）心脏钾电流。引出一种快速激活的钾选择性外向电流。毒蕈碱激动剂氧肟内酯M强烈抑制CHO细胞中KCNQ1/KCNE1电流，其中克隆的KCNQ1/KCNE1通道与M1毒蕈碱受体共表达。也可与KCNE3（miRP2）结合形成钾通道，这对环AMP刺激的氯离子肠分泌是重要的，在囊性纤维化中减少，并在霍乱和其他形式的分泌性腹泻中受到病理刺激。疾病参与：