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货号： bs-6760R-AF555 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-6760R-AF555
- 英文名称
- Anti-KCNQ1/AF555
- 中文名称
- AF555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.