| 產(chǎn)品編號(hào) | bs-6760R-BF594 |
| 英文名稱 | Rabbit Anti-KCNQ1/BF594 Conjugated antibody |
| 中文名稱 | BF594標(biāo)記的鉀離子通道蛋白家族KCNQ1抗體 |
| 別 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. |
| 規(guī)格價(jià)格 | 100ul/2980元 購(gòu)買 大包裝/詢價(jià) |
| 說(shuō) 明 書 | 100ul |
| 研究領(lǐng)域 | 心血管 細(xì)胞生物 神經(jīng)生物學(xué) 通道蛋白 細(xì)胞膜受體 細(xì)胞表面分子 |
| 抗體來(lái)源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | |
| 產(chǎn)品應(yīng)用 | ICC=1:50-200 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 狀 | Lyophilized or Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human KCNQ-1 |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 儲(chǔ) 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(chǎn)品介紹 |
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. |
