货号：MABN55

品牌：Merck Millipore

规格：EA

目录价：￥3828.00

市场价格：￥3253.80

会员价格：￥3062.40

金山科研平台，产品价格货期咨询微信：jinshanbio Description: Anti-KCNQ1 Potassium Channel Antibody, clone N31A/10 | MABN55 View All» Promotional Text: Special Shipping Offer on Antibodies100% Performance Guaranteed View All» Molecular Weight: 75 kDa calculated View All» Immunogen: Recombinant protein corresponding to human KCNQ1. View All» Clone: N31A/10 View All» Isotype: IgG1κ View All» Background Information: KCNQ1 is a member of the K+ channel gene family that when mutated can cause hearing loss. The family is defined by transmembrane domains, single pore-loop K+ channel alpha subunits. KCNQ1 may have an important part in the repolarization of the cardiac action potential in the inner ear, as well as potassium homeostasis. KCNQ1 has been found to be responsible for over 50% of inherited long QT syndrome. View All» Species Reactivity:

• Rat

• Human
  View All» Species Reactivity Note: Demonstrated to react with rat. Predicted to react with human based on immunogen design. View All» Control: Rat brain tissue View All» Quality Assurance: Evaluated by Immunohistochemistry in rat brain tissue.Immunohistochemistry Analysis: 1:400 dilution of this antibody detected KCNQ1 in rat brain tissue. May have a tendency to stain vascular tissue. View All» Purification Method: Protein G View All» Presentation: Purified mouse monoclonal IgG1κ in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide. View All» Storage Conditions: Stable for 1 year at 2-8°C from date of receipt. View All» UniProt Number: P51787 View All» Entrez Gene Number: NP_000209 View All» Gene Symbol:
  • KCNQ1

  • ATFB3

  • KVLQT1

  • ATFB1

  • KCNA8

  • Kv1.9

  • KCNA9

  • LQT

  • JLNS1

  • WRS

  • Kv7.1

  • RWS

  • SQT2

  • LQT1
    View All» Alternate Names:
    • potassium voltage-gated channel, KQT-like subfamily, member 1

    • Voltage-gated potassium channel subunit Kv7.1

    • KQT-like 1

    • potassium voltage-gated channel subfamily KQT member 1

    • IKs producing slow voltage-gated potassium channel subunit alpha KvLQT1

    • kidney and cardiac voltage dependend K+ channel

    • slow delayed rectifier channel subunit
      View All» Usage Statement: Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals. View All» Key Applications: Immunohistochemistry View All» Entrez Gene Summary: This gene encodes a voltage-gated potassium channel required for the repolarization phase of the cardiac action potential. The gene product can form heteromultimers with two other potassium channel proteins, KCNE1 and KCNE3. Mutations in this gene are associated with hereditary long QT syndrome (also known as Romano-Ward syndrome), Jervell and Lange-Nielsen syndrome and familial atrial fibrillation. The gene is located in a region of chromosome 11 that contains a number of contiguous genes, which are abnormally imprinted in cancer and the Beckwith-Wiedemann syndrome. This gene is also imprinted, with preferential expression from the maternal allele in some tissues, excluding cardiac muscle. Alternatively spliced transcripts encoding distinct isoforms have been described. [provided by RefSeq]. View All» UniProt Summary: 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 STRUCTURE: 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 SPECIFICTY: Abundantly expressed in heart, pancreas, prostate, kidney, small intestine and peripheral blood leukocytes. Less abundant in placenta, lung, spleen, colon, thymus, testis and ovaries.DOMAIN: The segment S4 is probably the voltage-sensor and is characterized by a series of positively charged amino acids at every third position. INVOLVEMENT IN DISEASE: Defects in KCNQ1 are the cause of long QT syndrome type 1 (LQT1) [MIM:192500]; 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) [MIM:220400]. 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. Ref.36 Ref.24 Ref.28Defects in KCNQ1 are the cause of atrial fibrillation familial type 3 (ATFB3) [MIM:607554]. 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. Ref.38Defects in KCNQ1 are the cause of short QT syndrome type 2 (SQT2) [MIM:609621]. Short QT syndromes are heart disorders characterized by idiopathic persistently and uniformly short QT interval on ECG in the absence of structural heart disease in affected individuals. They cause syncope and sudden death. Ref.39MISCELLANEOUS: Mutagenesis experiments were carried out by expressing in Xenopus oocytes or COS-7 cells KCNQ1 mutants either individually (homomultimers) or in combination with both wild-type KCNQ1 (mut/wt homomultimers) and minK (heteromultimers).SEQUENCE SIMILARITIES: Belongs to the potassium channel family. KQT (TC 1.A.1.15) subfamily. Kv7.1/KCNQ1 sub-subfamily. [View classification] View All» Product Name: Anti-KCNQ1 Potassium Channel, clone N31A/10 View All» Concentration: 1.0 mg/mL View All» Antibody Type: Monoclonal Antibody View All» Qty/Pk: 100 μg View All» Format: Purified View All» Host: Mouse View All»
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