Mouse Anti-KCNQ1 Recombinant Antibody (S37A-10) (CBMAB-K0060-LY)

Mouse

Human, Mouse, Rat

S37A-10

IgG1

WB, IP, IF/ICC, IHC-P

Fusion protein amino acids 2-101 of human KCNQ1

Human, Mouse, Rat

IgG1

Monoclonal

The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Liquid

50% Glycerol

> 95% Purity determined by SDS-PAGE.

Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Potassium Voltage-Gated Channel Subfamily Q Member 1

This gene encodes a voltage-gated potassium channel required for repolarization phase of the cardiac action potential. This protein can form heteromultimers with two other potassium channel proteins, KCNE1 and KCNE3. Mutations in this gene are associated with hereditary long QT syndrome 1 (also known as Romano-Ward syndrome), Jervell and Lange-Nielsen syndrome, and familial atrial fibrillation. This gene exhibits tissue-specific imprinting, with preferential expression from the maternal allele in some tissues, and biallelic expression in others. This gene is located in a region of chromosome 11 amongst other imprinted genes that are associated with Beckwith-Wiedemann syndrome (BWS), and itself has been shown to be disrupted by chromosomal rearrangements in patients with BWS. Alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Aug 2011]

Potassium Voltage-Gated Channel Subfamily Q Member 1; IKs Producing Slow Voltage-Gated Potassium Channel Subunit Alpha KvLQT1; Potassium Channel; Voltage Gated KQT-Like Subfamily Q; Member 1; Potassium Voltage-Gated Channel; KQT-Like Subfamily; Member 1; Voltage-Gated Potassium Channel Subunit Kv7.1; KVLQT1; KCNA8; KCNA9; Potassium Voltage-Gated Channel Subfamily KQT Member 1; Kidney And Cardiac Voltage Dependend K+ Channel; Slow Delayed Rectifier Channel Subunit; Jervell And Lange-Nielsen Syndrome 1;

Potassium channel that plays an important role in a number of tissues, including heart, inner ear, stomach and colon (PubMed:10646604, PubMed:25441029).
Associates with KCNE beta subunits that modulates current kinetics (PubMed:9312006, PubMed:9108097, PubMed:8900283, PubMed:10646604, PubMed:11101505, PubMed:19687231).
Induces a voltage-dependent current by rapidly activating and slowly deactivating potassium-selective outward current (PubMed:9312006, PubMed:9108097, PubMed:8900283, PubMed:10646604, PubMed:11101505, PubMed:25441029).
Promotes also a delayed voltage activated potassium current showing outward rectification characteristic (By similarity).
During beta-adrenergic receptor stimulation participates in cardiac repolarization by associating with KCNE1 to form the I(Ks) cardiac potassium current that increases the amplitude and slows down the activation kinetics of outward potassium current I(Ks) (By similarity) (PubMed:9312006, PubMed:9108097, PubMed:8900283, PubMed:10646604, PubMed:11101505).
Muscarinic agonist oxotremorine-M strongly suppresses KCNQ1/KCNE1 current (PubMed:10713961).
When associated with KCNE3, forms the potassium channel that is important for cyclic AMP-stimulated intestinal secretion of chloride ions (PubMed:10646604).
This interaction with KCNE3 is reduced by 17beta-estradiol, resulting in the reduction of currents (By similarity).
During conditions of increased substrate load, maintains the driving force for proximal tubular and intestinal sodium ions absorption, gastric acid secretion, and cAMP-induced jejunal chloride ions secretion (By similarity).
Allows the provision of potassium ions to the luminal membrane of the secretory canaliculus in the resting state as well as during stimulated acid secretion (By similarity).
When associated with KCNE2, forms a heterooligomer complex leading to currents with an apparently instantaneous activation, a rapid deactivation process and a linear current-voltage relationship and decreases the amplitude of the outward current (PubMed:11101505).
When associated with KCNE4, inhibits voltage-gated potassium channel activity (PubMed:19687231).
When associated with KCNE5, this complex only conducts current upon strong and continued depolarization (PubMed:12324418).
Also forms a heterotetramer with KCNQ5; has a voltage-gated potassium channel activity (PubMed:24855057).
Binds with phosphatidylinositol 4,5-bisphosphate (PubMed:25037568).
Isoform 2
Non-functional alone but modulatory when coexpressed with the full-length isoform 1.

Adrenergic receptor signaling pathwayIEA:Ensembl
Atrial cardiac muscle cell action potentialManual Assertion Based On ExperimentIMP:BHF-UCL
Auditory receptor cell developmentIEA:Ensembl
Cardiac muscle contractionManual Assertion Based On ExperimentIMP:BHF-UCL
Cellular chloride ion homeostasisIEA:Ensembl
Cellular response to cAMPManual Assertion Based On ExperimentIDA:BHF-UCL
Cellular response to epinephrine stimulusManual Assertion Based On ExperimentTAS:BHF-UCL
Cellular response to xenobiotic stimulusManual Assertion Based On ExperimentIDA:BHF-UCL
Cochlea developmentIEA:Ensembl
Corticosterone secretionIEA:Ensembl
Detection of mechanical stimulus involved in sensory perception of soundIEA:Ensembl
Erythrocyte differentiationIEA:Ensembl
Gastrin-induced gastric acid secretionIEA:Ensembl
Gene expressionIEA:Ensembl
Glucose metabolic processIEA:Ensembl
Heart developmentIEA:Ensembl
Inner ear developmentISS:UniProtKB
Inner ear morphogenesisIEA:Ensembl
Intestinal absorptionISS:UniProtKB
Iodide transportIEA:Ensembl
Membrane repolarization during action potentialManual Assertion Based On ExperimentIDA:BHF-UCL
Membrane repolarization during atrial cardiac muscle cell action potentialManual Assertion Based On ExperimentIMP:BHF-UCL
Membrane repolarization during cardiac muscle cell action potentialManual Assertion Based On ExperimentIMP:BHF-UCL
Membrane repolarization during ventricular cardiac muscle cell action potentialManual Assertion Based On ExperimentIMP:BHF-UCL
Negative regulation of delayed rectifier potassium channel activityIDA:UniProtKB
Negative regulation of gene expressionIEA:Ensembl
Negative regulation of voltage-gated potassium channel activityIDA:UniProtKB
Neuromuscular processIEA:Ensembl
Non-motile cilium assemblyIEA:Ensembl
Positive regulation of cardiac muscle contractionManual Assertion Based On ExperimentIMP:BHF-UCL
Positive regulation of heart rateManual Assertion Based On ExperimentIMP:BHF-UCL
Positive regulation of potassium ion transmembrane transportManual Assertion Based On ExperimentIDA:BHF-UCL
Potassium ion export across plasma membraneManual Assertion Based On ExperimentIDA:BHF-UCL
Potassium ion homeostasisIEA:Ensembl
Potassium ion import across plasma membraneIEA:Ensembl
Potassium ion transmembrane transportManual Assertion Based On ExperimentIDA:BHF-UCL
Regulation of atrial cardiac muscle cell membrane repolarizationManual Assertion Based On ExperimentIMP:BHF-UCL
Regulation of blood pressureIEA:Ensembl
Regulation of gastric acid secretionISS:UniProtKB
Regulation of gene expression by genetic imprintingIEA:Ensembl
Regulation of heart contraction1 PublicationIC:BHF-UCL
Regulation of heart rate by cardiac conductionManual Assertion Based On ExperimentIMP:BHF-UCL
Regulation of membrane repolarizationManual Assertion Based On ExperimentIDA:BHF-UCL
Biological Process regulation of ventricular cardiac muscle cell membrane repolarizationManual Assertion Based On ExperimentIMP:BHF-UCL
Regulation of voltage-gated sodium channel activityIEA:Ensembl
Renal absorptionISS:UniProtKB
Renal sodium ion absorptionIEA:Ensembl
Response to insulinIEA:Ensembl
Response to nicotineIEA:Ensembl
Rhythmic behaviorIEA:Ensembl
Sensory perception of soundManual Assertion Based On ExperimentTAS:ProtInc
Social behaviorIEA:Ensembl
Stomach developmentIEA:Ensembl
Ventricular cardiac muscle cell action potentialManual Assertion Based On ExperimentIMP:BHF-UCL

Cell membrane; Cytoplasmic vesicle membrane; Early endosome; Membrane raft; Endoplasmic reticulum; Basolateral cell membrane. Colocalized with KCNE3 at the plasma membrane (PubMed:10646604).
Upon 17beta-oestradiol treatment, colocalizes with RAB5A at early endosome (PubMed:23529131).
Heterotetramer with KCNQ5 is highly retained at the endoplasmic reticulum and is localized outside of lipid raft microdomains (PubMed:24855057).
During the early stages of epithelial cell polarization induced by the calcium switch it removed from plasma membrane to the endoplasmic reticulum where it retained and it is redistributed to the basolateral cell surface in a PI3K-dependent manner at a later stage (PubMed:21228319).

Long QT syndrome 1 (LQT1):
A heart disorder 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, and can present with a sentinel event of sudden cardiac death in infancy.
Jervell and Lange-Nielsen syndrome 1 (JLNS1):
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.
Atrial fibrillation, familial, 3 (ATFB3):
An autosomal dominant form of atrial fibrillation, a common sustained cardiac rhythm disturbance. Atrial fibrillation 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.
Short QT syndrome 2 (SQT2):
A heart disorder characterized by idiopathic persistently and uniformly short QT interval on ECG in the absence of structural heart disease in affected individuals. It causes syncope and sudden death.
Diabetes mellitus, non-insulin-dependent (NIDDM):
A multifactorial disorder of glucose homeostasis caused by a lack of sensitivity to the body's own insulin. Affected individuals usually have an obese body habitus and manifestations of a metabolic syndrome characterized by diabetes, insulin resistance, hypertension and hypertriglyceridemia. The disease results in long-term complications that affect the eyes, kidneys, nerves, and blood vessels.

Cytoplasmic: 1-121
Helical: 122-142
Extracellular: 143-147
Helical: 148-168
Cytoplasmic: 169-196
Helical: 197-217
Extracellular: 218-225
Helical: 226-248
Cytoplasmic: 249-261
Helical: 262-282
Extracellular: 283-299
Pore-forming: 300-320
Extracellular: 321-327
Helical: 328-348
Cytoplasmic: 349-676

Phosphorylation at Ser-27 by PKA; increases delayed rectifier potassium channel activity of the KCNQ1-KCNE1 complex through a macromolecular complex that includes PKA, PP1, and the targeting protein AKAP9.
Ubiquitinated by NEDD4L; promotes internalization (PubMed:22024150).
The ubiquitinylated form is internalized through a clathrin-mediated endocytosis by interacting with AP2M1 and is recycled back to the cell membrane via RAB4A and RAB11A (PubMed:23529131).
Deubiquitinated by USP2; counteracts the NEDD4L-specific down-regulation of I(Ks) and restores the membrane localization.