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PrecisION hKCNQ1/hminK-CHO Recombinant Cell Line
Description:
PrecisION hKCNQ1/hminK-CHO Recombinant Cell Line
Trade Name:
Upstate (Millipore)
Qty/Pk:
2 mL
Product Overview:
Recombinant CHO-K1 cell line co-expressing the human KCNQ1 (potassium voltage-gated channel, KQT-like subfamily, member 1) and human minK (delayed rectifier potassium channel).
Format:
2 x 1 ml aliquots containing 180x 106cells/ml in 10% DMSO at passage 16.
Format:
2 x 1 ml aliquots containing 180x 106cells/ml in 10% DMSO at passage 16.
Background Information:
KCNQ1 and mink form the slow delayed rectifier potassium current, IKs in the heart. This multimeric ion channel contributes to cardiac action potential repolarization and determines the heartbeat rate. Mutations in either KCNQ1 or minK that reduce IKs have been shown to cause long-QT syndrome (LQTS), a disorder of ventricular repolarization that can result in cardiac arrhythmia and sudden death. A well-recognized potential treatment for LQTS caused by reduction of IKs is to enhance functional activation of cardiac KCNQ1/minK channels.
Application Notes:
CHO-K1 cells stably expressing human KCNQ1 and human minK were analysed by patch-clamp and IonWorks. Over 80% of cells expressed >500 pA outward current (n=3180) with a mean current amplitude of 1050 pA. KvLQT1/minK currents were inhibited by standard blockers such as clotrimazole (pIC50 = 5.3±0.1; n=4) and linopirdine (pIC50 5.1±0.1; n=4).
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.
Entrez Gene Summary:
This gene encodes a protein for 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, 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 large number of contiguous genes that are abnormally imprinted in cancer and the Beckwith-Wiedemann syndrome. Two alternative transcripts encoding distinct isoforms have been described.
UniProt Summary:
FUNCTION: SwissProt: P51787 # 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.
SIZE: 676 amino acids; 74699 Da
SUBUNIT: Heteromultimer with KCNE1 (MinK) or KCNE3 (MiRP2).
SUBCELLULAR LOCATION: 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.
DOMAIN: SwissProt: P51787 The segment S4 is probably the voltage-sensor and is characterized by a series of positively charged amino acids at every third position.
DISEASE: SwissProt: P51787 # 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 a cause of Jervell and Lange-Nielsen syndrome (JLNS) [MIM:220400]. JLNS is an autosomal recessive cardio-auditory syndrome characterized by a prolonged QT interval in the electrocardiogram and congenital deafness. & Defects in KCNQ1 are the cause of atrial fibrillation type 1 (ATFB1) [MIM:607554]. ATFB is characterized by rapid and irregular activation of the atrium. ATFB causes thromboembolism, tachycardia-mediated cardiomyopathy, heart failure and ventricular arrhythmia. & Defects 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.
SIMILARITY: Belongs to the potassium channel family. KQT subfamily.
MISCELLANEOUS: 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).
SIZE: 676 amino acids; 74699 Da
SUBUNIT: Heteromultimer with KCNE1 (MinK) or KCNE3 (MiRP2).
SUBCELLULAR LOCATION: 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.
DOMAIN: SwissProt: P51787 The segment S4 is probably the voltage-sensor and is characterized by a series of positively charged amino acids at every third position.
DISEASE: SwissProt: P51787 # 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 a cause of Jervell and Lange-Nielsen syndrome (JLNS) [MIM:220400]. JLNS is an autosomal recessive cardio-auditory syndrome characterized by a prolonged QT interval in the electrocardiogram and congenital deafness. & Defects in KCNQ1 are the cause of atrial fibrillation type 1 (ATFB1) [MIM:607554]. ATFB is characterized by rapid and irregular activation of the atrium. ATFB causes thromboembolism, tachycardia-mediated cardiomyopathy, heart failure and ventricular arrhythmia. & Defects 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.
SIMILARITY: Belongs to the potassium channel family. KQT subfamily.
MISCELLANEOUS: 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).
Species:
Human
Quality Assurance:
Mycoplasma Testing:
The cell line has been screened to confirm the absence of Mycoplasma species
The cell line has been screened to confirm the absence of Mycoplasma species
Cell Type:
CHO
Cell Line Type:
Ion Channel Cell Lines
Components:
Pack contains 2 vials of mycoplasma-free cells, 1 ml per vial
Brand Family:
Upstate
Host Cells:
Chinese Hamster Ovarian K-1 cells (CHO K-1)
Presentation:
2 x 1 ml aliquots
Protein Target:
hKCNQ1/hminK
UniProt Number:
Target Sub-Family:
Potassium
Packaging:
2 x 1 ml aliquots
Gene Symbol:
- KCNQ1
- JLNS1
- ATFB1
- SQT2
- KCNA8
- LQT
- WRS
- Kv1.9
- KVLQT1
- RWS
- Kv7.1
- KCNA9
- LQTS
- FLJ26167
- LQT1
Protein or Enzyme Type:
Ion Channels
Product Name:
PrecisION hKCNQ1/hminK-CHO Recombinant Cell Line
Entrez Gene Number:
Alternate Names:
- KCNQ1
- JLNS1
- ATFB1
- SQT2
- KCNA8
- LQT
- WRS
- Kv1.9
- KVLQT1
- RWS
- Kv7.1
- KCNA9
- LQTS
- FLJ26167
- LQT1