Abstract
In mammalian cardiac cells, a variety of transient or sustained K+ currents contribute to the repolarization of action potentials. There are two main components of the delayed-rectifier sustained K+ current, I(Kr) (rapid) and I(Ks), (slow). I(Kr) is the product of the gene HERG, which is altered in the long-QT syndrome, LQT2. A channel with properties similar to those of the I(Ks) channel is produced when the cardiac protein IsK is expressed in Xenopus oocytes. However, it is a small protein with a very unusual structure for a cation channel. The LQT1 gene is another gene associated with the LQT syndrome, a disorder that causes sudden death from ventricular arrhythmias. Here we report the cloning of the full-length mouse K(V)LQT1 complementary DNA and show that K(V)LQT1 associates with IsK to form the channel underlying the I(Ks) cardiac current, which is a target of class-III anti-arrhythmic drugs and is involved in the LQT1 syndrome.
Publication types
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Research Support, Non-U.S. Gov't
MeSH terms
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Amino Acid Sequence
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Animals
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COS Cells
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Cation Transport Proteins*
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Cell Line
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Cloning, Molecular
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DNA-Binding Proteins*
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ERG1 Potassium Channel
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Ether-A-Go-Go Potassium Channels
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Humans
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KCNQ Potassium Channels
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KCNQ1 Potassium Channel
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Mice
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Molecular Sequence Data
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Myocardium / metabolism*
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Point Mutation
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Potassium / metabolism
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Potassium Channels / genetics
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Potassium Channels / metabolism*
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Potassium Channels, Voltage-Gated*
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Protein Binding
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Sequence Homology, Amino Acid
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Trans-Activators*
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Transcriptional Regulator ERG
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Transfection
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Xenopus
Substances
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Cation Transport Proteins
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DNA-Binding Proteins
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ERG protein, human
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ERG1 Potassium Channel
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Ether-A-Go-Go Potassium Channels
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KCNH2 protein, human
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KCNH6 protein, human
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KCNQ Potassium Channels
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KCNQ1 Potassium Channel
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KCNQ1 protein, human
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Kcnq1 protein, mouse
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Potassium Channels
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Potassium Channels, Voltage-Gated
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Trans-Activators
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Transcriptional Regulator ERG
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potassium channel protein I(sk)
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Potassium