Literature DB >> 33322401

Insights into Cardiac IKs (KCNQ1/KCNE1) Channels Regulation.

Xiaoan Wu1, H Peter Larsson1.   

Abstract

The delayed rectifier potassium IKs channel is an important regulator of the duration of the ventricular action potential. Hundreds of mutations in the genes (KCNQ1 and KCNE1) encoding the IKs channel cause long QT syndrome (LQTS). LQTS is a heart disorder that can lead to severe cardiac arrhythmias and sudden cardiac death. A better understanding of the IKs channel (here called the KCNQ1/KCNE1 channel) properties and activities is of great importance to find the causes of LQTS and thus potentially treat LQTS. The KCNQ1/KCNE1 channel belongs to the superfamily of voltage-gated potassium channels. The KCNQ1/KCNE1 channel consists of both the pore-forming subunit KCNQ1 and the modulatory subunit KCNE1. KCNE1 regulates the function of the KCNQ1 channel in several ways. This review aims to describe the current structural and functional knowledge about the cardiac KCNQ1/KCNE1 channel. In addition, we focus on the modulation of the KCNQ1/KCNE1 channel and its potential as a target therapeutic of LQTS.

Entities:  

Keywords:  ATP; IKs; KCNE1; KCNQ1; Kv channel; PIP2; PKA; PUFA; cardiac arrhythmias; long QT syndrome

Year:  2020        PMID: 33322401      PMCID: PMC7763278          DOI: 10.3390/ijms21249440

Source DB:  PubMed          Journal:  Int J Mol Sci        ISSN: 1422-0067            Impact factor:   5.923


  150 in total

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3.  A trafficking-deficient KCNQ1 mutation, T587M, causes a severe phenotype of long QT syndrome by interfering with intracellular hERG transport.

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5.  TEA(+)-sensitive KCNQ1 constructs reveal pore-independent access to KCNE1 in assembled I(Ks) channels.

Authors:  J Kurokawa; H K Motoike; R S Kass
Journal:  J Gen Physiol       Date:  2001-01       Impact factor: 4.086

6.  The I Ks Ion Channel Activator Mefenamic Acid Requires KCNE1 and Modulates Channel Gating in a Subunit-Dependent Manner.

Authors:  Yundi Wang; Jodene Eldstrom; David Fedida
Journal:  Mol Pharmacol       Date:  2019-11-13       Impact factor: 4.436

7.  I Ks ion-channel pore conductance can result from individual voltage sensor movements.

Authors:  Maartje Westhoff; Jodene Eldstrom; Christopher I Murray; Emely Thompson; David Fedida
Journal:  Proc Natl Acad Sci U S A       Date:  2019-03-27       Impact factor: 11.205

8.  Characterization of KCNQ1 atrial fibrillation mutations reveals distinct dependence on KCNE1.

Authors:  Priscilla J Chan; Jeremiah D Osteen; Dazhi Xiong; Michael S Bohnen; Darshan Doshi; Kevin J Sampson; Steven O Marx; Arthur Karlin; Robert S Kass
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Review 9.  ArrhythmoGenoPharmacoTherapy.

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10.  Fatty acid analogue N-arachidonoyl taurine restores function of IKs channels with diverse long QT mutations.

Authors:  Sara I Liin; Johan E Larsson; Rene Barro-Soria; Bo Hjorth Bentzen; H Peter Larsson
Journal:  Elife       Date:  2016-09-30       Impact factor: 8.140

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4.  Regulation of acid-sensing ion channels by protein binding partners.

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6.  Disruption of a Conservative Motif in the C-Terminal Loop of the KCNQ1 Channel Causes LQT Syndrome.

Authors:  Maria Karlova; Denis V Abramochkin; Ksenia B Pustovit; Tatiana Nesterova; Valery Novoseletsky; Gildas Loussouarn; Elena Zaklyazminskaya; Olga S Sokolova
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7.  A general mechanism of KCNE1 modulation of KCNQ1 channels involving non-canonical VSD-PD coupling.

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