Abnormal sodium current properties contribute to cardiac electrical and contractile dysfunction in a mouse model of myotonic dystrophy type 1
Highlights • Flecainide unmasked conduction disorders in the DMSXL mouse model of DM1. • On action potential recording, the [dV/dt]max , was lower in DMSXL mice. • Inactivation and recovery from inactivation of sodium current INa were faster in DMSXL myocytes. • The lower [dV/dt]max was due to a 1.7...
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creator | Algalarrondo, Vincent Wahbi, Karim Sebag, Frédéric Gourdon, Geneviève Beldjord, Chérif Azibi, Kamel Balse, Elise Coulombe, Alain Fischmeister, Rodolphe Eymard, Bruno Duboc, Denis Hatem, Stéphane N |
description | Highlights • Flecainide unmasked conduction disorders in the DMSXL mouse model of DM1. • On action potential recording, the [dV/dt]max , was lower in DMSXL mice. • Inactivation and recovery from inactivation of sodium current INa were faster in DMSXL myocytes. • The lower [dV/dt]max was due to a 1.7 times faster rate of INa inactivation in DMSXL myocytes. • The study points the sodium current as a major component of the rhythmic phenotype in DM1. |
doi_str_mv | 10.1016/j.nmd.2014.11.018 |
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subjects | Action Potentials - drug effects Action Potentials - physiology Aging Animals Anti-Arrhythmia Agents - pharmacology Arrhythmia basic study Arrhythmias, Cardiac - physiopathology Brugada Syndrome Cardiac Conduction System Disease Cardiology and cardiovascular system Computer Simulation Conduction disorders Disease Models, Animal Echocardiography, Doppler Electrophysiology Flecainide - pharmacology Heart Conduction System - abnormalities Heart Conduction System - physiopathology Human health and pathology Life Sciences Male Mice, Transgenic Microelectrodes Models, Cardiovascular Models, Neurological Myocytes, Cardiac - drug effects Myocytes, Cardiac - physiology Myotonic Dystrophy - genetics Myotonic Dystrophy - physiopathology Myotonic dystrophy type 1 NAV1.5 Voltage-Gated Sodium Channel - genetics Neurology Patch-Clamp Techniques Sodium - metabolism Sodium current channelopathy Steinert disease Transgenic mice Voltage-Gated Sodium Channel Blockers - pharmacology |
title | Abnormal sodium current properties contribute to cardiac electrical and contractile dysfunction in a mouse model of myotonic dystrophy type 1 |
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