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...

Ausführliche Beschreibung

Gespeichert in:

Bibliographische Detailangaben
Veröffentlicht in:	Neuromuscular disorders : NMD 2015-04, Vol.25 (4), p.308-320
Hauptverfasser:	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
Format:	Artikel
Sprache:	eng
Schlagworte:	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
Online-Zugang:	Volltext
Tags:	Tag hinzufügen Keine Tags, Fügen Sie den ersten Tag hinzu!

Beschreibung
Zusammenfassung:	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.
ISSN:	0960-8966 1873-2364
DOI:	10.1016/j.nmd.2014.11.018