Abnormal sodium current properties contribute to cardiac electrical and contractile dysfunction in a mouse model of myotonic dystrophy type 1
V Algalarrondo, K Wahbi, F Sebag, G Gourdon… - Neuromuscular …, 2015 - Elsevier
Abstract Myotonic dystrophy type 1 (DM1) is the most common neuromuscular disorder and
is associated with cardiac conduction defects. However, the mechanisms of cardiac
arrhythmias in DM1 are unknown. We tested the hypothesis that abnormalities in the cardiac
sodium current (I Na) are involved, and used a transgenic mouse model reproducing the
expression of triplet expansion observed in DM1 (DMSXL mouse). The injection of the class-
I antiarrhythmic agent flecainide induced prominent conduction abnormalities and …
is associated with cardiac conduction defects. However, the mechanisms of cardiac
arrhythmias in DM1 are unknown. We tested the hypothesis that abnormalities in the cardiac
sodium current (I Na) are involved, and used a transgenic mouse model reproducing the
expression of triplet expansion observed in DM1 (DMSXL mouse). The injection of the class-
I antiarrhythmic agent flecainide induced prominent conduction abnormalities and …