Electrophysiological basis of cardiac arrhythmia in a mouse model of myotonic dystrophy type 1.

ABSTRACT: Introduction: Myotonic dystrophy type 1 (DM1) is a multisystemic genetic disorder caused by the increased number of CTG repeats in 3' UTR of Dystrophia Myotonia Protein Kinase (DMPK) gene. DM1 patients experience conduction abnormalities as well as atrial and ventricular arrhythmias with increased susceptibility to sudden cardiac death. The ionic basis of these electrical abnormalities is poorly understood. Methods: We evaluated the surface electrocardiogram (ECG) and key ion currents underlying the action potential (AP) in a mouse model of DM1, DMSXL, which express over 1000 CTG repeats. Sodium current (I_Na), L-type calcium current (I_CaL), transient outward potassium current (I_to), and APs were recorded using the patch-clamp technique.

SUBMITTER: Ginjupalli VKM 

PROVIDER: S-EPMC10551179 | biostudies-literature | 2023

REPOSITORIES: biostudies-literature