Cardiac electrophysiological characteristics of the mdx5cv mouse model of Duchenne muscular dystrophy

Dorothy M. Branco, Cordula M. Wolf, Megan Sherwood, Peter E. Hammer, Peter B. Kang, Charles I. Berul

Research output: Contribution to journalArticlepeer-review

Abstract

Background: Duchenne muscular dystrophy (DMD) is a progressive muscle disease caused by mutations in the dystrophin gene. Cardiomyopathy, conduction abnormalities, and ventricular arrhythmias are significant complications of this disease. The mdx5cv mouse carries a dystrophin mutation and demonstrates a more severe phenotype than the classic mdx mouse. Methods: Comprehensive electrophysiological phenotyping was performed in adult mdx 5cv and wildtype mice, including electrocardiography (ECG), implantable Holter monitoring, intracardiac electrophysiological testing, echocardiography, and exercise treadmill testing. Results: ECG performed in mdx5cv mice revealed significantly shorter PR intervals and prominent R waves in surface lead V1. During electrophysiological testing, mdx 5cv mice exhibited longer ventricle effective refractory periods and mildly increased ventricular tachycardia inducibility. There was no evidence for cardiomyopathy or ventricular dysfunction on echocardiography. Histopathology showed no increased myocardial fibrosis. Exercise endurance was lower in mdx5cv mice without arrhythmias or other cardiac abnormalities. Conclusion: Taken together at the age range examined, mdx5cv mice exhibit discrete cardiac electrophysiological dysfunction but display no evidence of structural or contractile abnormalities. Thus, the mdx5cv mouse recapitulates some of the electrophysiological, but not hemodynamic cardiac defects present in human DMD. In certain settings, the mdx5cv mouse may be an appropriate subject for studying electrical pathophysiology and therapy of the cardiac complications of DMD.

Original languageEnglish (US)
Pages (from-to)1-7
Number of pages7
JournalJournal of Interventional Cardiac Electrophysiology
Volume20
Issue number1-2
DOIs
StatePublished - Nov 2007
Externally publishedYes

Bibliographical note

Funding Information:
Supported in part by the Boston Children’s Heart Foundation; PBK was supported by NINDS K08 NS48180 Electronic supplementary material The online version of this article (doi:10.1007/s10840-007-9168-z) contains supplementary material, which is available to authorized users. D.M.Branco.P.E.Hammer.C.I.Berul Department of Cardiology, Children’s Hospital Boston, Boston, MA 02115, USA

Keywords

  • Animal models
  • Electrophysiology
  • Muscular dystrophy

Fingerprint

Dive into the research topics of 'Cardiac electrophysiological characteristics of the mdx<sup>5cv</sup> mouse model of Duchenne muscular dystrophy'. Together they form a unique fingerprint.

Cite this