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Published in Volume 99, Issue 4 (February 15,1997)
J. Clin. Invest. 99(4): 570-576 (1997). doi:10.1172/JCI119197.
Copyright © 1997, The American Society for Clinical Investigation.

Research Article

Electrophysiological abnormalities and arrhythmias in alpha MHC mutant familial hypertrophic cardiomyopathy mice.

C I Berul, M E Christe, M J Aronovitz, C E Seidman, J G Seidman and M E Mendelsohn

Division of Pediatric Cardiology, Tufts University School of Medicine, Massachusetts 02111, USA. charles.berul@es.nemc.org

Published February 15, 1997

A new mouse cardiac electrophysiology method was used to study mice harboring an alpha-myosin heavy chain Arg403Gln missense mutation (alpha-MHC403/+), which results in histological and hemodynamic abnormalities characteristic of familial hypertrophic cardiomyopathy (FHC) and sudden death of uncertain etiology during exercise. Wild-type animals had completely normal cardiac electrophysiology. In contrast, FHC mice demonstrated (a) electrocardiographic abnormalities including prolonged repolarization intervals and rightward axis; (b) electrophysiological abnormalities including heterogeneous ventricular conduction properties and prolonged sinus node recovery time; and (c) inducible ventricular ectopy. These data identify distinct electrophysiologic abnormalities in FHC mice with a specific alpha-myosin mutation, and also validate a novel method to explore in vivo the relationship between specific genotypes and their electrophysiologic phenotypes.

This article Copyright © 1997, The American Society for Clinical Investigation.

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