A new mouse cardiac electrophysiology method was used to study mice harboring an α-myosin heavy chain Arg403Gln missense mutation (α- MHC(403/403)), 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 α-myosin mutation, and also validate a novel method to explore in vivo the relationship between specific genotypes and their electrophysiologic phenotypes.
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Berul, C. I., Christe, M. E., Aronovitz, M. J., Seidman, C. E., Seidman, J. G., & Mendelsohn, M. E. (1997). Electrophysiological abnormalities and arrhythmias in αMHC mutant familial hypertrophic cardiomyopathy mice. Journal of Clinical Investigation, 99(4), 570–576. https://doi.org/10.1172/JCI119197