Electrophysiological abnormalities and arrhythmias in alpha MHC mutant familial hypertrophic cardiomyopathy mice
Electrophysiological abnormalities and arrhythmias in alpha MHC mutant familial hypertrophic cardiomyopathy mice
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DOI：
10.1172/jci119197
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发表时间：
1997-02-15
影响因子：
15.9
通讯作者：
Mendelsohn, ME
中科院分区：
文献类型：
--
作者：
Berul, CI;Christe, ME;Mendelsohn, ME
采用一种新的小鼠心脏电生理学方法，对携带α-肌球蛋白重链Arg403Gln错义突变(α-MHC(403/+))的小鼠进行研究，该突变可导致以家族性肥厚型心肌病(FHC)为特征的组织学和血流动力学异常，并导致原因不明的运动猝死。野生型动物心脏电生理完全正常。相比之下，FHC小鼠表现出(A)心电图异常，包括复极间期延长和电轴向右；(B)电生理异常，包括不同的心室传导特性和窦房结恢复时间延长；以及(C)诱导性心室异位。这些数据确定了具有特定α-肌球蛋白突变的FHC小鼠明显的电生理异常，并验证了一种在体内探索特定基因类型与其电生理表型之间关系的新方法。
A new mouse cardiac electrophysiology method was used to study mice harboring an alpha-myosin heavy chain Arg403Gln missense mutation (alpha-MHC(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 alpha-myosin mutation, and also validate a novel method to explore in vivo the relationship between specific genotypes and their electrophysiologic phenotypes.