Description
Myh6-p.R404Q is a point mutation knock-in mouse on the C57BL/6J background carrying the murine equivalent of the human pathogenic MYH7 R403Q mutation. The model spontaneously develops cardiac hypertrophy, enhanced myocardial contractility, reduced ventricular chamber size, decreased cardiac output, and myocardial fibrosis, closely recapitulating familial hypertrophic cardiomyopathy. It is suitable for disease mechanism studies and evaluation of therapeutics for hypertrophic cardiomyopathy