Altered Crossbridge Kinetics in the αMHC 403/+ Mouse Model of Familial Hypertrophic Cardiomyopathy
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چکیده
منابع مشابه
Altered Crossbridge Kinetics in the aMHC Mouse Model of Familial Hypertrophic Cardiomyopathy
A mutation in the cardiac b-myosin heavy chain, Arg403Gln (R403Q), causes a severe form of familial hypertrophic cardiomyopathy (FHC) in humans. We used small-amplitude (0.25%) length-perturbation analysis to examine the mechanical properties of skinned left ventricular papillary muscle strips from mouse hearts bearing the R403Q mutation in the a-myosin heavy chain (aMHC). Myofibrillar disarray...
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Familial hypertrophic cardiomyopathy (FHC) is an inherited cardiac disease that can result in sudden death in the absence of any overt symptoms. Many of the cases documented to date have been linked with missense mutations in the beta-myosin heavy chain gene. Here we present data detailing the functional impact of one of the most deadly mutations, R403Q, on myosin motor function. Experiments we...
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ژورنال
عنوان ژورنال: Circulation Research
سال: 1999
ISSN: 0009-7330,1524-4571
DOI: 10.1161/01.res.84.4.475