Mouse Genome Informatics
hm
    Mybpc3tm1Rmos/Mybpc3tm1Rmos
either: (involves: 129) or (involves: 129 * C57BL/6)
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
cardiovascular system
• myocyte disarray and loss of the close lateral alignment of myofibrils are seen
• hypertrophy is seen in 3 week old homozygotes
• heart to body weight ratios are increased as is the thickness of the anterior and posterior walls
• foci of interstitial fibrosis were seen in 3 out of 5 homozygotes
• Ca2+ sensitivity of tension is reduced in myocytes from mutant hearts
• left ventricular fractional shortening is significantly reduced
• skinned cardiomyocytes from homozygotes exhibit faster loaded shortening velocities, greater power output, and increased force redevelopment rates, due to increased crossbridge interaction kinetics resulting from absence of myosin head constraints
• peak normalized power output is increased by 26% in mutant cardiomyocytes during maximal Ca2+ activations
• peak power output is increased to an even greater extent (46%) during half-maximal Ca2+ activations
• the rate constant of force redevelopment (ktr) is unaltered during maximal Ca2+ activations; however, ktr at half-maximal Ca2+ activation is significantly greater in mutant cardiomyocytes relative to wild-type
• hypertrophic cardiomyopathy is seen

muscle
• Ca2+ sensitivity of tension is reduced in myocytes from mutant hearts
• left ventricular fractional shortening is significantly reduced
• hypertrophic cardiomyopathy is seen

Mouse Models of Human Disease
OMIM IDRef(s)
Cardiomyopathy, Familial Hypertrophic, 4; CMH4 115197 J:95725