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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Mybpc3tm1Rmos
targeted mutation 1, Richard L Moss
MGI:3526881
Summary 2 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
hm1
Mybpc3tm1Rmos/Mybpc3tm1Rmos either: (involves: 129) or (involves: 129 * C57BL/6) MGI:3527229
hm2
Mybpc3tm1Rmos/Mybpc3tm1Rmos involves: 129 MGI:5523784


Genotype
MGI:3527229
hm1
Allelic
Composition
Mybpc3tm1Rmos/Mybpc3tm1Rmos
Genetic
Background
either: (involves: 129) or (involves: 129 * C57BL/6)
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mybpc3tm1Rmos mutation (0 available); any Mybpc3 mutation (43 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• myocyte disarray and loss of the close lateral alignment of myofibrils are seen
• heart to body weight ratios are increased as is the thickness of the anterior and posterior walls
• hypertrophy is seen in 3 week old homozygotes
• 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

growth/size/body
• heart to body weight ratios are increased as is the thickness of the anterior and posterior walls
• hypertrophy is seen in 3 week old homozygotes

muscle
• myocyte disarray and loss of the close lateral alignment of myofibrils are seen
• Ca2+ sensitivity of tension is reduced in myocytes from mutant hearts
• left ventricular fractional shortening is significantly reduced
• hypertrophic cardiomyopathy is seen

cellular
• foci of interstitial fibrosis were seen in 3 out of 5 homozygotes

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
hypertrophic cardiomyopathy 4 DOID:0110310 OMIM:115197
J:95725




Genotype
MGI:5523784
hm2
Allelic
Composition
Mybpc3tm1Rmos/Mybpc3tm1Rmos
Genetic
Background
involves: 129
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mybpc3tm1Rmos mutation (0 available); any Mybpc3 mutation (43 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• decreased ejection fraction, increased left ventricle posterior wall thickness, decreased isovolumic relaxation time and increased left ventricle internal diameter
• following transaortic constriction, tamoxifen-treated mice exhibit increased left ventricle wall thickness, increased left ventricle internal dimension and reduced fractional shortening compared with control mice

homeostasis/metabolism
• following transaortic constriction, tamoxifen-treated mice exhibit increased left ventricle wall thickness, increased left ventricle internal dimension and reduced fractional shortening compared with control mice

muscle
• decreased ejection fraction, increased left ventricle posterior wall thickness, decreased isovolumic relaxation time and increased left ventricle internal diameter





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Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
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last database update
11/19/2024
MGI 6.24
The Jackson Laboratory