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Phenotypes Associated with This Genotype
Genotype
MGI:3799202
Allelic
Composition
Tg(Myh6*)140Lnwd/0
Genetic
Background
involves: C57BL/6 * CBA
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phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• mice show increased mortality by one year compared to controls

cardiovascular system
• thickening of the smooth muscle layer of the small coronary vessels (J:36522)
• hearts show evidence of small vessel coronary disease, exhibiting thickening of the smooth muscle layer of the small coronary vessels (J:36522)
• myocardial fiber degeneration [MP:0004566] myofibrillar disarray and degeneration
• severely affected myocytes often border normal cells with frequent loss of myofibrils bordering the intercalated disc
• mice have slightly increased heart weight to body weight ratio compared to control mice
• mutants exhibit hypertrophic cardiomyopathy including focal myocyte hypertrophy and myocellular disarray with increased matrix accumulation (J:31537)
• hypertrophied myocytes are seen throughout the left ventricle with the largest number in the septum (J:31537)
• cardiac hypertrophy is seen by 12 weeks of age (J:36522)
• fibrosis and cellular disarray are apparent to variable extent at 8 months of age (J:134706)
• left ventricular hypertrophy (35% increase) is observed by 14-15 weeks of age (J:31537)
• hypertrophic cardiopathy in the left ventricle at 12-14 weeks of age, with focal myocyte hypertrophy and myocellular disarray with increased matrix accumulation (J:36522)
• hypertrophy increases with age in females such than by 8 months of age, it more than doubles that observed at 12 weeks of age (J:36522)
• males do not exhibit increased hypertrophy at 8 months of age but exhibit severe dilation (J:36522)
• mice show modest hypertrophy of the left ventricle (J:134706)
• males do not exhibit increased hypertrophy at 8 months of age but exhibit severe dilation, with a dilated ventricular cavity that is almond-shaped
• mice have decreased fractional shortening at 8 and 12 months but not 4 months of age

homeostasis/metabolism
• mice have impaired exercise tolerance on a treadmill compared to controls at 8 months of age

muscle
• thickening of the smooth muscle layer of the small coronary vessels (J:36522)
• myocardial fiber degeneration [MP:0004566] myofibrillar disarray and degeneration
• severely affected myocytes often border normal cells with frequent loss of myofibrils bordering the intercalated disc
• mice have decreased fractional shortening at 8 and 12 months but not 4 months of age

behavior/neurological
• mice have impaired exercise tolerance on a treadmill compared to controls at 8 months of age

Mouse Models of Human Disease
OMIM ID Ref(s)
Cardiomyopathy, Familial Hypertrophic, 14; CMH14 613251 J:31537 , J:36522


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Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
11/22/2016
MGI 6.06
The Jackson Laboratory