mortality/aging
• all mice die suddenly starting at about 6 weeks of age, between 6 and 12 weeks of age and with a median age of death of 2 months
• mice often die during mating and during attempted surgical procedures and hearts show evidence of stress at the molecular level, indicating increased cardiac physiological stress
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cardiovascular system
• disaggregation of adjacent cardiomyocytes within heart tissue
• however, mice show no evidence of skeletal muscle defects
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• hearts are grossly enlarged, with a 2-fold increase in the heart-to-body mass ratio
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• dilated left ventricular chambers
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• fibrosis in left ventricle and accumulation of interstitial fibrotic tissue in hearts
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• mice exhibit enlarged hearts and impaired contraction of hearts leading to heart failure by 6-12 weeks of age
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• ejection fraction is greatly reduced, indicating impaired pumping capacity of the heart
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• echocardiography indicates an increase in end diastolic and end systolic areas and reduced ejection fraction
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• mice exhibit labored breathing, lack of physical strength, disorientation, problems with balance, and hunched, withdrawn behavior before death, indicating heart failure
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muscle
• mice exhibit enlarged hearts and impaired contraction of hearts leading to heart failure by 6-12 weeks of age
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• ejection fraction is greatly reduced, indicating impaired pumping capacity of the heart
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growth/size/body
• hearts are grossly enlarged, with a 2-fold increase in the heart-to-body mass ratio
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cellular
• fibrosis in left ventricle and accumulation of interstitial fibrotic tissue in hearts
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
dilated cardiomyopathy | DOID:12930 |
OMIM:PS115200 |
J:112174 |