cardiovascular system
• adult hearts appear globose
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• mitochondrial abnormalities in cardiomyocytes with both the numbers and organization showing alterations in 35 week old mice and as early as 12 weeks of age
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• increase in heart weight/body weight ratios in 50 week old mice, with an approximate 20% increase by 12 months
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• mild hypertrophy in the papillary muscle and the left ventricular free wall in adult hearts
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• heart rates in response to exercise are decreased
• however, no arrhythmias are seen during or after exercise
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• skinned fibers from the left ventricular papillary muscles of young adults show decreased maximal unloading shortening velocity and decreased maximal relative power output
• however, hearts exhibit unchanged cardiac work, time to peak pressure, relaxation, and contractility
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behavior/neurological
• mice exhibit impaired exercise capacity, with decreased heart rates in response to exercise
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homeostasis/metabolism
• mice exhibit impaired exercise capacity, with decreased heart rates in response to exercise
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muscle
• mitochondrial abnormalities in cardiomyocytes with both the numbers and organization showing alterations in 35 week old mice and as early as 12 weeks of age
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• cardiac sarcomere arrangements are altered, with sarcomeres out of register with each other and the normal pattern disrupted, in 35 week old mice and as early as 12 weeks of age
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• Z lines do not retain their normal arrangements between the cardiac sarcomeric rows
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cellular
• mitochondrial abnormalities in cardiomyocytes with both the numbers and organization showing alterations in 35 week old mice and as early as 12 weeks of age
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growth/size/body
• increase in heart weight/body weight ratios in 50 week old mice, with an approximate 20% increase by 12 months
|
• mild hypertrophy in the papillary muscle and the left ventricular free wall in adult hearts
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
hypertrophic cardiomyopathy 4 | DOID:0110310 |
OMIM:115197 |
J:59702 |