Analysis Tools
mortality/aging
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• 100% mortality by 21 days of age, with survival declining rapidly from 16 days of age
• mean life span of males is 16.8 days, while the mean life span of females is 18.3 days
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cardiovascular system
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• abnormal myofiber alignment
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• altered alignment of myofibrils, myocyte atrophy and fragmentation, altered distribution of mitochondria between myofibrils, myofibril disarray and discontinuity, degeneration and loss of myocyte structure, collagen accumulation, greater infiltration of other cells, and enlarged extracellular spaces
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• cardiomyocyte hypertrophy
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• degeneration and loss of cardiac myocyte structure
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• areas of necrosis in left ventricular myocardium
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• increase in heart weight to body weight ratio at 14 and 16 days of age
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• 4-chamber cardiac enlargement at 14 days of age
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• biatrial dilatation
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• mice develop severe dilated cardiomyopathy, with enlargement of left ventricular cardiac chambers and a reduction in fractional shortening
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• reduction in cardiac function at 16 to 18 days of age, with decreased fractional shortening which is reduced to below 20% in some mice
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• increase in left ventricular end-diastolic and end-systolic diameter
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• at 14 days of age
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• adrenaline administration induces ventricular arrhythmias in some mice
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• first degree atrioventricular block
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• at 14 days of age
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• increase in P-wave height
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• the corrected QT interval is increased
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immune system
liver/biliary system
homeostasis/metabolism
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• premorbid left atrial thrombus is seen in a subgroup of hearts
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muscle
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• abnormal myofiber alignment
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• altered alignment of myofibrils, myocyte atrophy and fragmentation, altered distribution of mitochondria between myofibrils, myofibril disarray and discontinuity, degeneration and loss of myocyte structure, collagen accumulation, greater infiltration of other cells, and enlarged extracellular spaces
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• cardiomyocyte hypertrophy
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• degeneration and loss of cardiac myocyte structure
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• areas of necrosis in left ventricular myocardium
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• mice develop severe dilated cardiomyopathy, with enlargement of left ventricular cardiac chambers and a reduction in fractional shortening
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• reduction in cardiac function at 16 to 18 days of age, with decreased fractional shortening which is reduced to below 20% in some mice
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respiratory system
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• ratio of lung weight to body weight is increased
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growth/size/body
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• increase in heart weight to body weight ratio at 14 and 16 days of age
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• ratio of lung weight to body weight is increased
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cellular
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| hypertrophic cardiomyopathy | DOID:11984 | J:153302 | ||
