Analysis Tools
mortality/aging
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• mice die at 12-16 months of age
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cardiovascular system
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• the desmin network is disrupted in hearts, with the striated pattern absent and aberrant aggregates of desmin
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• cardiomyocytes show eosinophilic aggregates, with number and size of aggregates increasing with age
• cardiomyocytes show two populations of electron-dense granular aggregates in the cytoplasm, type I and type II
• type I aggregates have low electron density and occupy a large portion of the central part of the cardiomyocyte, are larger and more regular in shape than type II aggregates, have clear boundaries but are not enclosed in a membrane, are Cryab positive
• type II aggregates are small but more numerous, irregular in shape and surrounded by numerous fine filaments, some are associated with the nuclear envelope and others with the Z-band, are Cryab and desmin positive
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• cardiomyocyte size progressively increases in both the left and right ventricles
• both cardiomyocyte length and transverse sectional area are larger
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• hearts exhibit hypertrophy, with increases in the ventricular weight/tibial length ratios
• both cardiomyocyte length and transverse sectional area are larger, indicating concentric hypertrophy
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• hearts are grossly enlarged and dilated
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• sometimes calcification is seen in hearts
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• +dP/dt is higher in hearts, indicating contractile function is impaired
• baseline absolute value of dP/dt max is decreased
• when stimulated with catecholamines, hearts are unable to maintain normal contractility
• the cardiac response to beta-agonist stimulation via dobutamine infusion is blunted
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• relaxation, as measured by the first derivative of left ventricular pressure (-dP/dt) is lower
• baseline absolute value of dP/dt min is decreased
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• mice exhibit pulmonary and hepatic congestion, pleural effusion, ascites, and subcutaneous edema are seen in mice that die, indicating congestive heart failure
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homeostasis/metabolism
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• atrial thrombosis
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liver/biliary system
muscle
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• cardiomyocytes show eosinophilic aggregates, with number and size of aggregates increasing with age
• cardiomyocytes show two populations of electron-dense granular aggregates in the cytoplasm, type I and type II
• type I aggregates have low electron density and occupy a large portion of the central part of the cardiomyocyte, are larger and more regular in shape than type II aggregates, have clear boundaries but are not enclosed in a membrane, are Cryab positive
• type II aggregates are small but more numerous, irregular in shape and surrounded by numerous fine filaments, some are associated with the nuclear envelope and others with the Z-band, are Cryab and desmin positive
|
|
• cardiomyocyte size progressively increases in both the left and right ventricles
• both cardiomyocyte length and transverse sectional area are larger
|
|
• +dP/dt is higher in hearts, indicating contractile function is impaired
• baseline absolute value of dP/dt max is decreased
• when stimulated with catecholamines, hearts are unable to maintain normal contractility
• the cardiac response to beta-agonist stimulation via dobutamine infusion is blunted
|
|
• relaxation, as measured by the first derivative of left ventricular pressure (-dP/dt) is lower
• baseline absolute value of dP/dt min is decreased
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• alignment of adjacent myofibrils at the Z-band is perturbed in hearts
• Z-band thickness is increased and its normal uniformity lost in hearts
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respiratory system
integument
growth/size/body
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• hearts exhibit hypertrophy, with increases in the ventricular weight/tibial length ratios
• both cardiomyocyte length and transverse sectional area are larger, indicating concentric hypertrophy
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| myofibrillar myopathy 2 | DOID:0080093 |
OMIM:608810 |
J:133093 | |
