mortality/aging
• most mice die within 1.5 months
|
cardiovascular system
• the ventricular wall shows moderate myocyte hypertrophy with severe diffuse hyalinization of the myocyte cytoplasm
• myocyte cytoplasm shows loss of striation and a homogenous, ground-glass appearance
|
• the ventricular wall shows moderate myocyte hypertrophy
|
• increase in heart weight-to-body weight ratio at 1 month of age
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• dilation in both ventricles by 2 weeks of age
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• mice develop a severe dilated cardiomyopathy phenotype by 2 weeks, with dilation seen in both the ventricles
|
• cardiac output is reduced in 1 month old mice
• however, heart rate is unaffected
|
• 1 month old hearts ex vivo show reduced rates of relaxation and contraction with increased time to peak pressure and half-time to relaxation
|
• hearts show increased left ventricle diastolic and systolic diameter and a reduction in the left ventricle fractional shortening in 1 month old mice
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• 1 month old hearts ex vivo show reduced rates of relaxation and contraction with increased time to peak pressure and half-time to relaxation
|
• end-diastolic and diastolic pressures are increased in 1 month old hearts ex vivo
|
• systolic pressure is decreased in 1 month old hearts ex vivo
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• skinned left ventricular fiber bundles from 1 month old mice show a severe depression in maximum tension and a decrease in myofilament calcium sensitivity
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homeostasis/metabolism
• hearts show a blunted response to both low and high concentrations of isoproterenol, a beta-adrenergic agonist
|
muscle
• myocyte cytoplasm shows loss of striation and a homogenous, ground-glass appearance
• the ventricular wall shows moderate myocyte hypertrophy with severe diffuse hyalinization of the myocyte cytoplasm
|
• the ventricular wall shows moderate myocyte hypertrophy
|
• mice develop a severe dilated cardiomyopathy phenotype by 2 weeks, with dilation seen in both the ventricles
|
• 1 month old hearts ex vivo show reduced rates of relaxation and contraction with increased time to peak pressure and half-time to relaxation
|
• hearts show increased left ventricle diastolic and systolic diameter and a reduction in the left ventricle fractional shortening in 1 month old mice
|
• 1 month old hearts ex vivo show reduced rates of relaxation and contraction with increased time to peak pressure and half-time to relaxation
|
growth/size/body
• increase in heart weight-to-body weight ratio at 1 month of age
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
dilated cardiomyopathy 1Y | DOID:0110457 |
OMIM:611878 |
J:137813 |