mortality/aging
• approximately 20% of mice exhibit preweaning mortality
|
cardiovascular system
• P10 cardiomyocytes show disorganization of the entire transverse-axial tubule system, with the T-tubule component decreased and the longitudinal component increased
• mice have a higher number of cardiomyocytes missing the transverse component of transverse-axial tubule system, suggesting delayed T-tubule network formation
|
• hearts show slightly enlarged left ventricle at P10
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• mice exhibit progressive dilated cardiomyopathy
• hearts show a progressive chamber dilation that is visible at 3 months of age
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• hearts show reduced percentage of fractional shortening at P10 and 3 months of age
|
• echocardiography shows increased left ventricular dimensions and reduced cardiac function at P10 and 3 months of age
• however, no alterations in thickness of left ventricular posterior wall end diastole, calculated ratio of left ventricular mass to body weight, or thickness of interventricular septal end diastole are seen
|
muscle
• P10 cardiomyocytes show disorganization of the entire transverse-axial tubule system, with the T-tubule component decreased and the longitudinal component increased
• mice have a higher number of cardiomyocytes missing the transverse component of transverse-axial tubule system, suggesting delayed T-tubule network formation
|
• mice exhibit progressive dilated cardiomyopathy
• hearts show a progressive chamber dilation that is visible at 3 months of age
|
• hearts show reduced percentage of fractional shortening at P10 and 3 months of age
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
dilated cardiomyopathy 1CC | DOID:0110424 |
OMIM:613122 |
J:301807 |