mortality/aging
• about 60% die within the first 2 weeks after birth
|
cardiovascular system
• show signs of advanced chronic hepatic and pulmonary congestion
|
• mitochondria are randomly dispersed in clusters and appear swollen
|
• cardiomyocyte hypertrophy and disarray
|
• extensive focal interstitial fibrosis in ventricles
|
• heart size of adults is increased
|
cellular
• extensive focal interstitial fibrosis in ventricles
|
• MEFs exhibit a marked increase in basal polarization of mitochondria and fast mitochondrial depolarization in response to oligomycin
|
homeostasis/metabolism
behavior/neurological
muscle
• mitochondria are randomly dispersed in clusters and appear swollen
|
• heart size of adults is increased
|
respiratory system
• mutants surviving the first month after birth become dyspneic
|
liver/biliary system
growth/size/body
• cardiomyocyte hypertrophy and disarray
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
dilated cardiomyopathy | DOID:12930 |
OMIM:PS115200 |
J:95691 |