Analysis Tools|
Allele Symbol Allele Name Allele ID |
Tg(Myh7-Phc1)#Yota transgene insertion, Yoshihiro Takihara MGI:5906059 |
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| Summary |
2 genotypes
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• mice exhibit cardiac abnormalities similar to that of single Phc1 homozygous mutants
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• tetralogy of Fallot
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• membranous or muscular ventricular septal defects
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• 100% lethality within 8 months of age
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• ventricles are occupied by abnormal cardiomyocytes at 3 and 6 months of age
• in the most severe regions, H lines are not visible
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• thinning of the myocardium associated with cardiac failure
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• hearts are enlarged with severe reduction of the ventricular wall thickness at autopsy
• however, hypertrophy of the heart is not seen at any stage
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• left ventricular dimension is increased and left ventricular ejection fraction is decreased in 5-6 month old mice
• however, no reduction in left ventricular posterior wall thickness or interventricular septal thickness is seen at 5-6 months of age
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• mice show characteristics of heart failure such as general weakness, lack of food and water intake, labored breathing, generalized edema with gain of body weight
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• the number of the stacks of the mitochondrial cristae is reduced in cardiomyocytes
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• very elongated mitochondria are sometimes seen in cardiomyocytes
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• increase in cardiomyocyte apoptosis at 3 and 6 months of age
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• intracavitary thrombi are frequently seen in the left atrium at autopsy
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• ventricles are occupied by abnormal cardiomyocytes at 3 and 6 months of age
• in the most severe regions, H lines are not visible
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• thinning of the myocardium associated with cardiac failure
|
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• left ventricular dimension is increased and left ventricular ejection fraction is decreased in 5-6 month old mice
• however, no reduction in left ventricular posterior wall thickness or interventricular septal thickness is seen at 5-6 months of age
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• increase in cardiomyocyte apoptosis at 3 and 6 months of age
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• cardiomyocyte sarcomeres show abnormal arrangements with irregular and loose myofilaments
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• in the most severe regions, M lines are not visible in cardiomyocytes
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• Z-lines are vague and enlarged in cardiomyocytes
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• death is preceded for several days by dyspnea
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• hearts are enlarged with severe reduction of the ventricular wall thickness at autopsy
• however, hypertrophy of the heart is not seen at any stage
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| dilated cardiomyopathy | DOID:12930 |
OMIM:PS115200 |
J:75968 | |
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO) |
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last database update 11/05/2024 MGI 6.24 |
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