Analysis Tools|
Allele Symbol Allele Name Allele ID |
Tg(Myh6-Rxra)41Pcn transgene insertion 41, Pierre Chambon MGI:5906918 |
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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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• no fetuses are recovered after E16.5
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• ventricular myocardial hypoplasia at E14.5
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• about 20% of mice die over a period of 1 year
• death occurs either suddenly or is preceded by general weakness, dyspnea, and lack of food and water intake
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• myocardium shows many necrotic cardiomyocytes showing loss of myofibrils and nuclear destruction
• abnormal myofibril organization in cardiomyocytes is abnormal and myofibrils are thinner
• shape of the nuclei in myocytes is often altered, showing numerous deep indentations
• however, hypertrophy of cardiac myocytes is not seen
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• swelling of cardiomyocytes, absence of compaction of the myofibrils and loss of myofibrils resulting in a relative increase of intervening sarcoplasm and a more dispersed arrangement of mitochondria, and necrolysis in cardiomyocytes
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• enlarged hearts, with all 4 chambers dilated
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• activity of the respiratory chain complex I and complex II are reduced in diseased hearts, indicating mitochondrial dysfunction
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• intracavitary thrombi are present in the atria and/or ventricles
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• myocardium shows many necrotic cardiomyocytes showing loss of myofibrils and nuclear destruction
• abnormal myofibril organization in cardiomyocytes is abnormal and myofibrils are thinner
• shape of the nuclei in myocytes is often altered, showing numerous deep indentations
• however, hypertrophy of cardiac myocytes is not seen
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• swelling of cardiomyocytes, absence of compaction of the myofibrils and loss of myofibrils resulting in a relative increase of intervening sarcoplasm and a more dispersed arrangement of mitochondria, and necrolysis in cardiomyocytes
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• cardiomyocyte myofibrils often show reduced sarcomeric length
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• M lines of cardiomyocyte myofibrils are poorly defined
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• Z lines are occasionally duplicated in cardiomyocyte myofibrils
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• enlarged hearts, with all 4 chambers dilated
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| dilated cardiomyopathy | DOID:12930 |
OMIM:PS115200 |
J:60351 | |
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 11/19/2024 MGI 6.24 |
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