Analysis Tools
mortality/aging
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• no mutants live for more than 24 hours, with the majority either stillborn or dying within the first few hours of life
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Allele Symbol Allele Name Allele ID |
Tg(Myh6-Tnnt2)117Lnwd transgene insertion 117, Leslie A Leinwand MGI:3692598 |
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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 mutants live for more than 24 hours, with the majority either stillborn or dying within the first few hours of life
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• neonatal and adult hearts show myocellular disorganization and degeneration
(J:48301)
• cardiac myocytes show areas of focal myofibrillar lysis, misregistration of Z bands, and distinct regions of myofibrillar disarray
(J:56911)
• however, cardiac fibrosis is rare, mice do not express markers of hypertrophy, mitochondrial structure is normal in myocytes, and no lipid deposition in myocytes is seen
(J:56911)
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• exhibit about 8-10% fewer myocytes than controls
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• mean length and midpoint width of quiescent ventricular cardiac myocytes are 15 and 13% smaller, respectively, than in controls and mean myocyte area is decreased by 17%
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• neonatal and adult hearts show myocellular degeneration
(J:48301)
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• neonatal and adult hearts show myocellular disorganization
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• exhibit a small, but significant, increase in atrial size and atrial wall thickening
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• heart weight to body weight ratio shows an 18-27% decrease in heart mass at 4-5 months of age, restricted to the left ventricle
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• exhibit a 17% decrease in left ventricular mass
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• altering the physiologic load on the heart shows some impairment in contractility
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• at a baseline workload, hearts exhibit a 25% decrease in the maximal rate of relaxation
• hearts show a moderately good correlation of +dP/dT to increased work, however the slope of the Starling curve is severely decreased during relaxation suggesting that hearts cannot respond to an increased work load by shortening their relaxation time
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• isolated adult cardiac myocytes and hypersensitive to calcium; addition of calcium led to immediate cellular hypercontracture and death
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• neonatal and adult hearts show myocellular disorganization and degeneration
(J:48301)
• cardiac myocytes show areas of focal myofibrillar lysis, misregistration of Z bands, and distinct regions of myofibrillar disarray
(J:56911)
• however, cardiac fibrosis is rare, mice do not express markers of hypertrophy, mitochondrial structure is normal in myocytes, and no lipid deposition in myocytes is seen
(J:56911)
|
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• exhibit about 8-10% fewer myocytes than controls
|
|
• mean length and midpoint width of quiescent ventricular cardiac myocytes are 15 and 13% smaller, respectively, than in controls and mean myocyte area is decreased by 17%
|
|
• neonatal and adult hearts show myocellular degeneration
(J:48301)
|
|
• neonatal and adult hearts show myocellular disorganization
|
|
• altering the physiologic load on the heart shows some impairment in contractility
|
|
• at a baseline workload, hearts exhibit a 25% decrease in the maximal rate of relaxation
• hearts show a moderately good correlation of +dP/dT to increased work, however the slope of the Starling curve is severely decreased during relaxation suggesting that hearts cannot respond to an increased work load by shortening their relaxation time
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• misregistration of Z bands in cardiac myocytes
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| hypertrophic cardiomyopathy 2 | DOID:0110308 |
OMIM:115195 |
J:48301 | |
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 05/07/2024 MGI 6.23 |
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