mortality/aging
|
• no mutants live for more than 24 hours, with the majority either stillborn or dying within the first few hours of life
|
|
tg1
|
Tg(Myh6-Tnnt2)117Lnwd/Tg(Myh6-Tnnt2)117Lnwd
involves: C57BL/6 |
|
|||||||||||||||||
|
• no mutants live for more than 24 hours, with the majority either stillborn or dying within the first few hours of life
|
|
tg2
|
Tg(Myh6-Tnnt2)117Lnwd/0
involves: C57BL/6 |
|
|||||||||||||||||
|
• exhibit a small, but significant, increase in atrial size and atrial wall thickening
|
|
• neonatal and adult hearts show myocellular disorganization and degeneration
|
|
• exhibit about 8-10% fewer myocytes than controls
|
|
• neonatal and adult hearts show myocellular degeneration
|
|
• 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 disorganization
|
|
• 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
|
|
• exhibit a 17% decrease in left ventricular mass
|
|
• 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
|
|
• isolated adult cardiac myocytes and hypersensitive to calcium; addition of calcium led to immediate cellular hypercontracture and death
|
|
• neonatal and adult hearts show myocellular disorganization and degeneration
|
|
• exhibit about 8-10% fewer myocytes than controls
|
|
• neonatal and adult hearts show myocellular degeneration
|
|
• 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%
|
|
• 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
|
Mouse Models of Human Disease |
OMIM ID | Ref(s) | |
| Cardiomyopathy, Familial Hypertrophic, 2; CMH2 | 115195 | J:48301 | |