Mouse Genome Informatics
tg1
    Tg(Myh6-Tnnt2)117Lnwd/Tg(Myh6-Tnnt2)117Lnwd
involves: C57BL/6
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
mortality/aging
• no mutants live for more than 24 hours, with the majority either stillborn or dying within the first few hours of life


Mouse Genome Informatics
tg2
    Tg(Myh6-Tnnt2)117Lnwd/0
involves: C57BL/6
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
cardiovascular system
• 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

muscle
• 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 IDRef(s)
Cardiomyopathy, Familial Hypertrophic, 2; CMH2 115195 J:48301