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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Tg(Myh6-Tnnt2)117Lnwd
transgene insertion 117, Leslie A Leinwand
MGI:3692598
Summary 2 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
tg1
Tg(Myh6-Tnnt2)117Lnwd/Tg(Myh6-Tnnt2)117Lnwd involves: C57BL/6 MGI:3719607
tg2
Tg(Myh6-Tnnt2)117Lnwd/0 involves: C57BL/6 MGI:3719609


Genotype
MGI:3719607
tg1
Allelic
Composition
Tg(Myh6-Tnnt2)117Lnwd/Tg(Myh6-Tnnt2)117Lnwd
Genetic
Background
involves: C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
No mouse lines available in IMSR.
See publication links below for author information.
phenotype observed in females
phenotype observed in males
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




Genotype
MGI:3719609
tg2
Allelic
Composition
Tg(Myh6-Tnnt2)117Lnwd/0
Genetic
Background
involves: C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
No mouse lines available in IMSR.
See publication links below for author information.
phenotype observed in females
phenotype observed in males
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 (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)
• exhibit about 8-10% fewer myocytes than controls
• neonatal and adult hearts show myocellular degeneration (J:48301)
• 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 (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)
• exhibit about 8-10% fewer myocytes than controls
• neonatal and adult hearts show myocellular degeneration (J:48301)
• 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
• misregistration of Z bands in cardiac myocytes

Mouse Models of Human Disease
OMIM ID Ref(s)
Cardiomyopathy, Familial Hypertrophic, 2; CMH2 115195 J:48301





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last database update
04/26/2016
MGI 6.03
The Jackson Laboratory