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Phenotypes Associated with This Genotype
Genotype
MGI:3719609
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
• 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
• 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
• exhibit a small, but significant, increase in atrial size and atrial wall thickening
• 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
• 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
• misregistration of Z bands in cardiac myocytes

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
hypertrophic cardiomyopathy 2 DOID:0110308 OMIM:115195
J:48301


Contributing Projects:
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
03/19/2024
MGI 6.23
The Jackson Laboratory