Phenotypes Associated with This Genotype

Genotype
MGI:5906377

• Allelic Composition: Ptger4^tm1.1Matb/Ptger4^tm1.1Matb; Tg(Myh6-cre)2182Mds/0
• Genetic Background: involves: 129S6/SvEvTac * FVB/N

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

cardiovascular system

abnormal cardiac muscle tissue morphology ( J:158439 )

♂ • myofiber disarray

abnormal myocardial fiber morphology ( J:158439 )

♂ • length of myocytes from 31-33 week old males is increased, whereas width is normal

increased heart weight ( J:158439 )

♂ • heart weight to body weight ratio is increased in aged males

cardiac hypertrophy ( J:158439 )

♂ • males develop eccentric hypertrophy

decreased heart left ventricle posterior wall thickness ( J:158439 )

♂ • males show reduced posterior wall thickness at diastole

dilated heart left ventricle ( J:158439 )

• dilated left ventricle in males and a slight increase in left ventricular dimension in females

cardiac interstitial fibrosis ( J:158439 )

♂ • 39% increase in interstitial collagen fraction and greater collagen deposition in hearts

dilated cardiomyopathy ( J:158439 )

• older males show reduced ejection fraction and dilated left ventricle, indicating development of dilated cardiomyopathy

• females develop dilated cardiomyopathy at an older age and to a lesser extent than males

decreased cardiac muscle contractility ( J:158439 )

• ejection fraction is lower in 23-33 week old males; two groups are evident, those mice with ejection fraction greater than 70% and those with ejection fraction less than 70%

• 30-32 week old females show a modest, but significant, decline in ejection fraction

• a decline in ejection fraction begins around 16 weeks of age in males and after 24-28 weeks of age in females

• however, systolic blood pressure is normal in males and females at 28 weeks of age

abnormal heart echocardiography feature ( J:158439 )

• 28 week old male mice at with ejection fraction less than 70% exhibit increased left ventricular mass, increased left ventricular dimension at systole and left ventricular dimension at diastole, and reduced posterior wall thickness at diastole

• 31 week old females exhibit increased left ventricular dimension at systole

heart inflammation ( J:158439 )

♂ • patches of infiltration cells in hearts, however, the percentage of macrophages is not different from wild-type mice

immune system

heart inflammation ( J:158439 )

♂ • patches of infiltration cells in hearts, however, the percentage of macrophages is not different from wild-type mice

muscle

abnormal cardiac muscle tissue morphology ( J:158439 )

♂ • myofiber disarray

abnormal myocardial fiber morphology ( J:158439 )

♂ • length of myocytes from 31-33 week old males is increased, whereas width is normal

dilated cardiomyopathy ( J:158439 )

• older males show reduced ejection fraction and dilated left ventricle, indicating development of dilated cardiomyopathy

• females develop dilated cardiomyopathy at an older age and to a lesser extent than males

decreased cardiac muscle contractility ( J:158439 )

• ejection fraction is lower in 23-33 week old males; two groups are evident, those mice with ejection fraction greater than 70% and those with ejection fraction less than 70%

• 30-32 week old females show a modest, but significant, decline in ejection fraction

• a decline in ejection fraction begins around 16 weeks of age in males and after 24-28 weeks of age in females

• however, systolic blood pressure is normal in males and females at 28 weeks of age

growth/size/body

increased heart weight ( J:158439 )

♂ • heart weight to body weight ratio is increased in aged males

cardiac hypertrophy ( J:158439 )

♂ • males develop eccentric hypertrophy

cellular

cardiac interstitial fibrosis ( J:158439 )

♂ • 39% increase in interstitial collagen fraction and greater collagen deposition in hearts

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
dilated cardiomyopathy		DOID:12930	OMIM:PS115200	J:158439