Phenotypes Associated with This Genotype

Genotype
MGI:5907012

• Allelic Composition: Tg(Myh6-cre)TG9Pjay/0
• Genetic Background: involves: FVB/N

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

mortality/aging

premature death ( J:119070 , J:198043 )

• swim exercise training before cardiac function abnormalities improves lifespan, with mean age increased by about 20% and 16% in males and females, respectively (J:119070)

• maximum life span of females and males is 11 and 13 weeks, respectively (J:198043)

• treatment with captopril, an angiotensin converting enzyme inhibitor, prolongs survival by 1 week in females and by 2 weeks in males (J:198043)

• treatment with metoprolol, a beta1-adrenergic receptor blocker, has a small effect on increased survival (J:198043)

cardiovascular system

pulmonary vascular congestion ( J:198043 )

• severe pulmonary congestion in end-stage heart failure as indicated by increased lung-to-body weight ratios

dilated heart ventricle ( J:198043 )

• ventricular chamber dilation with a thin myocardial wall

dilated heart left ventricle ( J:198043 )

• left ventricular dilation is present by 57 days of age and becomes more pronounced over the next 2 weeks

dilated cardiomyopathy ( J:119070 , J:198043 )

• mice develop severe dilated cardiomyopathy showing progressive dilation of the left ventricular end-diastolic diameter, worsening systolic function, and a trend towards thinning of the left ventricular wall (J:198043)

• however, no myocardial fibrosis is present (J:198043)

decreased cardiac muscle contractility ( J:198043 )

• systolic dysfunction is present by 57 days of age and becomes more pronounced over the next 2 weeks, with decreased fractional shortening and progressive dilation of the left ventricular end-diastolic diameter

congestive heart failure ( J:198043 )

• mice become fatigued and sedentary, and show labored breathing before death due to heart failure

cellular

increased cardiomyocyte apoptosis ( J:198043 )

• 2-fold increase in incidence of cardiac myocyte apoptosis at 7 weeks of age

homeostasis/metabolism

abnormal atrial thrombosis ( J:198043 )

• organized thrombi in the left atrium

edema ( J:198043 )

• peripheral edema

ascites ( J:198043 )

• abdominal ascites

pleural effusion ( J:198043 )

muscle

dilated cardiomyopathy ( J:119070 , J:198043 )

• mice develop severe dilated cardiomyopathy showing progressive dilation of the left ventricular end-diastolic diameter, worsening systolic function, and a trend towards thinning of the left ventricular wall (J:198043)

• however, no myocardial fibrosis is present (J:198043)

decreased cardiac muscle contractility ( J:198043 )

• systolic dysfunction is present by 57 days of age and becomes more pronounced over the next 2 weeks, with decreased fractional shortening and progressive dilation of the left ventricular end-diastolic diameter

increased cardiomyocyte apoptosis ( J:198043 )

• 2-fold increase in incidence of cardiac myocyte apoptosis at 7 weeks of age

respiratory system

pulmonary vascular congestion ( J:198043 )

• severe pulmonary congestion in end-stage heart failure as indicated by increased lung-to-body weight ratios

pleural effusion ( J:198043 )

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
congestive heart failure		DOID:6000		J:198043
dilated cardiomyopathy		DOID:12930	OMIM:PS115200	J:119070