Phenotypes Associated with This Genotype

Genotype
MGI:3056188

• Allelic Composition: Tg(Myh6-Ppp3ca)37Eno/0
• Genetic Background: involves: FVB

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

mortality/aging

premature death ( J:93176 )

• transgenic mice are highly susceptible to sudden death from heart failure

growth/size/body

increased heart weight ( J:109586 , J:213308 )

• at 3 weeks of age, transgenic mice display a ~2-fold increase in heart weight to tibia length ratio (J:109586)

cardiac hypertrophy ( J:93176 , J:94665 , J:213308 )

• the heart to body weight ratio is increased 2-3 fold as early as P18 with concentric hypertrophy seen, both ventricles and atria are affected (J:93176)

• cyclosporin A treatment prevented hypertrophy and fibrosis (J:93176)

• at 3 weeks of age, showed a 149% increase in heart weight/body weight ratio compared to wild-type (J:94665)

cardiovascular system

increased myocardial fiber size ( J:94665 )

• 34% increase in myocyte area compared to wild-type

myocardial fiber disarray ( J:93176 )

• the ventricular cardiomyocytes are disorganized, hypertrophic and display enlarged nuclei

increased heart weight ( J:109586 , J:213308 )

• at 3 weeks of age, transgenic mice display a ~2-fold increase in heart weight to tibia length ratio (J:109586)

cardiac hypertrophy ( J:93176 , J:94665 , J:213308 )

• the heart to body weight ratio is increased 2-3 fold as early as P18 with concentric hypertrophy seen, both ventricles and atria are affected (J:93176)

• cyclosporin A treatment prevented hypertrophy and fibrosis (J:93176)

• at 3 weeks of age, showed a 149% increase in heart weight/body weight ratio compared to wild-type (J:94665)

dilated heart left ventricle ( J:93176 )

• dilation of the ventricles is seen in older transgenic mice

dilated heart right ventricle ( J:93176 )

• dilation of the ventricles is seen in older transgenic mice

cardiac interstitial fibrosis ( J:93176 )

• extensive, primarily interstitial fibrosis is seen in the ventricular walls sometimes associated with myofiber degeneration

homeostasis/metabolism

pulmonary edema ( J:93176 )

• perivascular edema in the lungs is seen in transgenic mice that die suddenly

respiratory system

pulmonary edema ( J:93176 )

• perivascular edema in the lungs is seen in transgenic mice that die suddenly

muscle

increased myocardial fiber size ( J:94665 )

• 34% increase in myocyte area compared to wild-type

myocardial fiber disarray ( J:93176 )

• the ventricular cardiomyocytes are disorganized, hypertrophic and display enlarged nuclei

cellular

cardiac interstitial fibrosis ( J:93176 )

• extensive, primarily interstitial fibrosis is seen in the ventricular walls sometimes associated with myofiber degeneration

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
dilated cardiomyopathy		DOID:12930	OMIM:PS115200	J:93176