Phenotypes Associated with This Genotype

Genotype
MGI:5908182

• Allelic Composition: Tg(Myh6-Mtpn)4Ssen/0
• Genetic Background: involves: FVB

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

mortality/aging

premature death ( J:89823 )

• hypertrophy eventually leads to heart failure at around 36 weeks of age

growth/size/body

increased heart weight ( J:89823 )

• heart weight/body weight ratio is increased

cardiac hypertrophy ( J:89823 )

• mice develop progressive cardiac hypertrophy

cardiovascular system

increased myocardial fiber size ( J:89823 )

• large nuclei in myocytes, indicating myocyte hypertrophy

• cross-sectional area of myocytes in 4 week old and 9 month old mice progressively increases, indicating hypertrophy

• however, no myocyte atrophy is seen

myocardial fiber disarray ( J:89823 )

• multiple foci of classic myocyte disarray are seen in heart tissue

increased heart atrium size ( J:89823 )

• atria are enlarged by 4 weeks of age

thick interventricular septum ( J:89823 )

• both right and left ventricles are enlarged and display increased septal thickness

increased heart weight ( J:89823 )

• heart weight/body weight ratio is increased

cardiac hypertrophy ( J:89823 )

• mice develop progressive cardiac hypertrophy

increased heart ventricle size ( J:89823 )

• ventricles are enlarged by 4 weeks of age

thick ventricular wall ( J:89823 )

• the left ventricle heart walls are severely thickened at 18 weeks of age and show concentric hypertrophy

cardiac fibrosis ( J:89823 )

• fibrotic foci are seen in hearts

dystrophic cardiac calcinosis ( J:89823 )

• dystrophic calcification in hearts

decreased cardiac muscle contractility ( J:89823 )

• mice exhibit decreased fractional shortening at 9 months of age

abnormal heart echocardiography feature ( J:89823 )

• echocardiography shows increased left atrial diameter, intraventricular septal wall thickness and left ventricular posterior wall thickness, with no change in fractional shorting at 4 weeks of age

• echocardiography shows hypertrophied septum, enlarged left ventricular diastolic and systolic dimensions, lower fractional shortening, a trend toward left atrial diameter enlargement, and increased left ventricular posterior wall thickness at 9 months of age

congestive heart failure ( J:89823 )

• mice develop symptoms of heart failure, including lethargy, edema, pulmonary effusion and lack of alertness

behavior/neurological

lethargy ( J:89823 )

• at end stage of disease

homeostasis/metabolism

edema ( J:89823 )

• at end stage of disease

pleural effusion ( J:89823 )

• large amount of pleural effusion is seen in 9 month old mice

muscle

increased myocardial fiber size ( J:89823 )

• large nuclei in myocytes, indicating myocyte hypertrophy

• cross-sectional area of myocytes in 4 week old and 9 month old mice progressively increases, indicating hypertrophy

• however, no myocyte atrophy is seen

myocardial fiber disarray ( J:89823 )

• multiple foci of classic myocyte disarray are seen in heart tissue

decreased cardiac muscle contractility ( J:89823 )

• mice exhibit decreased fractional shortening at 9 months of age

respiratory system

pleural effusion ( J:89823 )

• large amount of pleural effusion is seen in 9 month old mice

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
congestive heart failure		DOID:6000		J:89823
hypertrophic cardiomyopathy		DOID:11984		J:89823