Phenotypes Associated with This Genotype

Genotype
MGI:4367214

• Allelic Composition: Nos1^tm1Plh/Nos1^tm1Plh; Nos3^tm1Plh/Nos3^tm1Plh
• Genetic Background: involves: 129S4/SvJae * C57BL/6

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

mortality/aging

decreased survivor rate ( J:129064 )

• survival at 20 months is reduced compared to Nos3 or Nos1 single mutant mice

premature death ( J:129064 )

• survival at 20 months is reduced compared to Nos3 or Nos1 single mutant mice

• the relative risk of death by 20 months of age is increased 7.3 fold compared to Nos3 single mutants and 3.0 fold compared to Nos1 single mutants

• mortality is increased in males compared to females

cardiovascular system

heart left ventricle hypertrophy ( J:75645 )

• develop age related concentric remodeling with marked increase in relative wall thickness

• increase in wall thickness and decrease in systolic and diastolic dimensions with age

decreased cardiac muscle contractility ( J:75645 )

• attenuation of the beta-adrenergic inotropic responses indicating a decrease in myocardial contractile reserve

increased cardiac muscle contractility ( J:75645 )

• increase in basal contractility

• hypertrophy is associated with hypercontractility

abnormal cardiac muscle relaxation ( J:75645 )

• hypertrophy is associated with increased diastolic stiffness

increased heart rate ( J:75645 )

• relative to wild-type mice and Nos3 single mutants

increased systemic arterial systolic blood pressure ( J:75645 )

• identical to that in Nos3 single mutants

muscle

heart left ventricle hypertrophy ( J:75645 )

• develop age related concentric remodeling with marked increase in relative wall thickness

• increase in wall thickness and decrease in systolic and diastolic dimensions with age

decreased cardiac muscle contractility ( J:75645 )

• attenuation of the beta-adrenergic inotropic responses indicating a decrease in myocardial contractile reserve

increased cardiac muscle contractility ( J:75645 )

• increase in basal contractility

• hypertrophy is associated with hypercontractility

abnormal cardiac muscle relaxation ( J:75645 )

• hypertrophy is associated with increased diastolic stiffness

growth/size/body

heart left ventricle hypertrophy ( J:75645 )

• develop age related concentric remodeling with marked increase in relative wall thickness

• increase in wall thickness and decrease in systolic and diastolic dimensions with age

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
hypertrophic cardiomyopathy		DOID:11984		J:129064