Phenotypes Associated with This Genotype

Genotype
MGI:3652395

• Allelic Composition: Tg(Myh6-Ppara)404-3Dpk/0
• Genetic Background: involves: C57BL/6 * CBA/J

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

cardiovascular system

abnormal myocardial fiber morphology ( J:110580 )

• exhibit neutral lipid droplet accumulation in cardiac myocytes after a short-term fast

• myocardial triacylglycerol levels are higher in fasted mutants than in controls, especially the long-chain fatty acid-containing triacylglycerol

• myocyte hypertrophy is seen in 3-month old mutants

cardiac hypertrophy ( J:110580 )

• exhibit an increase in biventricular weight/body weight ratio

abnormal cardiovascular system physiology ( J:110580 )

• myocardial uptake of ¹¹C-palmitate is increased, indicating enhanced myocardial uptake of fatty acid

decreased cardiac muscle cell glucose uptake ( J:110580 )

• myocardial uptake of ¹⁸FDG is lower indicating decreased glucose uptake

decreased heart ventricle muscle contractility ( J:110580 )

• the posterior wall thickness is increased, LV chamber dilatation, as determined by systolic and diastolic internal diameters, is increased, and fractional shortening is reduced

cardiomyopathy ( J:81834 , J:110580 )

• insulin deficient mutants and mutants fed a diet enriched in triglyceride containing long-chain fatty acid develop more severe cardiomyopathy than untreated controls (J:81834)

• exhibit signatures of diabetic cardiomyopathy including ventricular hypertrophy, activation of gene markers of pathologic hypertrophic growth, and alteration systolic ventricular function (J:110580)

homeostasis/metabolism

abnormal lipid level ( J:110580 )

• exhibit neutral lipid droplet accumulation in cardiac myocytes after a short-term fast

increased fatty acids level ( J:110580 )

• myocardial triacylglycerol levels are higher in fasted mutants than in controls, specifically long-chain fatty acid-containing triacylglycerol

muscle

abnormal myocardial fiber morphology ( J:110580 )

• exhibit neutral lipid droplet accumulation in cardiac myocytes after a short-term fast

• myocardial triacylglycerol levels are higher in fasted mutants than in controls, especially the long-chain fatty acid-containing triacylglycerol

• myocyte hypertrophy is seen in 3-month old mutants

decreased cardiac muscle cell glucose uptake ( J:110580 )

• myocardial uptake of ¹⁸FDG is lower indicating decreased glucose uptake

decreased heart ventricle muscle contractility ( J:110580 )

• the posterior wall thickness is increased, LV chamber dilatation, as determined by systolic and diastolic internal diameters, is increased, and fractional shortening is reduced

cardiomyopathy ( J:81834 , J:110580 )

• insulin deficient mutants and mutants fed a diet enriched in triglyceride containing long-chain fatty acid develop more severe cardiomyopathy than untreated controls (J:81834)

• exhibit signatures of diabetic cardiomyopathy including ventricular hypertrophy, activation of gene markers of pathologic hypertrophic growth, and alteration systolic ventricular function (J:110580)

cellular

decreased cardiac muscle cell glucose uptake ( J:110580 )

• myocardial uptake of ¹⁸FDG is lower indicating decreased glucose uptake

growth/size/body

cardiac hypertrophy ( J:110580 )

• exhibit an increase in biventricular weight/body weight ratio

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
diabetes mellitus		DOID:9351		J:110580