Phenotypes Associated with This Genotype

Genotype
MGI:4429501

• Allelic Composition: Tg(Myh6-rtTA)8585Jam/0; Tg(tetO-Ppargc1a)1Dpk/0
• Genetic Background: involves: FVB/N * FVB/NTac

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

cardiovascular system

abnormal myocardial fiber morphology ( J:96614 )

• following doxycycline treatment of neonates, myofibril mitochondria density is increased 3.5-fold compared with wild-type myofibrils due to increased biogenesis

• doxycycline-treated adult mice exhibit an increased in mitochondrial biogenesis in myofibrils compared with wild-type mice but not to the extent observed in doxycycline treated neonates

• however, removal of doxycycline restores normal mitochondria

cardiac hypertrophy ( J:96614 )

• following doxycycline treatment, adult mice exhibit eccentric hypertrophy unlike in wild-type mice

abnormal heart left ventricle morphology ( J:96614 )

• 2 week after doxycycline treatment, adult mice exhibit increased end-diastolic and end-systolic left ventricle diameter and a mild increase in left ventricular wall thickness compared with wild-type mice

dilated heart left ventricle ( J:96614 )

• following doxycycline treatment of adult mice

dilated heart right ventricle ( J:96614 )

• following doxycycline treatment of adult mice

decreased cardiac muscle contractility ( J:96614 )

• following doxycycline treatment, adult mice exhibit decreased fractional shortening compared with wild-type mice

• however, removal of doxycycline restores cardiac muscle contractility

decreased heart rate ( J:96614 )

• following doxycycline treatment of adult mice

increased heart rate ( J:96614 )

• following removal of doxycycline

cardiomyopathy ( J:96614 )

• following induction of expression with doxycycline, adult mice exhibit reversible cardiomyopathy unlike wild-type mice

• however, no increase in cardiac cell apoptosis is observed

cellular

abnormal mitochondrial morphology ( J:96614 )

• following doxycycline treatment, adult mice exhibit an increase in mitochondrial number along with mitochondrial ultrastructural abnormalities unlike in wild-type mice

increased mitochondrial fission ( J:96614 )

• following doxycycline treatment of neonates, myofibril mitochondria density is increased 3.5-fold compared with wild-type myofibrils due to increased biogenesis

• doxycycline-treated adult mice exhibit an increased in mitochondrial biogenesis in myofibrils compared with wild-type mice but not to the extent observed in doxycycline treated neonates

• however, removal of doxycycline restores normal mitochondria

muscle

abnormal myocardial fiber morphology ( J:96614 )

• following doxycycline treatment of neonates, myofibril mitochondria density is increased 3.5-fold compared with wild-type myofibrils due to increased biogenesis

• doxycycline-treated adult mice exhibit an increased in mitochondrial biogenesis in myofibrils compared with wild-type mice but not to the extent observed in doxycycline treated neonates

• however, removal of doxycycline restores normal mitochondria

decreased cardiac muscle contractility ( J:96614 )

• following doxycycline treatment, adult mice exhibit decreased fractional shortening compared with wild-type mice

• however, removal of doxycycline restores cardiac muscle contractility

cardiomyopathy ( J:96614 )

• following induction of expression with doxycycline, adult mice exhibit reversible cardiomyopathy unlike wild-type mice

• however, no increase in cardiac cell apoptosis is observed

growth/size/body

cardiac hypertrophy ( J:96614 )

• following doxycycline treatment, adult mice exhibit eccentric hypertrophy unlike in wild-type mice

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
cardiomyopathy		DOID:0050700		J:96614
congestive heart failure		DOID:6000		J:96614