Phenotypes Associated with This Genotype

Genotype
MGI:5467575

• Allelic Composition: Cenpf^tm1Dbdr/Cenpf^tm1Dbdr; Tg(Tnnt2-cre)5Blh/0
• Genetic Background: involves: C57BL/6 * DBA/2 * ICR

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

cardiovascular system

cardiovascular system phenotype ( J:185133 )

N • development of heart structure is grossly normal

abnormal heart morphology ( J:185133 )

• modest reduction in coronary vasculature

abnormal myocardial fiber morphology ( J:185133 )

• costamere structure is impaired

• decreased number of myocytes in adults

abnormal intercalated disk morphology ( J:185133 )

• intercalated disc number is reduced 3.5 fold in mature ventricles

abnormal heart atrium morphology ( J:185133 )

• thinner walls

small heart ( J:185133 )

• smaller ventricles

myocardial trabeculae hypoplasia ( J:185133 )

• trabeculae are thinner and blunted

decreased heart weight ( J:185133 )

• weight at 4 days of age is 8.2mg compared to 10.5mg for controls

abnormal heart ventricle morphology ( J:185133 )

• smaller ventricles

dilated heart left ventricle ( J:185133 )

• internal dimension somewhat increased

thin ventricular wall ( J:185133 )

abnormal epicardium morphology ( J:185133 )

• modest reduction in epicardial thickness

cardiac fibrosis ( J:185133 )

• cardiac fibrosis in adults

abnormal cardiovascular system physiology ( J:185133 )

• decreasing ventricular function with age

dilated cardiomyopathy ( J:185133 )

• mice develop progressive dilated cardiomyopathy

decreased fetal cardiomyocyte proliferation ( J:185133 )

• three fold decrease in prenatal myocardiocyte proliferation

abnormal heart electrocardiography waveform feature ( J:185133 )

• at 3 months

prolonged PR interval ( J:185133 )

prolonged P wave ( J:185133 )

• P-P intervals greater than 200 ms

• slowing sinus rhythms

abnormal myocardial fiber physiology ( J:185133 )

• lower myocyte mitotic rates on days 1-4 after birth

growth/size/body

decreased body size ( J:185133 )

• normal to slightly smaller body size

mortality/aging

premature death ( J:185133 )

• 20% mortality in the 12 to 21 month age period

muscle

abnormal myocardial fiber morphology ( J:185133 )

• costamere structure is impaired

• decreased number of myocytes in adults

abnormal intercalated disk morphology ( J:185133 )

• intercalated disc number is reduced 3.5 fold in mature ventricles

myocardial trabeculae hypoplasia ( J:185133 )

• trabeculae are thinner and blunted

dilated cardiomyopathy ( J:185133 )

• mice develop progressive dilated cardiomyopathy

decreased fetal cardiomyocyte proliferation ( J:185133 )

• three fold decrease in prenatal myocardiocyte proliferation

cellular

decreased fetal cardiomyocyte proliferation ( J:185133 )

• three fold decrease in prenatal myocardiocyte proliferation

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
dilated cardiomyopathy		DOID:12930	OMIM:PS115200	J:185133