Phenotypes Associated with This Genotype

Genotype
MGI:6188933

• Allelic Composition: Tg(Myh6-Pkp2*/mRuby)4Rbrug/0
• Genetic Background: involves: C57BL/6 * DBA/2

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

cardiovascular system

abnormal heart right ventricle morphology ( J:237859 )

• mice exhibit progressive structural right ventricle remodeling, with 12 month old mice showing right ventricle outflow tract and right ventricle diameter and area dilation

abnormal heart right ventricle outflow tract morphology ( J:237859 )

• 12 month old, but not 7 month old, mice show right ventricle outflow tract dilation

• long-term physical activity promotes right ventricle outflow tract dilation

dilated heart right ventricle ( J:237859 )

• mice show progressive right ventricle dilation from 7 to 12 months of age, indicating progressive structural remodeling

• endurance training and aging trigger right ventricular enlargement

• however, no changes in left ventricle structure and function are seen

abnormal heart echocardiography feature ( J:237859 )

• aged mice exhibit right ventricle dysfunction, with an increase in right ventricle end-diastolic area parasternal axis view and a decrease in tricuspid annular plane systolic excursion at 7 and 12 months of age

ventricular tachycardia ( J:237859 )

• electrical programmed stimulation induces ventricular tachycardia in 54% of sedentary mice compared to 31% of wild-type mice indicating increased susceptibility to ventricular arrhythmias

• however, no differences are seen in endurance training-dependent arrhythmia induction

abnormal impulse conducting system conduction ( J:237859 )

• atrio-ventricular conduction assessment shows a prolongation of the Atrial-His conduction (A-H interval) in 7 month old mice

prolonged PR interval ( J:237859 )

• in 3, 7, and 12 month old mice

• 7 month old exercised trained mice show a greater PR interval prolongation than controls

cardiomyopathy ( J:237859 )

• aged mice develop mild arrhythmogenic cardiomyopathy

• however, mice do not exhibit cardiac hypertrophy and no evidence of collagen deposition or fatty tissue replacement in the heart

homeostasis/metabolism

abnormal response to exercise ( J:237859 )

• long-term physical activity promotes right ventricle outflow tract dilation and right ventricular enlargement

• however, exercise results in similar cardiac hypertrophy and QRS prolongation as in wild-type mice

muscle

cardiomyopathy ( J:237859 )

• aged mice develop mild arrhythmogenic cardiomyopathy

• however, mice do not exhibit cardiac hypertrophy and no evidence of collagen deposition or fatty tissue replacement in the heart

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
arrhythmogenic right ventricular dysplasia 9		DOID:0110077	OMIM:609040	J:237859