All Phenotypes Ppp1r13l<tm1.1Xlu> MGI Mouse

premature death ( J:220162 )

• mice show a more rapid mortality beginning at 10 weeks of age, with a median survival of 40 weeks

• mice die of sudden cardiac death without signs of prior illness

cardiac muscle degeneration ( J:220162 )

• loss of cardiac muscle in the right ventricular wall

abnormal heart morphology ( J:220162 )

• accumulation of fat droplets is only seen at sites of fibrosis in all hearts

abnormal myocardium layer morphology ( J:220162 )

• E16.5 and E18.5 hearts show severe disruption of the myocardial structure, with a variegated pattern of muscle damage

• loss of myocardial integrity that includes the interventricular septum is seen by E18.5

abnormal myocardial fiber morphology ( J:220162 )

• interaction between the desmin-containing intermediate filaments is disrupted in desmosomes

abnormal intercalated disk morphology ( J:220162 )

• intercalated discs of cardiomyocytes show abnormal distribution of proteins

• 12 week old mice show irregularly shaped intercalated discs with widened gaps between the two-electron dense desmosomal plates

disorganized myocardium ( J:220162 )

• myocardial disarray and increased intercellular spaces between myocytes in myocardial regions

decreased heart right ventricle wall thickness ( J:220162 )

• E16.5 and E18.5 hearts show erosion and thinning of the right ventricular wall

dilated heart ventricle ( J:220162 )

• both the left and right ventricles are dilated in 12 week old mice

dilated heart right ventricle ( J:220162 )

• right ventricular dilation in E16.5 hearts

cardiac fibrosis ( J:220162 )

decreased cardiac output ( J:220162 )

• in 12 week old mice

decreased cardiac stroke volume ( J:220162 )

• in 12 week old mice

decreased ventricle muscle contractility ( J:220162 )

• increase in end-systolic volumes and decrease in ventricular ejection fraction at 12 weeks of age

• contraction of the ventricles is minimal as indicated by minimal change in the cross-sectional area between end-diastolic and end-systolic frames at midpapillary level

abnormal heart echocardiography feature ( J:220162 )

• increase in end-systolic volumes and decrease in stroke volume, ventricular ejection fraction, and cardiac output at 12 weeks of age

ventricular tachycardia ( J:220162 )

• young adult mice show frequent spontaneous runs of nonsustained ventricular tachycardia

ventricular premature beat ( J:220162 )

• young adult mice show frequent multifocal ventricular ectopy

ventricular cardiomyopathy ( J:220162 )

• heart exhibit features of human arrhythmogenic right ventricular cardiomyopathy

abnormal cardiac thrombosis ( J:220162 )

• blood clots are seen in E16.5 and E18.5 hearts