Phenotypes for Dmd Terc MGI:5529018 MGI Mouse

abnormal myocardial fiber morphology ( J:200365 )

• cardiomyocyte nuclear area is moderately reduced in hearts of second generation (G2) 32 week old males as compared to controls

• cardiomyocyte diameter is smaller in hearts of 32 week old G2 males

• hearts from G2 males exhibit a loss of thick and thin myofilaments

increased heart left ventricle size ( J:200365 )

• left ventricle enlargement is observed by 32 weeks in hearts of G2 males as compared to controls

dilated heart left ventricle ( J:200365 )

• increased chamber size is observed in 32 week old G2 males as compared to controls

• left ventricular transverse area is increased in diastole and systole in G2 males

cardiac fibrosis ( J:200365 )

• ventricular fibrosis is observed by 32 weeks in hearts of G2 males

dilated cardiomyopathy ( J:200365 )

• reduced cardiac function is observed in older G1 males (80 weeks), but is not observed at 32 weeks

• cardiac dysfunction is observed at 8 weeks in third generation males

• cardiac dysfunction can be induced by angiotension II infusion in younger second generation males

decreased heart ventricle muscle contractility ( J:200365 )

• 32 week old G2 males exhibit reduced ventricular contractility as assessed by a reduction in fractional shortening as compared to controls

prolonged QRS complex duration ( J:200365 )

• wide QRS interval is observed in both G1 and G2 males as compared to controls

ventricular cardiomyopathy ( J:200365 )

decreased telomere length ( J:200365 )

• 50% reduction in telomere length in G2 cardiomyocytes as compared to controls

• however, telomere lengths are similar to controls in vascular smooth muscle cells

disorganized mitochondrial cristae ( J:200365 )

• lack of well-defined cristae observed in cardiac muscle of second generation mice

abnormal mitochondrial shape ( J:200365 )

• extensive mitochondrial fragmentation observed in cardiac muscle of second generation mice

decreased mitochondrial size ( J:200365 )

• mitochondria size is decreaseed in cardiac muscle of second generation mice

increased mitochondrial number ( J:200365 )

• moderately increased number of mitochondria is observed in cardiac muscle of second generation mice

oxidative stress ( J:200365 )

• an increased number of 8-OHdg-positive nuclei, a marker of oxidative damage, is observed in G2 hearts as compared to controls

premature death ( J:200365 )

• death occurs as early as 30 weeks in first generation males, T50 is 120 weeks

• death occurs as early as 19 weeks in second generation males, T50 is 80 weeks