mortality/aging
• mutants show a large increase in mortality occurring between the 8th and 15th week after birth, with a half-life of 12 weeks
|
cardiovascular system
• cellular organization in hearts is altered after 11 weeks of age, with evidence of myocyte loss and interstitial cell infiltratio
• the number of apoptotic cells in the hear is increased at 11 weeks of age
|
• at 11 weeks of age, but not 6 weeks, heart left ventricle is severely dilated
|
• at 11 weeks of age, the left ventricle free wall is thinner during systole than in controls, suggesting the development of a hypokinetic dilated cardiomyopathy
|
• mutants exhibit systolic dysfunction at 11 weeks of age, indicated by reduced left ventricle fractional shortening
|
• ECG intervals during the last ECG recording before death show differences in PQ, QRS, QT, and QTc but not of RR
• mice that survive exhibit ECG parameters with normal values, with a moderate prolongation of PQ and QRS intervals
|
• mice that die during the 15th week, show complete atrioventricular block, with dissociation between the sinus and idioventricular escape rhythms
|
• at 11 weeks of age
|
• at 11 weeks of age, mutants show a moderately prolonged QRS interval
|
• at 11 weeks of age, ECGs show an abnormal repolarization with disappearance of the early positive phase of the T wave commonly seen in controls
• at 11 weeks of age, the T-wave duration is prolonged
• however, the QT interval is not prolonged
|
• hearts are infiltrated by a large number of interstitial cells; about 50% of infiltrating cells express panleukocyte CD45 marker
|
myocarditis
(
J:194810
)
immune system
• hearts are infiltrated by a large number of interstitial cells; about 50% of infiltrating cells express panleukocyte CD45 marker
|
myocarditis
(
J:194810
)
muscle
• at 11 weeks of age, the left ventricle free wall is thinner during systole than in controls, suggesting the development of a hypokinetic dilated cardiomyopathy
|
• mutants exhibit systolic dysfunction at 11 weeks of age, indicated by reduced left ventricle fractional shortening
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
dilated cardiomyopathy | DOID:12930 |
OMIM:PS115200 |
J:194810 |