Mouse Genome Informatics
cx
    Dmdmdx/?
Myod1tm1Jae/Myod1tm1Jae

involves: 129S4/SvJae * C57BL/10ScSn
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       

Dmdmdx/? Myod1tm1Jae/Myod1tm1Jae mice develop cardiomyopathy

mortality/aging
• premature death around 12 months of age

cardiovascular system
• ventricles contain regions in which individual cardiac myocytes are enlarged; these hypertrophic myocytes are more common in the left ventricle than the right ventricle
• cardiac myocyte hypertrophy precedes necrosis
• fibrotic areas are composed of necrotic myocytes
• hearts show an increase in ventricular diameter without a change in the thickness of the ventricular wall
• more than 50% of mutants show evidence of fibrosis at 10 months of age, while more than 80% display extensive fibrosis by 12 months of age
• fibrosis is seen in the left ventricle and only rarely in the right ventricle and are confined primarily to the epicardial region of the left ventricle
• fibrotic areas are composed of necrotic myocytes associated with interstitial fibrosis
• progressive development of dilated cardiomyopathy that is evident by 5 months of age

muscle
• ventricles contain regions in which individual cardiac myocytes are enlarged; these hypertrophic myocytes are more common in the left ventricle than the right ventricle
• cardiac myocyte hypertrophy precedes necrosis
• progressive development of dilated cardiomyopathy that is evident by 5 months of age

Mouse Models of Human Disease
OMIM IDRef(s)
Muscular Dystrophy, Duchenne Type; DMD 310200 J:52248