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Phenotypes Associated with This Genotype
Genotype
MGI:3784305
Allelic
Composition
Dmdmdx/?
Myod1tm1Jae/Myod1tm1Jae
Genetic
Background
involves: 129S4/SvJae * C57BL/10ScSn
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dmdmdx mutation (39 available); any Dmd mutation (156 available)
Myod1tm1Jae mutation (2 available); any Myod1 mutation (7 available)
phenotype observed in females
phenotype observed in males
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 ID Ref(s)
Muscular Dystrophy, Duchenne Type; DMD 310200 J:52248


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
05/17/2016
MGI 6.03
The Jackson Laboratory