Mouse Genome Informatics
cx
    Barx2tm1Rsd/Barx2tm1Rsd
Dmdmdx/Dmdmdx

involves: 129 * C57BL/6 * C57BL/10ScSn
Key:
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• mutants are under-represented at weaning age

growth/size/body
• mutants are about 30% smaller than single Dmd mutants at 4 weeks of age; organs are reduced proportionately

behavior/neurological
• mutants become progressively less ambulatory
• mutants become progressively less ambulatory with a waddling gait
• gait abnormalities appear earlier and are more severe than in single Barx2 mutants

muscle
• organization of tibialis anterior muscle is extremely aberrant with greater variability in myofiber size and fewer central nuclei releative to muscle from single Dmd mutants
• soleus muscles show greater variability in myofiber size and shape compared to muscle from single Dmd mutants
• tibialis anterior muscle weighs on average 50% less than in single Dmd mutants, indicating muscle wasting
• at 12 months of age, diaphragms show a greater instance of focal myofiber necrosis and necrotic myofibers undergoing myophagocytosis and more fibrosis compared to single Dmd mutants
• diaphragm muscles at 6 months of age are much thinner than in single Dmd mutants and show more frequent rounded opaque fibers often with clear vacuoles, indicating hypercontracted atrophic fibers
• soleus muscles show more endomysial and perimysial fibrosis compared to muscle from single Dmd mutants
• diaphragms show more fibrosis than single Dmd mutants
• tibialis anterior muscle shows fewer central nuclei relative to muscle from single Dmd mutants, indicating reduction in repair of muscle damage
• mutants become progressively weak

skeleton
• mutants develop spine deformation resembling kyphosis by 6 months of age
• spine deformation appears earlier and is more severe than in single Barx2 mutants

Mouse Models of Human Disease
OMIM IDRef(s)
Muscular Dystrophy, Duchenne Type; DMD 310200 J:187799