Mouse Genome Informatics
cx
    Dmdmdx/Dmdmdx
Utrntm1Ked/Utrntm1Ked

involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * C57BL/10ScSn * DBA
Key:
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• all succumb to premature death by 20 weeks of age

behavior/neurological
• slack posture by 4 to 6 weeks of age
• decrease in mobility and abnormal field behavior by 4 to 6 weeks of age
• waddling gait

growth/size/body

muscle
• muscle fibers are irregularly shaped and sized, many with centralized nuclei and some lymphocyte infiltration
• some fibers contain fat or appear to be replaced by adipose cells
• muscular dystrophy begins at 6 days of age and becomes severe by 10 weeks of age, earlier than in single Dmd mutants

respiratory system
• exhibit abnormal breathing by 4 to 6 weeks of age

skeleton

nervous system
• postsynaptic endplate regions exhibit a virtual absence of junctional folding at the postsynaptic membrane
• about 40% decrease in acetylcholine receptors at the synapse

limbs/digits/tail
• abnormally postured hindlimbs with joint contractures

Mouse Models of Human Disease
OMIM IDRef(s)
Muscular Dystrophy, Duchenne Type; DMD 310200 J:42388