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Phenotypes Associated with This Genotype
Genotype
MGI:2176879
Allelic
Composition
Dmdmdx/Dmdmdx
Utrntm1Ked/Utrntm1Ked
Genetic
Background
involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * C57BL/10ScSn * DBA
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dmdmdx mutation (39 available); any Dmd mutation (156 available)
Utrntm1Ked mutation (12 available); any Utrn mutation (220 available)
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 ID Ref(s)
Muscular Dystrophy, Duchenne Type; DMD 310200 J:42388


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
04/26/2016
MGI 6.03
The Jackson Laboratory