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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 (135 available)
Utrntm1Ked mutation (12 available); any Utrn mutation (154 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• all succumb to premature death by 20 weeks of age (J:42388)
• all succumb to premature death by 20 weeks of age (J:42388)

behavior/neurological
• slack posture by 4 to 6 weeks of age (J:42388)
• slack posture by 4 to 6 weeks of age (J:42388)
• decrease in mobility and abnormal field behavior by 4 to 6 weeks of age (J:42388)
• decrease in mobility and abnormal field behavior by 4 to 6 weeks of age (J:42388)
• waddling gait (J:42388)
• waddling gait (J:42388)

growth/size/body
(J:42388)
(J:42388)

muscle
• muscle fibers are irregularly shaped and sized, many with centralized nuclei and some lymphocyte infiltration (J:42388)
• some fibers contain fat or appear to be replaced by adipose cells (J:42388)
• muscle fibers are irregularly shaped and sized, many with centralized nuclei and some lymphocyte infiltration (J:42388)
• some fibers contain fat or appear to be replaced by adipose cells (J:42388)
(J:42388)
(J:42388)
• muscular dystrophy begins at 6 days of age and becomes severe by 10 weeks of age, earlier than in single Dmd mutants (J:42388)
• muscular dystrophy begins at 6 days of age and becomes severe by 10 weeks of age, earlier than in single Dmd mutants (J:42388)
(J:42388)
(J:42388)

respiratory system
• exhibit abnormal breathing by 4 to 6 weeks of age (J:42388)
• exhibit abnormal breathing by 4 to 6 weeks of age (J:42388)

skeleton
(J:42388)
(J:42388)

nervous system
• postsynaptic endplate regions exhibit a virtual absence of junctional folding at the postsynaptic membrane (J:42388)
• about 40% decrease in acetylcholine receptors at the synapse (J:42388)
• postsynaptic endplate regions exhibit a virtual absence of junctional folding at the postsynaptic membrane (J:42388)
• about 40% decrease in acetylcholine receptors at the synapse (J:42388)

limbs/digits/tail
• abnormally postured hindlimbs with joint contractures (J:42388)
• abnormally postured hindlimbs with joint contractures (J:42388)

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
02/02/2016
MGI 6.02
The Jackson Laboratory