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Phenotypes Associated with This Genotype
Genotype
MGI:4936865
Allelic
Composition
Dmdmdx/Y
Terctm1Rdp/Terctm1Rdp
Genetic
Background
involves: 129/Sv * C57BL/6J * C57BL/10ScSn * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dmdmdx mutation (42 available); any Dmd mutation (159 available)
Terctm1Rdp mutation (3 available); any Terc mutation (4 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• second generation mice beginning at 48 weeks of age likely due to respiratory failure

muscle
• first and second generation mice exhibit a progressive reduction in revertant myofiber cluster size compared to in control mice
• at 8 weeks in second generation mice
• in first and second generation mice
• diaphragms in first and second generation mice exhibit increased diameter compared with control mice
• by 76 weeks, diaphragm muscle is atrophied unlike in control mice
• in aged second generation mice
• in second generation mice with age
• in second generation mice
• at 8 weeks in second generation mice
• second generation mice exhibit progressive muscular dystrophy with age unlike control mice
• however, dystrophic phenotypes are improved by transplantation with wild-type muscle stem cells
• in first and second generation mice prior to and after damage
• at 8 weeks, second generation mice exhibit increased myofiber membrane permeability compared with control mice
• second generation mice exhibit impaired muscle stem cell proliferation compared with control mice
• muscle stem cells from second generation mice exhibit impaired proliferative potential in culture compared with control cells
• muscle stem cells from second generation mice exhibit impaired engrafting after transplantation compared with control cells
• at 8 weeks in second generation mice
• at 8 weeks, first and second generation mice exhibit decreased muscle twitch force, tetanic force, and tetanic tension compared with control mice
• at 8 weeks, second generation mice spend less time running on a treadmill than control mice
• at 8 weeks, second generation mice hold onto a grid for less time than control mice

homeostasis/metabolism
• at 8 weeks, second generation mice spend less time running on a treadmill than control mice
• at 8 weeks in second generation mice, but declining after 60 weeks of age
• second generation mice exhibit calcium depositions in the muscles unlike control mice

cellular
• myoblasts from first and second generation exhibit shortened telomeres compared with control cells
• myoblasts from first and second generation exhibit fused chromosomes unlike control cells

skeleton
• in aged second generation mice

respiratory system
• second generation mice beginning at 48 weeks of age likely due to respiratory failure

immune system
• at 8 weeks in second generation mice

behavior/neurological
• at 8 weeks, second generation mice spend less time running on a treadmill than control mice

Mouse Models of Human Disease
OMIM ID Ref(s)
Muscular Dystrophy, Duchenne Type; DMD 310200 J:167294


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
07/19/2016
MGI 6.04
The Jackson Laboratory