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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 (39 available); any Dmd mutation (135 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 (J:167294)
• second generation mice beginning at 48 weeks of age likely due to respiratory failure (J:167294)

muscle
• first and second generation mice exhibit a progressive reduction in revertant myofiber cluster size compared to in control mice (J:167294)
• first and second generation mice exhibit a progressive reduction in revertant myofiber cluster size compared to in control mice (J:167294)
• at 8 weeks in second generation mice (J:167294)
• at 8 weeks in second generation mice (J:167294)
• in first and second generation mice (J:167294)
• in first and second generation mice (J:167294)
• diaphragms in first and second generation mice exhibit increased diameter compared with control mice (J:167294)
• by 76 weeks, diaphragm muscle is atrophied unlike in control mice (J:167294)
• diaphragms in first and second generation mice exhibit increased diameter compared with control mice (J:167294)
• by 76 weeks, diaphragm muscle is atrophied unlike in control mice (J:167294)
• in aged second generation mice (J:167294)
• in aged second generation mice (J:167294)
• in second generation mice with age (J:167294)
• in second generation mice with age (J:167294)
• in second generation mice (J:167294)
• in second generation mice (J:167294)
• at 8 weeks in second generation mice (J:167294)
• at 8 weeks in second generation mice (J:167294)
• second generation mice exhibit progressive muscular dystrophy with age unlike control mice (J:167294)
• however, dystrophic phenotypes are improved by transplantation with wild-type muscle stem cells (J:167294)
• second generation mice exhibit progressive muscular dystrophy with age unlike control mice (J:167294)
• however, dystrophic phenotypes are improved by transplantation with wild-type muscle stem cells (J:167294)
• in first and second generation mice prior to and after damage (J:167294)
• in first and second generation mice prior to and after damage (J:167294)
• at 8 weeks, second generation mice exhibit increased myofiber membrane permeability compared with control mice (J:167294)
• second generation mice exhibit impaired muscle stem cell proliferation compared with control mice (J:167294)
• muscle stem cells from second generation mice exhibit impaired proliferative potential in culture compared with control cells (J:167294)
• muscle stem cells from second generation mice exhibit impaired engrafting after transplantation compared with control cells (J:167294)
• at 8 weeks, second generation mice exhibit increased myofiber membrane permeability compared with control mice (J:167294)
• second generation mice exhibit impaired muscle stem cell proliferation compared with control mice (J:167294)
• muscle stem cells from second generation mice exhibit impaired proliferative potential in culture compared with control cells (J:167294)
• muscle stem cells from second generation mice exhibit impaired engrafting after transplantation compared with control cells (J:167294)
• at 8 weeks in second generation mice (J:167294)
• at 8 weeks in second generation mice (J:167294)
• at 8 weeks, first and second generation mice exhibit decreased muscle twitch force, tetanic force, and tetanic tension compared with control mice (J:167294)
• at 8 weeks, first and second generation mice exhibit decreased muscle twitch force, tetanic force, and tetanic tension compared with control mice (J:167294)
• at 8 weeks, second generation mice spend less time running on a treadmill than control mice (J:167294)
• at 8 weeks, second generation mice hold onto a grid for less time than control mice (J:167294)
• at 8 weeks, second generation mice spend less time running on a treadmill than control mice (J:167294)
• at 8 weeks, second generation mice hold onto a grid for less time than control mice (J:167294)

homeostasis/metabolism
• at 8 weeks, second generation mice spend less time running on a treadmill than control mice (J:167294)
• at 8 weeks, second generation mice spend less time running on a treadmill than control mice (J:167294)
• at 8 weeks in second generation mice, but declining after 60 weeks of age (J:167294)
• at 8 weeks in second generation mice, but declining after 60 weeks of age (J:167294)
• second generation mice exhibit calcium depositions in the muscles unlike control mice (J:167294)
• second generation mice exhibit calcium depositions in the muscles unlike control mice (J:167294)

cellular
• myoblasts from first and second generation exhibit shortened telomeres compared with control cells (J:167294)
• myoblasts from first and second generation exhibit shortened telomeres compared with control cells (J:167294)
• myoblasts from first and second generation exhibit fused chromosomes unlike control cells (J:167294)
• myoblasts from first and second generation exhibit fused chromosomes unlike control cells (J:167294)

skeleton
• in aged second generation mice (J:167294)
• in aged second generation mice (J:167294)

respiratory system
• second generation mice beginning at 48 weeks of age likely due to respiratory failure (J:167294)
• second generation mice beginning at 48 weeks of age likely due to respiratory failure (J:167294)

immune system
• at 8 weeks in second generation mice (J:167294)
• at 8 weeks in second generation mice (J:167294)

behavior/neurological
• at 8 weeks, second generation mice spend less time running on a treadmill than control mice (J:167294)
• at 8 weeks, second generation mice spend less time running on a treadmill than control mice (J:167294)

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


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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