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Phenotypes Associated with This Genotype
Genotype
MGI:5697652
Allelic
Composition
Dmdmdx/Dmdmdx
Genetic
Background
D2.B10-Dmdmdx/J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dmdmdx mutation (32 available); any Dmd mutation (153 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
muscle
• lower ejection fraction and shortening fraction than DBA/2J controls at 28 and 52 weeks of age
• Background Sensitivity: ejection fraction and shortening fraction is lower than in Dmdmdx mice on C57BL/10ScSn background at 28 weeks of age
• cardiomyopathy is observed at 7 weeks of age in mice on the DBA/2J background
• Background Sensitivity: cardiomyopathy is observed at 28 weeks of age in Dmdmdx mice on the C57BL/10ScSn background
• forelimb and hindlimb inflammation (as measured by cathepsin activity) is observed at 7 weeks of age
• inflammation in the forelimb is still observed at 52 weeks of age
• inflammation and mononuclear cell infiltration is observed in the quadriceps beginning at 7 weeks of age
• Background Sensitivity: forelimb inflammation at 52 weeks is not observed in mice on on the C57BL/10ScSn background
• high incidence of primary and complex branching in myofibers from extensor digitorum longus (EDL) in 12 week old male mice
• increase in average number of nuclei per myofiber and decreased F-actin content from EDL at 12 weeks of age
• decrease in myonuclear domain from EDL at 12 weeks of age
• increased number of small fibers and smaller number of normal sized fibers
• tibialis anterior muscle fibers are reduced in diameter (375 m2) as compared to DBA/2J mice (375-750 m2)
• mice exhibit an increased number of centrally nucleated fibers
• myonuclei are predominantly located in juxtasarcolemmal position on the DBA/2J background
• Background Sensitivity: increase is less on the DBA/2J background than on the C57BL/10ScSn background
• Background Sensitivity: myonuclei are predominantly centrally located on the C57BL/10ScSn background
• loss of muscle mass in gastrocnemius, tibialis anterior and quadriceps as compared to controls
• reduced muscle mass in extensor digitorum longus (EDL) as compared to DBA/2J at 28 and 52 weeks of age
• Background Sensitivity: reduced muscle mass in EDL as compared to Dmdmdx mice on C57BL/10ScSn background at 7, 28 and 52 weeks of age
• muscle atrophy is observed in mice on the DBA/2J background
• Background Sensitivity: however, muscle hypertrophy is observed in Dmdmdx mice on the C57BL/10ScSn background
• lesions that are observed in quadriceps and diaphragm exhibit muscle degeneration, regeneration and mononuclear infiltrating cells at 7 weeks of age
• increased uptake of Evans blue dye, a measure of muscle damage, as compared to DBA/2J controls at 8 weeks of age
• Background Sensitivity: uptake is higher than in Dmdmdx mice on the C57BL/10ScSn background
• calcifications in quadriceps, heart, diaphragm and epicardium of heart at 7 weeks of age
• Background Sensitivity: Dmdmdx mice on the C57BL/10ScSn background did not exhibit calcifications until 52 weeks of age
• Background Sensitivity: onset of muscular dystrophy is later and less severe in Dmdmdx mice on on the C57BL/10ScSn background
• decrease in specific and maximal force generation in EDL at 7, 28 and 52 weeks as compared to DBA/2J controls
• Background Sensitivity: decrease in specific and maximal force generation in EDL at 7 and 28 weeks as compared to Dmdmdx mice on C57BL/10ScSn background
• lesions that are observed in quadriceps and diaphragm exhibit muscle degeneration, regeneration and mononuclear infiltrating cells at 7 weeks of age

cardiovascular system
• calcifications in quadriceps, heart, diaphragm and epicardium of heart at 7 weeks of age
• Background Sensitivity: Dmdmdx mice on the C57BL/10ScSn background did not exhibit calcifications until 52 weeks of age
• decreased left ventricular (stroke) volume
• lower ejection fraction and shortening fraction than DBA/2J controls at 28 and 52 weeks of age
• Background Sensitivity: ejection fraction and shortening fraction is lower than in Dmdmdx mice on C57BL/10ScSn background at 28 weeks of age
• cardiomyopathy is observed at 7 weeks of age in mice on the DBA/2J background
• Background Sensitivity: cardiomyopathy is observed at 28 weeks of age in Dmdmdx mice on the C57BL/10ScSn background
• inflammation within cardiac tissue is observed by 7 weeks of age
• Background Sensitivity: inflammation is not observed in in Dmdmdx mice on the C57BL/10ScSn background

growth/size/body
• mice have reduced body mass as compared to DBA/2J controls
• Background Sensitivity: Dmdmdx mice on the DBA/2J background have reduced body mass as compared to mice on the C57BL/10ScSn background at 28 weeks

homeostasis/metabolism
• elevated levels of serum creatine kinase as compared to DBA/2J controls at 24 and 28 weeks
• Background Sensitivity: levels are lower at 24 weeks as compared to Dmdmdx mice on the C57BL/10ScSn background
• increased creatine kinase activity as compared to DBA/2J controls at 24 and 28 weeks
• Background Sensitivity: activity is lower at 24 weeks as compared to Dmdmdx mice on the C57BL/10ScSn background

immune system
• inflammation within cardiac tissue is observed by 7 weeks of age
• Background Sensitivity: inflammation is not observed in in Dmdmdx mice on the C57BL/10ScSn background
• forelimb and hindlimb inflammation (as measured by cathepsin activity) is observed at 7 weeks of age
• inflammation in the forelimb is still observed at 52 weeks of age
• inflammation and mononuclear cell infiltration is observed in the quadriceps beginning at 7 weeks of age
• Background Sensitivity: forelimb inflammation at 52 weeks is not observed in mice on on the C57BL/10ScSn background

behavior/neurological
• reduced forelimb grip strength at 12 weeks and 24 weeks as compared to controls
• reduced hindlimb grip strength at 24 weeks, as compared to controls, however, gap closes by 52 weeks
• Background Sensitivity: forelimb grip strength at 28 weeks is higher than grip strength in Dmdmdx mice on C57BL/10ScSn background, but still less than controls

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
Duchenne muscular dystrophy DOID:11723 OMIM:310200
J:226314


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB), Gene Ontology (GO)
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last database update
02/16/2021
MGI 6.16
The Jackson Laboratory