Mouse Genome Informatics
ot
    Dmdmdx/Y
C57BL/10ScSn-Dmdmdx
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
muscle
N
• limb and diaphragm muscle weights are similar to controls in mice at 1 year of age (J:177391)
• unlike in wild-type mice, cardiac fiber degeneration with phagocytosis is observed (J:9638)
• increased collagen deposition in the diaphragm and quadriceps muscles between 12 weeks and 1 year of age (J:177391)
• at 15 days of age necrotic fibers are found, occassionally in clusters, at 20 days of age necrotic fibers are numerous as are groups of regenerating fibers with internally placed nuclei, necrotic fibers are most numerous between 30 and 60 days of age and gradually dissapear although a few scattered necrotic fibers are present at 120 and 180 days of age, and the regenerating fibers increase in size after 30 days of age and almost all of the fibers have internally placed nuclei by 60 to 120 days of age, but there is almost no fibrous tissue proliferation or adipose tissue replacement (J:152525)
• mice exhibit skeletal muscle fiber degeneration with phagocytosis unlike in wild-type mice (J:9638)
• scattered hypercontracted fibers are found by electron microscopy at 10 days of age (J:152525)
• decrease in the tetanic force produced by the diaphragm and extensor digitorum longus muscles compared to wild-type controls (J:177391)
• electromyograms reveal peudomyotonia unlike in wild-type mice (J:9638)

behavior/neurological
N
• performance in a rotarod assay is not significantly different from controls in mice at 6 - 20 weeks of age (J:177391)
• in fore and hind paws (J:177391)
• increased hind paw base width (J:177391)
• at 1 year of age (J:177391)
• at 6 weeks of age, display a decrease in rearings after exercise compared to wild-type controls (J:177391)
• at 12 weeks of age, display a decrease in ambulation and increase in rest time after exercise compared to wild-type controls (J:177391)
• at 1 year of age, display a decrease in distance traveled after exercise compared to wild-type controls (J:177391)
• interindividual responses to exercise induced fatigue are highly variable (J:177391)

adipose tissue
• decrease in the accumulation of abdominal fat compared to wild-type controls (J:177391)

growth/size/body
• between 8 and 20 weeks of age compared to wild-type controls (J:177391)
• however, body size as determined by measures of tibia length or body length is similar to wild-type controls (J:177391)

cardiovascular system
• unlike in wild-type mice, cardiac fiber degeneration with phagocytosis is observed (J:9638)

homeostasis/metabolism
• increased collagen deposition in the diaphragm and quadriceps muscles between 12 weeks and 1 year of age (J:177391)

Mouse Models of Human Disease
OMIM IDRef(s)
Muscular Dystrophy, Duchenne Type; DMD 310200 J:177391