Mouse Genome Informatics
ot
    Dmdmdx-5Cv/Y
B6Ros.Cg-Dmdmdx-5Cv
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)
• mice exhibit progressive fibrosis of the diaphragm with age, unlike in wild-type mice (J:23502)
• increased collagen deposition in the diaphragm and quadriceps muscles between 12 weeks and 1 year of age (J:177391)
• mice exhibit fibrosis, fatty infiltration and necrosis in the diaphragm unlike in wild-type mice that increases with age (J:23502)
• decrease in the tetanic force produced by the diaphragm muscle compared to wild-type controls and mice hemizygous for Dmdmdx (J:177391)
• however, the decrease in the tetanic force produced by the extensor digitorum longus muscle is similar to mice hemizygous for Dmdmdx (J:177391)
• the diaphragm muscle shows enhanced susceptibility to fatigue in a repetitive isometric contraction protocol compared to wild-type controls and mice hemizygous for Dmdmdx (J:177391)

behavior/neurological
• significant motor deficit is detected in a rotarod assay at 6 - 20 weeks of age (J:177391)
• in fore and hind paws (J:177391)
• the hind paw does not always land on the same position as the front paw (J:177391)
• increased hind paw base width (J:177391)
• at 8 weeks, 12 weeks and 1 year of age (J:177391)
• display extreme fatigue after 15 min of downhill treadmill running (J:177391)
• by 8 weeks of age show maximum decreases in both ambulation and rearing events after exercise, earlier than in mice hemizygous for Dmdmdx (J:177391)
• by 12 weeks of age show a decrease in distance traveled after exercise compared to wild-type controls and mice hemizygous for Dmdmdx (J:177391)
• response to exercise induced fatigue is uniform across individuals unlike in mice hemizygous for Dmdmdx (J:177391)

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

growth/size
• between 8 and 20 weeks of age compared to wild-type controls (J:177391)
• lower than in mice hemizygous for Dmdmdx at most ages tested (J:177391)
• however, body size as determined by measures of tibia length or body length is similar to wild-type controls (J:177391)

homeostasis/metabolism
• mice exhibit progressive fibrosis of the diaphragm with age, unlike in wild-type mice (J:23502)
• 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