Phenotypes associated with this allele
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dmdmdx-5Cv mutation
(1 available);
any
Dmd mutation
(153 available)
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muscle
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• mice exhibit fibrosis, fatty infiltration and necrosis in the diaphragm unlike in wild-type mice that increases with age
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• mice exhibit progressive fibrosis of the diaphragm with age, unlike in wild-type mice
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dmdmdx-5Cv mutation
(1 available);
any
Dmd mutation
(153 available)
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cardiovascular system
N |
• despite defects in conduction, mice do not exhibit cardiac fibrosis or cellular hypertrophy in cardiac tissues
• heart rate and ventricular repolarization, reflected on the mouse ECG by the ST interval, are similar to controls
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• decreased amplitude and prolonged duration
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• sodium current is decreased compared to in wild-type cardiomyocytes
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muscle
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• compared with wild-type mice
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• mice exhibit reduced muscle contractile function compared with wild-type mice
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mortality/aging
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• compared with Dmdmdx-5Cv Utrntm1Jrs homozygotes
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muscle
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• mice exhibit fewer muscle fibers with centrally localized nuclei compared with Dmdmdx-5Cv Utrntm1Jrs homozygotes
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growth/size/body
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• compared with Dmdmdx-5Cv Utrntm1Jrs homozygotes
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muscle
N |
• mice exhibit more stable and less degenerative muscle fibers with fewer calcium deposits and reduced fibrosis in the diaphragm compared with Dmdmdx-5Cv homozygotes
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• mice exhibit an increase in total satellite cells and myogenic precursor cells compared with Dmdmdx-5Cv homozygotes
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• mice exhibit an increase in the number of slow muscle fibers compared with Dmdmdx-5Cv homozygotes
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• mice exhibit less variability in skeletal muscle fiber size compared with Dmdmdx-5Cv homozygotes
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• mice exhibit an increase in total satellite cells and myogenic precursor cells compared with Dmdmdx-5Cv homozygotes and wild-type mice
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• mice exhibit improved muscle contractile function compared with Dmdmdx-5Cv homozygotes
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cardiovascular system
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• capillary density in the tibialis anterior muscle and diaphragm is increased compared to in Dmdmdx-5Cv homozygotes
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• mice exhibit increased blood perfusion compared with Dmdmdx-5Cv homozygotes
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dmdmdx-5Cv mutation
(1 available);
any
Dmd mutation
(153 available)
|
|
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muscle
N |
• limb and diaphragm muscle weights are similar to controls in mice at 1 year of age
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• mice exhibit fibrosis, fatty infiltration and necrosis in the diaphragm unlike in wild-type mice that increases with age
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• 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)
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• decrease in the tetanic force produced by the diaphragm muscle compared to wild-type controls and mice hemizygous for Dmdmdx
• however, the decrease in the tetanic force produced by the extensor digitorum longus muscle is similar to mice hemizygous for Dmdmdx
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• the diaphragm muscle shows enhanced susceptibility to fatigue in a repetitive isometric contraction protocol compared to wild-type controls and mice hemizygous for Dmdmdx
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behavior/neurological
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• significant motor deficit is detected in a rotarod assay at 6 - 20 weeks of age
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• the hind paw does not always land on the same position as the front paw
• increased hind paw base width
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• at 8 weeks, 12 weeks and 1 year of age
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• display extreme fatigue after 15 min of downhill treadmill running
• by 8 weeks of age show maximum decreases in both ambulation and rearing events after exercise, earlier than in mice hemizygous for Dmdmdx
• by 12 weeks of age show a decrease in distance traveled after exercise compared to wild-type controls and mice hemizygous for Dmdmdx
• response to exercise induced fatigue is uniform across individuals unlike in mice hemizygous for Dmdmdx
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adipose tissue
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• decrease in the accumulation of abdominal fat compared to wild-type controls
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growth/size/body
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• decrease in the accumulation of abdominal fat compared to wild-type controls
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• between 8 and 20 weeks of age compared to wild-type controls
• lower than in mice hemizygous for Dmdmdx at most ages tested
• however, body size as determined by measures of tibia length or body length is similar to wild-type controls
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