Analysis Tools|
Allele Symbol Allele Name Allele ID |
Dmdmdx-4Cv X linked muscular dystrophy 4, Verne Chapman MGI:1856331 |
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| Summary |
6 genotypes
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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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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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• skeletal muscle fibers undergo cycles of degeneration and regeneration accompanied by necrosis, fibrosis and centrally located nuclei
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• cardiomyocyte nuclear area is moderately reduced in hearts of second generation (G2) 32 week old males as compared to controls
• cardiomyocyte diameter is smaller in hearts of 32 week old G2 males
• hearts from G2 males exhibit a loss of thick and thin myofilaments
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• left ventricle enlargement is observed by 32 weeks in hearts of G2 males as compared to controls
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• increased chamber size is observed in 32 week old G2 males as compared to controls
• left ventricular transverse area is increased in diastole and systole in G2 males
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• ventricular fibrosis is observed by 32 weeks in hearts of G2 males
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• reduced cardiac function is observed in older G1 males (80 weeks), but is not observed at 32 weeks
• cardiac dysfunction is observed at 8 weeks in third generation males
• cardiac dysfunction can be induced by angiotension II infusion in younger second generation males
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• 32 week old G2 males exhibit reduced ventricular contractility as assessed by a reduction in fractional shortening as compared to controls
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• wide QRS interval is observed in both G1 and G2 males as compared to controls
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• 50% reduction in telomere length in G2 cardiomyocytes as compared to controls
• however, telomere lengths are similar to controls in vascular smooth muscle cells
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• lack of well-defined cristae observed in cardiac muscle of second generation mice
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• extensive mitochondrial fragmentation observed in cardiac muscle of second generation mice
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• mitochondria size is decreaseed in cardiac muscle of second generation mice
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• moderately increased number of mitochondria is observed in cardiac muscle of second generation mice
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• an increased number of 8-OHdg-positive nuclei, a marker of oxidative damage, is observed in G2 hearts as compared to controls
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• death occurs as early as 30 weeks in first generation males, T50 is 120 weeks
• death occurs as early as 19 weeks in second generation males, T50 is 80 weeks
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• cardiomyocyte nuclear area is moderately reduced in hearts of second generation (G2) 32 week old males as compared to controls
• cardiomyocyte diameter is smaller in hearts of 32 week old G2 males
• hearts from G2 males exhibit a loss of thick and thin myofilaments
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• reduced cardiac function is observed in older G1 males (80 weeks), but is not observed at 32 weeks
• cardiac dysfunction is observed at 8 weeks in third generation males
• cardiac dysfunction can be induced by angiotension II infusion in younger second generation males
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• 32 week old G2 males exhibit reduced ventricular contractility as assessed by a reduction in fractional shortening as compared to controls
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| Duchenne muscular dystrophy | DOID:11723 |
OMIM:310200 |
J:200365 | |
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• muscle fiber diameter is twice as large as in wild-type mice
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• the number of necrotic fibers and the size of necrotic foci is larger than in Dmdmdx-4Cv homozygotes
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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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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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• skeletal muscles exhibit variation in fiber size and centrally located nuclei unlike in wild-type mice
• mice exhibit macrophage invasion into muscles unlike in wild-type mice
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• highest specific tetanic force is lower than in wild-type mice
• at high frequencies mice titanic force is lower than in Dmdmdx-3Cv mice
• following eccentric contraction damage, force is reduced to 20% of starting level unlike in wild-type mice that experience only a moderate drop in force
• age exacerbates force losses
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• derived positive (P2) responses are delayed compared to in wild-type mice
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• mice exhibit weaker grip strength than Dmdmdx-3Cv mice
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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 12/10/2024 MGI 6.24 |
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