Analysis Tools
mortality/aging
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• mice with a severe phenotype display postnatal lethality
• lethality is not rescued by bisphosphonate alendronate treatment
• mice with a mild phenotype survive to adulthood
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cardiovascular system
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• in mice with a severe phenotype cardiac vessels have disordered cell arrangements and thinner walls
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• mice with a severe phenotype display reduction in the amount of collagen and a disordered collagen matrix with fewer and thinner collagen fibrils
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• in mice with a severe phenotype
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• enlarged septa in mice with a severe phenotype
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• mice with a severe phenotype display a septal deformation with a convex bulge into the left ventricle during contraction
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• in mice with a severe phenotype
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• left ventricular end-systolic internal diameter is increased resulting in a reduced ejection fraction in mice with a severe phenotype
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• extended J-T interval in mice with a mild phenotype
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• in mice with a mild phenotype
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respiratory system
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• in mice with a severe phenotype
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• infiltrates of polymorphonuclear neutrophils and alveolar macrophages in mice with a severe phenotype
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cellular
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• cardiac fibroblasts from mice with a severe phenotype show disordered cytoplasm and Golgi abnormalities
• cardiac fibroblasts from mice with a severe or mild phenotype show a strong or slight decrease in collagen staining, respectively
• marginal decrease in collagen in lung fibroblasts from mice with a severe phenotype
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• in cardiac fibroblasts
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• in cardiac fibroblasts from mice with a severe phenotype
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growth/size/body
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• in mice with a severe phenotype
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• at 6-11 days of age in mice with a mild phenotype weight is 75% that of wild-type mice
• at 6-11 days of age in mice with a severe phenotype weight is 53% that of wild-type mice
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skeleton
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• rib fractures with callus formation are equally frequent in mice with severe or mild phenotypes
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homeostasis/metabolism
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• reduction of 44% in arterial pO2 and an increase of 40% in pCO2 accompanied by a 61% decrease in oxygen saturation in mice with a severe phenotype
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immune system
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• infiltrates of polymorphonuclear neutrophils and alveolar macrophages in mice with a severe phenotype
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muscle
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• mice with a severe phenotype display reduction in the amount of collagen and a disordered collagen matrix with fewer and thinner collagen fibrils
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• in mice with a severe phenotype
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• left ventricular end-systolic internal diameter is increased resulting in a reduced ejection fraction in mice with a severe phenotype
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| osteogenesis imperfecta type 3 | DOID:0110339 |
OMIM:259420 |
J:185988 | |
