Analysis Tools
reproductive system
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• both males and females show reduced fertility
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skeleton
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• marker analysis of cultured mouse calvarial osteoblasts indicates enhanced osteoblast differentiation
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• males show enhanced radiopacity in mandibles at 16 weeks of age
• the mandible bulges slightly, especially at the mandibular angle
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• nearly 80% reduction in the number of mature osteoclasts in the germinal center of the femur, indicating a deficiency in osteoclast maturation in vivo
• bone marrow-derived macrophages (BMMs) cultured in osteoclast differentiation medium show a reduction in the number and size of TRAP+ osteoclasts indicating decreased osteoclastogenesis
• cultured mouse calvarial osteoblast and bone marrow-derived macrophage cocultures show few mature osteoclasts
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• osteoclast cultures show smaller resorption pits indicating suppressed bone resorption
• F-actin ring formation is inhibited in osteoclasts and the actin ring is broken
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• expression of IL-6, TNF-alpha, and CD31 is increased in the periodontal ligament tissue, indicating inflammatory responses in the mandible
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bowed tibia
(
J:336715
)
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• tibias are bowed at an unnatural angle
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• tibia show disordered arrangement of collagen fibers and an appearance of woven bone
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• males how increased bone mineral content in both mandibles and diaphyses of the limb bones
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• males how increased bone mineral density in both mandibles and diaphyses of the limb bones
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• males how cortical thickening of femurs and tibias at 16 weeks of age
• the long bone cortex thickening causes narrowing of medullary cavity
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• mandibles and diaphyses of femurs and tibias exhibit decreased trabecular number
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• mandibles and diaphyses of femurs and tibias show elevated trabecular spacing
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• mandibles and diaphyses of femurs and tibias show elevated trabecular pattern factor
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• mandibles and diaphyses of femurs and tibias are under trabeculated with decreased trabecular thickness
• trabeculation is drastically decreased in the tibial and femoral metaphysis
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• cultured mouse calvarial osteoblasts (mCOBs) show more mineralized nodules after osteogenic induction with higher calcium content, indicating mature osteoblasts and increased osteogenesis
• ratio of calcium and phosphorus is higher in mineralized nodules and mCOBs have a lower level of free calcium in the cytoplasm
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• mice show increased bone fragility at 8 weeks of age, with the tibia breaking more easily under smaller loads
• the maximum load that bone can bear before breaking and the fracture load show decreased tendency in tibia
• the maximum stress and fracture stress of tibia are decreased and the elastic modulus is reduced
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immune system
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• nearly 80% reduction in the number of mature osteoclasts in the germinal center of the femur, indicating a deficiency in osteoclast maturation in vivo
• bone marrow-derived macrophages (BMMs) cultured in osteoclast differentiation medium show a reduction in the number and size of TRAP+ osteoclasts indicating decreased osteoclastogenesis
• cultured mouse calvarial osteoblast and bone marrow-derived macrophage cocultures show few mature osteoclasts
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• osteoclast cultures show smaller resorption pits indicating suppressed bone resorption
• F-actin ring formation is inhibited in osteoclasts and the actin ring is broken
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• expression of IL-6, TNF-alpha, and CD31 is increased in the periodontal ligament tissue, indicating inflammatory responses in the mandible
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homeostasis/metabolism
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• level of serum alkaline phosphatase is increased by about 76%
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hematopoietic system
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• nearly 80% reduction in the number of mature osteoclasts in the germinal center of the femur, indicating a deficiency in osteoclast maturation in vivo
• bone marrow-derived macrophages (BMMs) cultured in osteoclast differentiation medium show a reduction in the number and size of TRAP+ osteoclasts indicating decreased osteoclastogenesis
• cultured mouse calvarial osteoblast and bone marrow-derived macrophage cocultures show few mature osteoclasts
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• osteoclast cultures show smaller resorption pits indicating suppressed bone resorption
• F-actin ring formation is inhibited in osteoclasts and the actin ring is broken
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craniofacial
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• males show enhanced radiopacity in mandibles at 16 weeks of age
• the mandible bulges slightly, especially at the mandibular angle
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limbs/digits/tail
bowed tibia
(
J:336715
)
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• tibias are bowed at an unnatural angle
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cellular
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• marker analysis of cultured mouse calvarial osteoblasts indicates enhanced osteoblast differentiation
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| gnathodiaphyseal dysplasia | DOID:0111533 |
OMIM:166260 |
J:336715 | |
