Analysis Tools
cellular
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• mice exhibit progressive reduction of chondrocyte proliferation (a 25% reduction at birth, 60% reduction at 3 weeks and no proliferation at 6 weeks)
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mortality/aging
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• most mice die shortly after birth due to respiratory distress
• however, 3 of 223 mice survive
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skeleton
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• mice exhibit progressive reduction of chondrocyte proliferation (a 25% reduction at birth, 60% reduction at 3 weeks and no proliferation at 6 weeks)
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• at E17.5, mice exhibit reductions of the half tibia length (17.2% reduction), the distance between the growth plate and the middle of diaphysis (43.2% reduction), the length of the bone marrow zone (45% reduction) and the length of the hypertrophic zone (15% reduction) compared to wild-type mice
• at E17.5, the resting zone is increased in length by 10% compared to wild-type mice
• the growth plate is severely disorganized with large and round-shaped chondrocytes failing to glide over each other and form columns as in wild-type mice
• at 6 weeks of age, growth plates are completely disorganized and broadened
• however, the length of the proliferative zone and the total growth plate height are normal
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• at E17.5, mice exhibit 15% reduction in the length of the hypertrophic zone compared to wild-type mice
• while the hyperproliferative zone is reduced, the prehyperproliferative zone is increased
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• at E17.5, bones are severely shortened
• at birth, long bones in surviving mice are short and broad
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short humerus
(
J:85994
)
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• at E14.5
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short tibia
(
J:85994
)
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• at E17.5, mice exhibit 17.2% reduction of the half tibia length compared to wild-type mice
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• at birth, long bones in surviving mice are short and broad
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• cartilage differentiation is impaired
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• chondrocyte spreading is defective and adhesion to collagen and laminin is absent while adhesion to fibronectin is reduced 55% compared to in wild-type mice
• however, chondrocytes bind vitronectin normally
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• mice exhibit progressive reduction of chondrocyte proliferation (a 25% reduction at birth, 60% reduction at 3 weeks and no proliferation at 6 weeks) and increased apoptosis resulting in fewer chondrocytes than in wild-type mice
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• surviving mice fail to exhibit secondary ossification centers in the epiphysis
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• mineralization is delayed as evidenced by absent ossification centers in cervical vertebrae
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growth/size/body
cleft palate
(
J:85994
)
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• in 50% of mice
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• at birth
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• mice that survive develop progressive dwarfism with a 40% reduction in skeletal length by 9 weeks of age
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craniofacial
cleft palate
(
J:85994
)
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• in 50% of mice
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respiratory system
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• at birth
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limbs/digits/tail
short humerus
(
J:85994
)
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• at E14.5
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short tibia
(
J:85994
)
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• at E17.5, mice exhibit 17.2% reduction of the half tibia length compared to wild-type mice
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digestive/alimentary system
cleft palate
(
J:85994
)
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• in 50% of mice
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