growth/size/body
• mice are shorter at birth and shorter stature is seen at weaning and persists into adulthood
|
• reduction in nose-to-anus body length
|
limbs/digits/tail
short femur
(
J:226676
)
|
short tibia
(
J:226676
)
|
skeleton
• chondrocyte proliferation in the tibiae is reduced
|
short femur
(
J:226676
)
|
short tibia
(
J:226676
)
|
• 22% reduction of trabecular volumetric bone mineral density
|
• reduction in cortical thickness in the metaphyseal regions of distal femur and proximal tibia, with an 18% reduction in cross-sectional cortical thickness
|
• ALP-positive osteoblast cell number is reduced whereas TRAP-positive osteoclast number remains unaltered in trabecular bone on neonatal tibias
|
• lower trabecular bone in the metaphyseal regions of distal femur and proximal tibia
|
• 41% reduction in trabecular bone volume
|
• 33% reduction in trabecular number
|
• 15% reduction in trabecular thickness
|
• skeletogenesis is abnormal, with all appendicular (the upper and lower limbs) and axial (the skull, the vertebral column, and the rib cage) skeletal elements smaller
• bone marrow mesenchymal stromal cells cultured in osteogenic differentiation media show impeded osteogenic differentiation
|
• 20% reduction in the length of the proliferative chondrocyte zone of growth plates
|
• 15% reduction in the length of the hypertrophic chondrocyte zone of growth plates
|
• embryonic limb mesenchymal cells show decreased chondrogenic differentiation in culture
|
• lower dynamic bone formation rate
|
• compromised endochondrial ossification
|
cellular
• chondrocyte proliferation in the tibiae is reduced
|