mortality/aging
|
• most mice die after birth due to asphyxiation
|
cellular
|
• delayed osteoblast mineralization and reduced capacity to form calcified bone
• in vivo, osteoblasts exhibit reduced mineral deposition compared with wild-type cells
• however, osteoblast differentiation program is normal
|
skeleton
|
• delayed osteoblast mineralization and reduced capacity to form calcified bone
• in vivo, osteoblasts exhibit reduced mineral deposition compared with wild-type cells
• however, osteoblast differentiation program is normal
|
|
• newborns exhibit wider sutures of the skull bones than in wild-type mice
|
|
• absence of olecranon processes
|
short ribs
(
J:195901
)
|
• shortened and curved
|
|
• narrow
|
|
• trabecular bone from vertebrae and femora exhibit expanded layers of nonmineralized osteoid surrounding the trabeculae of the secondary spongiosa compared to in wild-type mice
|
|
• at 2 months
|
|
• relative to bone volume at 2 months
|
|
• at E15.5, mice lack mineralization of skeletal elements and exhibit delayed mineralization of intramembranous skull bones compared with wild-type mice
• however, mice exhibit distinct mineralization of all skeletal elements at E16.5
|
osteomalacia
(
J:195901
)
|
• at E16.5, ossified regions are smaller than in wild-type mice
|
growth/size/body
|
• at E15.5
|
homeostasis/metabolism
| N |
• mice exhibit normal serum levels of calcium, phosphate and cleaved procollagen I N-terminal propeptide
|
craniofacial
|
• newborns exhibit wider sutures of the skull bones than in wild-type mice
|
limbs/digits/tail
|
• absence of olecranon processes
|
short limbs
(
J:195901
)
|
• shortened and curved
|


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