skeleton
N |
• normal serum calcuim, phosphate, and bone-derived alkaline phosphatase
• normal expression of calvarial, long bone, and vertebrate alkaline phosphatase
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• calvarial osteoblast cultures induced with beta-glycerophosphate and ascorbic acid to differentiate produce less osteocalcin and osteopontin than those derived from wild-type mice but wnt7b expression persists much longer than normal
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• bone marrow cultures with TNFSF11 and CSF1 have normal osteoclast differentiation
• cultured differentiating calvarial osteoblasts produce increased CSF1 longer than wild-type cultures although TNFSF11 and TNFRSF11B levels are normal
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• increased number of osteoclasts in proximal tibial trabecular bones
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• total femoral, trabecular, and cortical bone mineral density are 17%, 14%, and 16% lower than in heterozygotes with no abnormality detected in heterozygotes
• proximal tibial metaphyseal trabecular bones have 33% lower trabecular bone volume and 31% lower trabecular bone number and 52% higher trabecular separation than in heterozygotes
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• osteoid surface of proximal tibial metaphyseal trabecular bones is greater than in heterozygotes without increases in osteoblast surface or osteoblast number
• mineral apposition rate and bone formation rate in the proximal tibial metaphyseal trabecular bone is lower
• bone marrow and clavaria derived osteoblast cultures show delayed mineralization compared with those from heterozygotes when induced with beta glycerophosphate and ascorbic acid
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hematopoietic system
• bone marrow cultures with TNFSF11 and CSF1 have normal osteoclast differentiation
• cultured differentiating calvarial osteoblasts produce increased CSF1 longer than wild-type cultures although TNFSF11 and TNFRSF11B levels are normal
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• increased number of osteoclasts in proximal tibial trabecular bones
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immune system
• bone marrow cultures with TNFSF11 and CSF1 have normal osteoclast differentiation
• cultured differentiating calvarial osteoblasts produce increased CSF1 longer than wild-type cultures although TNFSF11 and TNFRSF11B levels are normal
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• increased number of osteoclasts in proximal tibial trabecular bones
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cellular
• calvarial osteoblast cultures induced with beta-glycerophosphate and ascorbic acid to differentiate produce less osteocalcin and osteopontin than those derived from wild-type mice but wnt7b expression persists much longer than normal
|
• bone marrow cultures with TNFSF11 and CSF1 have normal osteoclast differentiation
• cultured differentiating calvarial osteoblasts produce increased CSF1 longer than wild-type cultures although TNFSF11 and TNFRSF11B levels are normal
|