Analysis Tools
mortality/aging
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• mice show progression of the bone overgrowth, requiring euthanasia by 30 weeks of age from complications of spinal stenosis, infection or failure to thrive
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growth/size/body
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• mice are shorter starting at 6 weeks of age
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skeleton
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• mice exhibit asymmetric enlargement of the skeleton, with progressive increase in bone accumulation starting at 3 weeks of age
• mice maintained on doxycycline from conception do not develop a bone phenotype
• mice maintained on doxycycline from conception to weaning and then maintained on doxycycline-containing food exhibit normal bone phenotype
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• increase in bone accrual within the skull is seen at 3 weeks of age
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• increase in osteoclast number
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• at 3 weeks of age, femurs show replacement of the normal long bone structures within the diaphysis by disorganized trabecular bone
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• large increase in mid-diaphyseal diameter, but not femur length, at 3 weeks of age
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• a moderate increase in femur size is seen at 3 weeks of age
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• a large increase in femur weight is seen at 3 weeks of age
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• spinal stenosis is seen by 30 weeks of age
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• almost complete loss of marrow space in 9 week old mice
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• bone marrow structure is disrupted, with loss of the normal bone marrow cavity in 9 week old mice
• bone marrow elements are scattered in small islands between the trabeculi
• however, red blood cell, white blood cell, and platelet counts are normal and extramedullary hematopoiesis is not seen
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• mice show a 380% increase in whole-body areal bone mineral density at 9 weeks of age
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• increase in total bone volume in 9 week old mice
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• mice show loss of cortical bone in bony lesions
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• marker analysis indicates an increase is osteoblast-lineage cells in bone lesions
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• at 3 weeks of age, femurs show replacement of the normal long bone structures within the diaphysis by disorganized trabecular bone
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• in 9 week old mice
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• mice exhibit an osteosclerotic phenotype, with increased bone mineral
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• mice show increased and disordered bone formation
• by 9 weeks of age, generalized bony lesions are seen in all mice
• bone expansion is not due to heterotopic ossification
• markers of bone turnover are increased in bony lesions
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craniofacial
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• increase in bone accrual within the skull is seen at 3 weeks of age
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hematopoietic system
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• increase in osteoclast number
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immune system
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• increase in osteoclast number
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limbs/digits/tail
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• at 3 weeks of age, femurs show replacement of the normal long bone structures within the diaphysis by disorganized trabecular bone
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• large increase in mid-diaphyseal diameter, but not femur length, at 3 weeks of age
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• a moderate increase in femur size is seen at 3 weeks of age
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• a large increase in femur weight is seen at 3 weeks of age
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