neoplasm
• myeloproliferative CML-like disease by 3-6 months of age
• massive infiltration of bone marrow and spleen with neutrophilic granulocytes
|
limbs/digits/tail
short tibia
(
J:88126
)
• tibia length was reduced by 6 months
|
skeleton
• 70% reduction in numbers of osteoclasts
|
short tibia
(
J:88126
)
• tibia length was reduced by 6 months
|
• severe osteopenia by 4 weeks of age wihich progressed with age
• bones were thin and brittle by 7 days of age
• by 1 month, vertebral bone mass was decreased
|
• cortical bone thickness severely reduced at 6 months
|
• number of osteoblasts is reduced by 7 days
• osteoblast numbers reduced up to 90% by 6 months
|
• defective osteoclast differentiation
|
• 60% reduction in cortical bone formation at 1 month, 40% reduction at 3 months
• 70% reduction in trabecular bone formation at 1 month, 30% reduction at 3 months
|
• bone resorption significantly reduced
|
• bone strength was significantly reduced
|
hematopoietic system
• defective osteoclast differentiation
|
• 70% reduction in numbers of osteoclasts
|
immune system
• defective osteoclast differentiation
|
• 70% reduction in numbers of osteoclasts
|
cellular
• defective osteoclast differentiation
|