mortality/aging
• mortality is increased in mutants after aortic banding (3 of 8 died during follow-up)
|
• life span is up to 9 months of age
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cellular
• increase in the number of active osteoblasts is due to accelerated differentiation of osteoprogenitors into mature osteoblasts rather than increased proliferation of osteoblasts
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• surfaces of bone trabeculae are nearly continuously covered by cuboidal osteoblasts and osteoid seams, indicating enlargement of the osteoblast compartment and functional activity
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growth/size/body
• grow slower starting at 4 weeks of age
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• show severe splenomegaly at around 9 months of age due to extramedullary hematopoiesis
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skeleton
• increase in radiodensity of the skull
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• calvariae display increased thickness and the bone marrow spaces are obliterated by bone tissue
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• increase in radiodensity of the distal and proximal ends of the long bones
• older mice display an increase in cortical thickness and in both the number and diameter of trabeculae in long bones
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short femur
(
J:64494
)
• femoral length is reduced at 8 weeks of age
|
• femoral metaphyses of 8 week old mutants show increased trabecular bone volume and osteoid volume and a 2-fold increase in osteoblast-covered bone surfaces
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• increase in radiodensity of the ribs
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• increase in radiodensity of vertebral bodies
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• increase in the number of active osteoblasts is due to accelerated differentiation of osteoprogenitors into mature osteoblasts rather than increased proliferation of osteoblasts
|
• older mice display an increase in cortical thickness of long bones
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• show a dramatic postnatal increase in bone mass of the entire skeleton, first apparent at 4 weeks of age
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• develop osteosclerosis as a result of increased skeletal mass due to increased bone formation
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• surfaces of bone trabeculae are nearly continuously covered by cuboidal osteoblasts and osteoid seams, indicating enlargement of the osteoblast compartment and functional activity
|
• exhibit an increase in mineralizing bone surfaces and slightly elevated mineral apposition rates
• cultures of primary osteoblasts show higher amounts of mineralized matrix at days 16 and 18
|
hematopoietic system
• show severe splenomegaly at around 9 months of age due to extramedullary hematopoiesis
|
immune system
• show severe splenomegaly at around 9 months of age due to extramedullary hematopoiesis
|
liver/biliary system
• develops in some mutants
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neoplasm
• some mutants develop bronchoalveolar tumors but no bone tumors
|
limbs/digits/tail
short femur
(
J:64494
)
• femoral length is reduced at 8 weeks of age
|
craniofacial
• increase in radiodensity of the skull
|
• calvariae display increased thickness and the bone marrow spaces are obliterated by bone tissue
|
cardiovascular system
• fractional shortening, velocity of circumferential shortening corrected for heart rate (VCFc) and peak aortic velocity corrected for heart rate (PAVc) are decreased, indicating reduced cardiac contractility
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muscle
• fractional shortening, velocity of circumferential shortening corrected for heart rate (VCFc) and peak aortic velocity corrected for heart rate (PAVc) are decreased, indicating reduced cardiac contractility
|