mortality/aging
• mice die at birth
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skeleton
• long bones are abnormal and bent (kyphosis) compared to in wild-type mice
• however, skeletal patterning and growth is normal
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• at E16.5, limb diaphyses are short and thick compared to in wild-type mice
• at E16.5, primary ossification centers are malformed compared to in wild-type mice
• at E16.5, ossification centers are laterally expanded with excessive, disorganized bone compared to in wild-type mice
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• at E16.5, rib diaphyses are short and thick compared to in wild-type mice
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• at E16.5, limb and rib diaphyses are short and thick compared to in wild-type mice
• bone lack a proper cortex and abnormally oriented trabecular-like structures extend inside the bone, obliterating the developing marrow cavity unlike in wild-type mice
• mice exhibit increased blood vessels in the bone compared with wild-type mice
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• abnormally oriented trabecular-like structures extend inside the bone, obliterating the developing marrow cavity unlike in wild-type mice
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• at E16.5, primary ossification centers are malformed compared to in wild-type mice
• at E16.5, ossification centers are laterally expanded with excessive, disorganized bone compared to in wild-type mice
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