skeleton
• by P30, limbs injected with adenovirus cre at P11 exhibit bony calluses encasing the tibia and fibula as well as fused with the pelvis and femur rendering hip, knee and ankle joints immobile unlike in similarly treated wild-type mice
• however, treatment of adenovirus cre exposed mice with LDN-193189, an inhibitor of BMP type I receptors, or dexamethasone reduces ectopic calcifications
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• by P30, limbs injected with adenovirus cre at P11 exhibit reduced mobility including loss of passive flexion in the hip, knee and ankle under aesthesia unlike in similarly treated wild-type mice
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muscle
• regardless of LDN-193189 treatment, limbs injected with adenovirus cre at P11 exhibit mononuclear cell infiltration and myocyte edema in tissues undergoing recombination unlike in similarly treated wild-type mice
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immune system
• regardless of LDN-193189 treatment, limbs injected with adenovirus cre at P11 exhibit mononuclear cell infiltration and myocyte edema in tissues undergoing recombination unlike in similarly treated wild-type mice
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
fibrodysplasia ossificans progressiva | DOID:13374 |
OMIM:135100 |
J:142253 |