Mouse Genome Informatics
cn
    Tg(CAG-LacZ,-ACVR1*,-EGFP)35-1Mis/0
involves: C57BL/6 * DBA/2
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
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
• 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

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

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

Mouse Models of Human Disease
OMIM IDRef(s)
Fibrodysplasia Ossificans Progressiva; FOP 135100 J:142253