Intriguingly, a similar skeletal phenotype with a high turnover rate and disorganized bone remodeling was observed in Camurati-Engelmann diseases with TGF-β1 mutation and in mice with TGF-β1 overexpression.411,412 As a coupling factor of bone remodeling,413 TGF-β1 guides BMSCs to the resorption sites, and this process is dependent on osteoclast resorption to activate the inactive TGF-β1 in the bone matrix.414 Nevertheless, the mechanism by which osteoclasts trigger such decoupling in PDB remains elusive. This evidence concerns the gene TGFB1 and Camurati-Engelmann disease.