RANKL Inhibition in Fibrous Dysplasia of Bone: A Preclinical Study in a Mouse Model of the Human Disease.

ABSTRACT: Fibrous dysplasia of bone/McCune-Albright syndrome (Polyostotic FD/MAS; OMIM#174800) is a crippling skeletal disease caused by gain-of-function mutations of G_s α. Enhanced bone resorption is a recurrent histological feature of FD and a major cause of fragility of affected bones. Previous work suggests that increased bone resorption in FD is driven by RANKL and some studies have shown that the anti-RANKL monoclonal antibody, denosumab, reduces bone turnover and bone pain in FD patients. However, the effect of RANKL inhibition on the histopathology of FD and its impact on the natural history of the disease remain to be assessed. In this study, we treated the EF1α-G_s α^R201C mice, which develop an FD-like phenotype, with an anti-mouse RANKL monoclonal antibody.

SUBMITTER: Palmisano B 

PROVIDER: S-EPMC8408916 | biostudies-literature | 2019 Dec

REPOSITORIES: biostudies-literature