Recent studies have underscored the significance of the impairment of WNT and BMP signaling pathways in OI genesis.428 Preclinical studies of sclerostin antibodies in the OI mouse model have demonstrated improved skeletal parameters compared to anti-resorption agents.429,430 In a recently finished 12-month phase 2b trial enrolling 112 types I, III, and IV adult OI patients (NCT03118570), administration of setrusumab (BPS804), a sclerostin antibody, showed increased lumbar, total body, and femoral neck BMD, bone strength and remodeling effect, and decreased fracture risk. This evidence concerns the gene SOST and osteogenesis imperfecta.