Osteoclastogenesis inhibitory factor (osteoprotegerin) acts as a decoy receptor for RANKL and thereby neutralizes its function in osteoclastogenesis. Osteoprotegerin also acts as a decoy receptor for TRAIL and protect against apoptosis. TRAIL binding blocks the inhibition of osteoclastogenesis. Osteoprotegerin inhibits the activation of osteoclasts and promotes osteoclast apoptosis in vitro. Bone homeostasis depends on the local RANKL/osteoprotegerin ratio. Defects in osteoprotegerin are the cause of juvenile Paget disease (JPD). JPD is a rare autosomal recessive osteopathy that presents in infancy or early childhood. The disorder is characterized by rapidly remodeling woven bone, osteopenia, debilitating fractures, and deformities due to a markedly accelerated rate of bone remodeling throughout the skeleton. Unless it is treated with drugs that block osteoclast-mediated skeletal resorption, the disease can be fatal.