Osteoprotegerin (OPG) ELISA Kit for Rabbit

The protein encoded by this gene is a member of the TNF-receptor superfamily. This protein is an osteoblast-secreted decoy receptor that functions as a negative regulator of bone resorption. This protein specifically binds to its ligand, osteoprotegerin ligand, both of which are key extracellular regulators of osteoclast development. Studies of the mouse counterpart also suggest that this protein and its ligand play a role in lymph-node organogenesis and vascular calcification. Alternatively spliced transcript variants of this gene have been reported, but their full length nature has not been determined. [provided by RefSeq, Jul 2008]

TNFRSF11B: Acts as decoy receptor for RANKL and thereby neutralizes its function in osteoclastogenesis. Inhibits the activation of osteoclasts and promotes osteoclast apoptosis in vitro. Bone homeostasis seems to depend on the local RANKL/OPG ratio. May also play a role in preventing arterial calcification. May act as decoy receptor for TRAIL and protect against apoptosis. TRAIL binding blocks the inhibition of osteoclastogenesis. Defects in TNFRSF11B are the cause of juvenile Paget disease (JPD); also known as hyperostosis corticalis deformans juvenilis or hereditary hyperphosphatasia or chronic congenital idiopathic hyperphosphatasia. 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. Approximately 40 cases of JPD have been reported worldwide. Unless it is treated with drugs that block osteoclast- mediated skeletal resorption, the disease can be fatal.

Protein type: Secreted; Secreted, signal peptide; Inhibitor

Chromosomal Location of Human Ortholog: 8q24

Cellular Component: extracellular region; extracellular space; integral to plasma membrane; plasma membrane; proteinaceous extracellular matrix

Molecular Function: cytokine activity; receptor activity; tumor necrosis factor receptor activity

Biological Process: extracellular matrix organization and biogenesis; immune response; inflammatory response; negative regulation of bone resorption; negative regulation of odontogenesis of dentine-containing teeth; positive regulation of MAPKKK cascade; regulation of apoptosis; regulation of cell proliferation; response to arsenic; response to drug; response to estrogen stimulus; response to lipopolysaccharide; response to magnesium ion; response to nutrient; signal transduction; skeletal development; tumor necrosis factor-mediated signaling pathway

Disease: Paget Disease, Juvenile