Osteoprotegerin [bony-1]

bony-1

Recombinant fragment corresponding to amino acids 22-202 of Human Osteoprotegerin.

Recognizes human OPG

Secreted

0. 02% Sodium Azide. Constituents: PBS

Secreted Osteoprotegerin (OPG) 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 (TNFSF11/OPGL) both of which are key extracellular regulators of osteoclast development. Studies of the mouse counterpart also suggest this protein and its ligand play a role in lymph-node organogenesis and vascular calcification. Alternatively spliced transcript variants of the gene have been reported but their full length nature has not been determined.

Homodimer.

Highly expressed in adult lung heart kidney liver spleen thymus prostate ovary small intestine thyroid lymph node trachea adrenal gland testis and bone marrow. Detected at very low levels in brain placenta and skeletal muscle. Highly expressed in fetal kidney liver and lung.

N-glycosylated. Contains sialic acid residues.

Defects in TNFRSF11B are the cause of juvenile Paget disease (JPD) [MIM:239000]; also called 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.

Contains 4 TNFR-Cys repeats.