TNFRSF11B
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]
Full Name
TNFRSF11B TNF Receptor Superfamily Member 11b
Function
Acts as decoy receptor for TNFSF11/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 ratio between TNFSF11 and TNFRSF11B. May also play a role in preventing arterial calcification. May act as decoy receptor for TNFSF10/TRAIL and protect against apoptosis. TNFSF10/TRAIL binding blocks the inhibition of osteoclastogenesis.
Biological Process
Biological Process apoptotic process Source:UniProtKB-KW
Biological Process extracellular matrix organization Source:Ensembl
Biological Process negative regulation of bone resorption Source:Ensembl
Biological Process negative regulation of odontogenesis of dentin-containing tooth Source:Ensembl
Biological Process negative regulation of osteoclast differentiation Source:Ensembl
Biological Process response to arsenic-containing substance Source:Ensembl
Biological Process response to estrogen Source:Ensembl
Biological Process response to magnesium ion Source:Ensembl
Biological Process response to nutrient Source:Ensembl
Biological Process response to xenobiotic stimulus Source:Ensembl
Biological Process signal transduction Source:ProtInc1 Publication
Biological Process skeletal system development Source:ProtInc1 Publication
Cellular Location
Secreted
Involvement in disease
Paget disease of bone 5, juvenile-onset (PDB5):
An autosomal recessive, juvenile-onset form of Paget disease, a disorder of bone remodeling characterized by increased bone turnover affecting one or more sites throughout the skeleton, primarily the axial skeleton. Osteoclastic overactivity followed by compensatory osteoblastic activity leads to a structurally disorganized mosaic of bone (woven bone), which is mechanically weaker, larger, less compact, more vascular, and more susceptible to fracture than normal adult lamellar bone. PDB5 clinical manifestations include short stature, progressive long bone deformities, fractures, vertebral collapse, skull enlargement, and hyperostosis with progressive deafness.
PTM
N-glycosylated. Contains sialic acid residues.
The N-terminus is blocked.