| Predicate | Object |
|---|---|
| rdf:type | |
| biopax3:comment |
FUNCTION: 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. SUBUNIT: Homodimer. SUBCELLULAR LOCATION: Secreted. TISSUE SPECIFICITY: 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. INDUCTION: Up-regulated by increasing calcium-concentration in the medium and estrogens. Down-regulated by glucocorticoids. PTM: N-glycosylated. Contains sialic acid residues. PTM: The N-terminus is blocked. DISEASE: Defects in TNFRSF11B are the cause of juvenile Paget disease (JPD) [MIM:239000]; 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. SIMILARITY: Contains 2 death domains. SIMILARITY: Contains 4 TNFR-Cys repeats. WEB RESOURCE: Name=Atlas of Genetics and Cytogenetics in Oncology and Haematology; URL="http://atlasgeneticsoncology.org/Genes/TNFRSF11BID42610ch8q24.html"; WEB RESOURCE: Name=NIEHS-SNPs; URL="http://egp.gs.washington.edu/data/tnfrsf11b/"; GENE SYNONYMS:TNFRSF11B OCIF OPG. COPYRIGHT: Protein annotation is derived from the UniProt Consortium (http://www.uniprot.org/). Distributed under the Creative Commons Attribution-NoDerivs License.,
SEQUENCE 401 AA; 46026 MW; 2A23AC07BFA1E2DE CRC64;
|
| biopax3:xref |
urn:biopax:RelationshipXref:HGNC_HGNC:11909,
urn:biopax:RelationshipXref:NCBI GENE_4982,
urn:biopax:RelationshipXref:REFSEQ_NP_002537,
urn:biopax:UnificationXref:UNIPROT_B2R9A8,
urn:biopax:UnificationXref:UNIPROT_O00300,
urn:biopax:UnificationXref:UNIPROT_O60236,
urn:biopax:UnificationXref:UNIPROT_Q53FX6,
urn:biopax:UnificationXref:UNIPROT_Q9UHP4
|
| biopax3:displayName |
TR11B_HUMAN
|
| biopax3:name |
Osteoclastogenesis inhibitory factor,
Osteoprotegerin,
TNFRSF11B
|
| biopax3:organism | |
| biopax3:sequence |
MNNLLCCALVFLDISIKWTTQETFPPKYLHYDEETSHQLLCDKCPPGTYLKQHCTAKWKTVCAPCPDHYYTDSWHTSDECLYCSPVCKELQYVKQECNRTHNRVCECKEGRYLEIEFCLKHRSCPPGFGVVQAGTPERNTVCKRCPDGFFSNETSSKAPCRKHTNCSVFGLLLTQKGNATHDNICSGNSESTQKCGIDVTLCEEAFFRFAVPTKFTPNWLSVLVDNLPGTKVNAESVERIKRQHSSQEQTFQLLKLWKHQNKDQDIVKKIIQDIDLCENSVQRHIGHANLTFEQLRSLMESLPGKKVGAEDIEKTIKACKPSDQILKLLSLWRIKNGDQDTLKGLMHALKHSKTYHFPKTVTQSLKKTIRFLHSFTMYKLYQKLFLEMIGNQVQSVKISCL
|
| biopax3:standardName |
Tumor necrosis factor receptor superfamily member 11B
|