|CD267||TNFRSF13B (tumor necrosis factor receptor superfamily, member 13B), TACI|
|Molecule Type||Antigen Expression||Molecular Weight|
Min / Max
|Non-lineage Restricted Molecule|
Type 3 glycoprotein
|32 / 32|
|CD267 is expressed on myeloma cells and on B cells, predominantly CD27+ memory B cells. High expression is in spleen, thymus, small intestine and peripheral blood leukocytes. Expression is also in resting B cells and activated T cells but there are conflicting reports wheither activated T cells are expressed on CD267 and there is no expression in resting T cells. Expression is not found in heart, brain, placenta, lung, liver, skeletal muscle, kidney or pancreas. Expression has been detected on multiple myeloma cells and B cell chronic lymphocytic leukemia (B-CLL).|
|MOLECULAR FAMILY NAME: Belongs to the tumor necrosis factor receptor family.|
CD267 is a single-pass type-3 293 aa glycoprotein. It contains an 165 aa extracellular domain which contains 2 TNFR cysteine-rich repeats and a potential N-linked glycosylation site, a 21 aa transmembrane domain and an 107 aa cytoplasmic domain. The protein is a lymphocyte-specific member of the TNF receptor superfamily.
Alternative splicing yields 2 different isoforms. The shorter isoform lacks one TNFR-Cys repeat but has been shown to retain its ligand binding capability.
POST-TRANSLATIONAL MODIFICATION: No information.
|LIGANDS AND MOLECULE ASSOCIATED WITH CD267|
CD267 binds CD257 (TALL1) and CD256 (TALL2) with high affinity.
|CD267 is the receptor for CD257 (TALL1) and CD256 (TALL2). CD267 interacts with calcium-modulator and cyclophilin ligand (CAML). The protein induces activation of the transcription factors NFAT, AP1 and NF-κB and plays a crucial role in humoral immunity by interacting with a TNF ligand and is involved in the stimulation of T cell function. CD267-deficient mice have normal B-cell development but B cell homeostatis and tolerance is impaired resulting in autoimmunity and the development of lymphomas. Specific activation of CD267 in a human B cell line induced apoptosis suggesting that CD267 is a negative regulator of B cell activation. T-independent type 2 response is almost abolished in CD267-/- mice, indicating CD267 provides an essential costimulatory signal for T-independent humoral responses. Mutations in CD267 are associated with common variable immunodeficiency and IgA deficiency in humans. However in contrast to CD267-/- mice, lymphoroliferation and autoimmunity are not common features of CD267 deficiency in humans.|
BIOCHEMICAL ACTIVITY: No information:
DISEASE RELEVANCE AND FUNCTION OF CD267
Agonistic antibody to CD267 is a potential therapeutic agent in the treatment of B cell lymphoproliferative disorders. CD267-Ig is being trialed as a decoy receptor for BAFF in the treatment of B cell malignancies and autoimmune disorders. Defects in CD267 are a cause of common variable immunodeficiency (CVID) and immunoglobulin A deficiency (IGAG1). These diseases are known to coexist. Individuals having IGAD1 later can develop CVID suggesting that some of these cases may have a common etiology.
PROTEINS AND DNA ELEMENTS WHICH REGULATE TRANSCRIPTION OF CD267: No information.
SUBSTRATES: No information.
ENZYMES WHICH MODIFY CD267: No information.
For further information see Xia, X. Z. et al (2000) J, Exp. Med. 192: 137-143.
Database accession numbers
Revised June 25, 2008