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tumor necrosis factor receptor superfamily member 13B; transmembrane activator & CAML interactor; CD267 (TNFRSF13B, TACI)
Function:
- receptor for TNFSF13/APRIL & TNFSF13B/TALL1/BAFF/BLYS
- binds both ligands with similar high affinity
- mediates calcineurin-dependent activation of NF-AT & activation of NF-kappa-B & AP-1
- role in stimulation of B-cell & T-cell function & in regulation of humoral immunity
- binds TRAF2, TRAF5 & TRAF6
- binds the NH2-terminal domain of CAMLG with its C-terminus
Structure: contains 2 TNFR-cys repeats
Compartment: membrane
Alternative splicing: named isoforms=2
Expression:
- highly expressed in spleen, thymus, small intestine & peripheral blood leukocytes
- expressed in resting B- cells & activated T-cells, but not in resting T-cells
Pathology:
- defects in TNFRSF13B are a cause of
a) common variable immunodeficiency
b) immunoglobulin A deficiency 2
Laboratory:
- TNFRSF13B gene mutation
Interactions
molecular events
General
glycoprotein
tumor necrosis factor [TNF] receptor family
Properties
SIZE: entity length = 293 aa
MW = 32 kD
COMPARTMENT: plasma membrane
MOTIF: TNFR-Cys {33-67}
MOTIF: cysteine residue {C34}
MODIFICATION: cysteine residue {C47}
cysteine residue {C47}
MODIFICATION: cysteine residue {C34}
cysteine residue {C50}
MODIFICATION: cysteine residue {C62}
cysteine residue {C54}
MODIFICATION: cysteine residue {C66}
cysteine residue {C62}
MODIFICATION: cysteine residue {C50}
cysteine residue {C66}
MODIFICATION: cysteine residue {C54}
TNFR-Cys {70-104}
MOTIF: cysteine residue {C71}
MODIFICATION: cysteine residue {C86}
cysteine residue {C86}
MODIFICATION: cysteine residue {C71}
cysteine residue {C89}
MODIFICATION: cysteine residue {C100}
cysteine residue {C93}
MODIFICATION: cysteine residue {C104}
cysteine residue {C100}
MODIFICATION: cysteine residue {C89}
cysteine residue {C104}
MODIFICATION: cysteine residue {C93}
N-glycosylation site {N128}
transmembrane domain {166-186}
Database Correlations
OMIM correlations
MORBIDMAP 604907
UniProt O14836
Pfam PF09305
Entrez Gene 23495
Kegg hsa:2349
References
- UniProt :accession O14836
- TNFRSF13Bbase; Note: TNFRSF13B mutation db
http://bioinf.uta.fi/TNFRSF13Bbase/
- GeneReviews
https://www.genecards.org/cgi-bin/carddisp.pl?gene=TNFRSF13B
Component-of
molecular complex