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FLASH GENE

Symbol

TNFRSF13B

contributors: mct/npt - updated : 20-09-2013

HGNC name	tumor necrosis factor receptor superfamily, member 13B
HGNC id	18153

FLASH GENE DNA RNA EXPRESSION PROTEIN DISORDER ANIMAL & CELL MODELS

Corresponding disease	CVID2 common variable immunodeficiency 2
Location	17p11.2      Physical location : 16.842.397 - 16.875.402
Synonym name	transmembrane activator and CAML interactor (calcium-modulator and cyclophilin ligand)
	CD267 antigen
Synonym symbol(s)	TACI, CD267, FLJ39942, MGC133214, MGC39952, TNFRSF14B

DNA

TYPE	functioning gene
STRUCTURE	33.03 kb     5 Exon(s)

10 Kb 5' upstream gene genomic sequence study

MAPPING

cloned

Y

linked

N

status

provisional

RNA

TRANSCRIPTS	type	messenger

identification nb exons type bp product Protein kDa AA specific expression Year Pubmed NM_012452 5 - 1377 NP_036584 31.7 293 - 2000 10880535

EXPRESSION

Type

expressed in	(based on citations)
organ(s)	System Organ level 1 Organ level 2 Organ level 3 Organ level 4 Level Pubmed Species Stage Rna symbol blood / hematopoietic spleen           thymus         Digestive intestine small intestine

tissue

System Tissue Tissue level 1 Tissue level 2 Level Pubmed Species Stage Rna symbol Blood / Hematopoietic         Lymphoid

cells

System Cell Pubmed Species Stage Rna symbol Blood/Hematopoietic leukocyte Lymphoid/Immune B cell

cell lineage
cell lines	jurkat T cells
fluid/secretion

at STAGE

PROTEIN

PHYSICAL PROPERTIES

STRUCTURE

motifs/domains	an extracellular domain with a cysteine rich TNFR repeat
	a transmembrane segment (TM1)
	intracellular domain interacting with TNFR-associated factor (TRAF)2, 5, and 6
	a cytoplasmic C terminus

HOMOLOGY

interspecies

homolog to murine TacI

Homologene

FAMILY

lymphocyte-specific member of the tumor necrosis factor (TNF) receptor superfamily

CATEGORY

signaling

SUBCELLULAR LOCALIZATION

plasma membrane

basic FUNCTION	playing a crucial role in humoral immunity by interacting with a TNF ligand
	key regulator of B cell function (Hymowitz 2005)
	its activation in a B cell line results in nuclear factor kappaB and c-Jun NH(2)-terminal kinase activation (Xia (2000)
	TRAF-interacting receptor for TNFSF13B, a tumor necrosis factor family member involved in B cell regulation (Xia 2000)
	triggers immunoglobulin class switching by activating B cells through the adaptor MYD88
	dual roles for TNFRSF13B in B-cell terminal differentiation
	has a dual role in B-cell terminal differentiation in limiting the expansion of GC B cells and mediating the survival of plasma cells
	is likely required for optimal T-cell–dependent humoral immune response
	is required for the survival of plasma cells and not for their differentiation

CELLULAR PROCESS

PHYSIOLOGICAL PROCESS

immunity/defense

text	humoral immune response

PATHWAY

metabolism

signaling

signal transduction

a component

INTERACTION

DNA

RNA

small molecule

protein	cyclophilin ligand protein CAML
	activator of NFAT
	TNFSF13, TNFSF13B (ligands of TNFRSF13)
	promotes sustained PRDM1 expression by B cells responding to antigen, which in turn limits B-cell clonal expansion and facilitates differentiation of long-lived antibody-secreting cells
	TNFRSF13B binds the cytokines TNFSF13B and ANP32B
	negatively regulates ICOSLG expression on B cells
	TNFRSF13B plays an important role in plasma cells and signals PRDM1 expression
	loss of TNFRSF13B leads to increased ICOSLG expression and expands T follicular helper (Tfh) and germinal center (GC) B cells
	requirement of an intact MYD88-binding site in TNFRSF13B to trigger class switch recombination (CSR)
	TNFRSF13B is released by ADAM10 and reflects B cell activation in autoimmunity

cell & other

REGULATION

ASSOCIATED DISORDERS

corresponding disease(s)

CVID2

Susceptibility

Variant & Polymorphism

Candidate gene
Marker
Therapy target	for B cell-mediated autoimmune diseases such as systemic lupus erythematosus (Xia 2000)

ANIMAL & CELL MODELS

	inactivation in mice results in a SLE-like phenotype (Salzer 2007)
	Taci-/- mice had increased numbers of B cells but exhibited decreased antibody responses to T-cell–independent type II antigens