BAFF receptor

TNFRSF13C
Available structures
PDB	Ortholog search: PDBe RCSB
List of PDB id codes
	1MPV, 1OQE, 1OSX, 2HFG, 3V56, 4V46
Identifiers
Aliases	TNFRSF13C, BAFF-R, BAFFR, BROMIX, CD268, CVID4, prolixin, tumor necrosis factor receptor superfamily member 13C, TNF receptor superfamily member 13C
External IDs	OMIM: 606269 MGI: 1919299 HomoloGene: 49897 GeneCards: TNFRSF13C
Gene location (Human)
Chr.	Chromosome 22 (human)
End	41,926,806 bp
Gene location (Mouse)
Chr.	Chromosome 15 (mouse)
End	82,108,570 bp
RNA expression pattern
	Top expressed in
	spleen; ; lymph node; ; appendix; ; bone marrow cells; ; blood; ; thymus; ; ganglionic eminence; ; skin of abdomen; ; rectum; ; gallbladder;
	Top expressed in
	spleen; ; blood; ; submandibular gland; ; subcutaneous adipose tissue; ; morula; ; white adipose tissue; ; spermatid; ; bone marrow; ; Paneth cell; ; hair follicle;
	More reference expression data
	n/a
Orthologs
	115650
	72049
	ENSG00000159958
	ENSMUSG00000068105
	Q96RJ3
	Q9D8D0
	NM_052945
	NM_028075; NM_001357758
	NP_443177
	NP_082351; NP_001344687
	Wikidata
View/Edit Human	View/Edit Mouse

BAFF receptor (B-cell activating factor receptor, BAFF-R), also known as tumor necrosis factor receptor superfamily member 13C (TNFRSF13C) and BLyS receptor 3 (BR3), is a membrane protein of the TNF receptor superfamily which recognizes BAFF, an essential factor for B cell maturation and survival.^[5]^[6] In humans it is encoded by the TNFRSF13C gene.^[7]

Function

B-cell activating factor (BAFF) enhances B-cell survival in vitro and is a regulator of the peripheral B-cell population. The protein encoded by this gene is a receptor for BAFF and is a type III transmembrane protein containing a single extracellular phenylalanine-rich domain. It is thought that this receptor is the principal receptor required for BAFF-mediated mature B-cell survival.^[7] In B cell maturation, due to regulation by BAFF-R, only a limited amount of B-cell will survive.^[8]

Clinical significance

Overexpression of BAFF in mice results in mature B-cell hyperplasia and symptoms of systemic lupus erythematosus (SLE). Also, some SLE patients have increased levels of BAFF in serum. Therefore, it has been proposed that abnormally high levels of BAFF may contribute to the pathogenesis of autoimmune diseases by enhancing the survival of autoreactive B cells, which are cells that show immune response to normal body cells.^[7] Autoreactive B cells are less sensitive toward BAFF and are usually outcompeted by the normal B cells in the maturation process regulated by low BAFF-R expression. An elevated level of BAFF-R can therefore overcome this decreased response and result in accumulation of autoreactive B cells.^[8]

BAFF and BAFF-R pair can also down-regulate the cell apoptosis process.^[9]

References

1 2 3 GRCh38: Ensembl release 89: ENSG00000159958 - Ensembl, May 2017
1 2 3 GRCm38: Ensembl release 89: ENSMUSG00000068105 - Ensembl, May 2017
↑ "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
↑ "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
↑ Thompson JS, Bixler SA, Qian F, Vora K, Scott ML, Cachero TG, Hession C, Schneider P, Sizing ID, Mullen C, Strauch K, Zafari M, Benjamin CD, Tschopp J, Browning JL, Ambrose C (September 2001). "BAFF-R, a newly identified TNF receptor that specifically interacts with BAFF". Science. 293 (5537): 2108–11. Bibcode:2001Sci...293.2108T. doi:10.1126/science.1061965. PMID 11509692. S2CID 42673198.
↑ Yan M, Brady JR, Chan B, Lee WP, Hsu B, Harless S, Cancro M, Grewal IS, Dixit VM (October 2001). "Identification of a novel receptor for B lymphocyte stimulator that is mutated in a mouse strain with severe B cell deficiency". Current Biology. 11 (19): 1547–52. doi:10.1016/S0960-9822(01)00481-X. PMID 11591325. S2CID 13925100.
1 2 3 "Entrez Gene: TNFRSF13C tumor necrosis factor receptor superfamily, member 13C".
1 2 Brink R (October 2006). "Regulation of B cell self-tolerance by BAFF". Seminars in Immunology. 18 (5): 276–83. doi:10.1016/j.smim.2006.04.003. PMID 16916609.
↑ Rauch M, Tussiwand R, Bosco N, Rolink AG (2009-05-06). "Crucial role for BAFF-BAFF-R signaling in the survival and maintenance of mature B cells". PLOS ONE. 4 (5): e5456. Bibcode:2009PLoSO...4.5456R. doi:10.1371/journal.pone.0005456. PMC 2673681. PMID 19421318.

External links

Human TNFRSF13C genome location and TNFRSF13C gene details page in the UCSC Genome Browser.

Laâbi Y, Egle A, Strasser A (December 2001). "TNF cytokine family: more BAFF-ling complexities". Current Biology. 11 (24): R1013-6. doi:10.1016/S0960-9822(01)00613-3. PMID 11747837. S2CID 17001236.
Defrance T, Casamayor-Pallejà M, Krammer PH (2003). The life and death of a B cell. Vol. 86. pp. 195–225. doi:10.1016/S0065-230X(02)86006-7. ISBN 9780120066865. PMID 12374279. {{cite book}}: |journal= ignored (help)
Ambrose CM (2003). "BAFF-R". Journal of Biological Regulators and Homeostatic Agents. 16 (3): 211–3. PMID 12456020.
Bossen C, Schneider P (October 2006). "BAFF, APRIL and their receptors: structure, function and signaling" (PDF). Seminars in Immunology. 18 (5): 263–75. doi:10.1016/j.smim.2006.04.006. PMID 16914324.
Treml LS, Crowley JE, Cancro MP (October 2006). "BLyS receptor signatures resolve homeostatically independent compartments among naïve and antigen-experienced B cells". Seminars in Immunology. 18 (5): 297–304. doi:10.1016/j.smim.2006.07.001. PMID 16919470.
Kalled SL (October 2006). "Impact of the BAFF/BR3 axis on B cell survival, germinal center maintenance and antibody production". Seminars in Immunology. 18 (5): 290–6. doi:10.1016/j.smim.2006.06.002. PMID 16931038.
Mackay F, Leung H (October 2006). "The role of the BAFF/APRIL system on T cell function". Seminars in Immunology. 18 (5): 284–9. doi:10.1016/j.smim.2006.04.005. PMID 16931039.
Schneider P, MacKay F, Steiner V, Hofmann K, Bodmer JL, Holler N, Ambrose C, Lawton P, Bixler S, Acha-Orbea H, Valmori D, Romero P, Werner-Favre C, Zubler RH, Browning JL, Tschopp J (June 1999). "BAFF, a novel ligand of the tumor necrosis factor family, stimulates B cell growth". The Journal of Experimental Medicine. 189 (11): 1747–56. doi:10.1084/jem.189.11.1747. PMC 2193079. PMID 10359578.
Hartley JL, Temple GF, Brasch MA (November 2000). "DNA cloning using in vitro site-specific recombination". Genome Research. 10 (11): 1788–95. doi:10.1101/gr.143000. PMC 310948. PMID 11076863.
Yan M, Brady JR, Chan B, Lee WP, Hsu B, Harless S, Cancro M, Grewal IS, Dixit VM (October 2001). "Identification of a novel receptor for B lymphocyte stimulator that is mutated in a mouse strain with severe B cell deficiency". Current Biology. 11 (19): 1547–52. doi:10.1016/S0960-9822(01)00481-X. PMID 11591325. S2CID 13925100.
Rolink AG, Melchers F (April 2002). "BAFFled B cells survive and thrive: roles of BAFF in B-cell development". Current Opinion in Immunology. 14 (2): 266–75. doi:10.1016/S0952-7915(02)00332-1. PMID 11869903.
Xu LG, Shu HB (December 2002). "TNFR-associated factor-3 is associated with BAFF-R and negatively regulates BAFF-R-mediated NF-kappa B activation and IL-10 production". Journal of Immunology. 169 (12): 6883–9. doi:10.4049/jimmunol.169.12.6883. PMID 12471121.
Kim HM, Yu KS, Lee ME, Shin DR, Kim YS, Paik SG, Yoo OJ, Lee H, Lee JO (May 2003). "Crystal structure of the BAFF-BAFF-R complex and its implications for receptor activation". Nature Structural Biology. 10 (5): 342–8. doi:10.1038/nsb925. hdl:10203/11495. PMID 12715002. S2CID 11294267.
Liu Y, Hong X, Kappler J, Jiang L, Zhang R, Xu L, Pan CH, Martin WE, Murphy RC, Shu HB, Dai S, Zhang G (May 2003). "Ligand-receptor binding revealed by the TNF family member TALL-1". Nature. 423 (6935): 49–56. Bibcode:2003Natur.423...49L. doi:10.1038/nature01543. PMID 12721620. S2CID 4373708.
Gordon NC, Pan B, Hymowitz SG, Yin J, Kelley RF, Cochran AG, Yan M, Dixit VM, Fairbrother WJ, Starovasnik MA (May 2003). "BAFF/BLyS receptor 3 comprises a minimal TNF receptor-like module that encodes a highly focused ligand-binding site". Biochemistry. 42 (20): 5977–83. doi:10.1021/bi034017g. PMID 12755599.

This article incorporates text from the United States National Library of Medicine, which is in the public domain.

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[refGRCh38Ensembl-1] 1 2 3 GRCh38: Ensembl release 89: ENSG00000159958 - Ensembl, May 2017

[refGRCm38Ensembl-2] 1 2 3 GRCm38: Ensembl release 89: ENSMUSG00000068105 - Ensembl, May 2017

[3] "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.

[4] "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.

[pmid11509692-5] Thompson JS, Bixler SA, Qian F, Vora K, Scott ML, Cachero TG, Hession C, Schneider P, Sizing ID, Mullen C, Strauch K, Zafari M, Benjamin CD, Tschopp J, Browning JL, Ambrose C (September 2001). "BAFF-R, a newly identified TNF receptor that specifically interacts with BAFF". Science. 293 (5537): 2108–11. Bibcode:2001Sci...293.2108T. doi:10.1126/science.1061965. PMID 11509692. S2CID 42673198.

[6] Yan M, Brady JR, Chan B, Lee WP, Hsu B, Harless S, Cancro M, Grewal IS, Dixit VM (October 2001). "Identification of a novel receptor for B lymphocyte stimulator that is mutated in a mouse strain with severe B cell deficiency". Current Biology. 11 (19): 1547–52. doi:10.1016/S0960-9822(01)00481-X. PMID 11591325. S2CID 13925100.

[entrez-7] 1 2 3 "Entrez Gene: TNFRSF13C tumor necrosis factor receptor superfamily, member 13C".

[:0-8] 1 2 Brink R (October 2006). "Regulation of B cell self-tolerance by BAFF". Seminars in Immunology. 18 (5): 276–83. doi:10.1016/j.smim.2006.04.003. PMID 16916609.

[9] Rauch M, Tussiwand R, Bosco N, Rolink AG (2009-05-06). "Crucial role for BAFF-BAFF-R signaling in the survival and maintenance of mature B cells". PLOS ONE. 4 (5): e5456. Bibcode:2009PLoSO...4.5456R. doi:10.1371/journal.pone.0005456. PMC 2673681. PMID 19421318.

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Proteins: clusters of differentiation (see also list of human clusters of differentiation)
1–50	CD1 a-c 1A 1B 1D 1E CD2 CD3 γ δ ε CD4 CD5 CD6 CD7 CD8 a CD9 CD10 CD11 a b c d CD13 CD14 CD15 CD16 A B CD18 CD19 CD20 CD21 CD22 CD23 CD24 CD25 CD26 CD27 CD28 CD29 CD30 CD31 CD32 A B CD33 CD34 CD35 CD36 CD37 CD38 CD39 CD40 CD41 CD42 a b c d CD43 CD44 CD45 CD46 CD47 CD48 CD49 a b c d e f CD50
51–100	CD51 CD52 CD53 CD54 CD55 CD56 CD57 CD58 CD59 CD61 CD62 E L P CD63 CD64 A B C CD66 a b c d e f CD68 CD69 CD70 CD71 CD72 CD73 CD74 CD78 CD79 a b CD80 CD81 CD82 CD83 CD84 CD85 a d e h j k CD86 CD87 CD88 CD89 CD90 CD91 - CD92 CD93 CD94 CD95 CD96 CD97 CD98 CD99 CD100
101–150	CD101 CD102 CD103 CD104 CD105 CD106 CD107 a b CD108 CD109 CD110 CD111 CD112 CD113 CD114 CD115 CD116 CD117 CD118 CD119 CD120 a b CD121 a b CD122 CD123 CD124 CD125 CD126 CD127 CD129 CD130 CD131 CD132 CD133 CD134 CD135 CD136 CD137 CD138 CD140b CD141 CD142 CD143 CD144 CD146 CD147 CD148 CD150
151–200	CD151 CD152 CD153 CD154 CD155 CD156 a b c CD157 CD158 (a d e i k) CD159 a c CD160 CD161 CD162 CD163 CD164 CD166 CD167 a b CD168 CD169 CD170 CD171 CD172 a b g CD174 CD177 CD178 CD179 a b CD180 CD181 CD182 CD183 CD184 CD185 CD186 CD191 CD192 CD193 CD194 CD195 CD196 CD197 CDw198 CDw199 CD200
201–250	CD201 CD202b CD204 CD205 CD206 CD207 CD208 CD209 CDw210 a b CD212 CD213a 1 2 CD217 CD218 (a b) CD220 CD221 CD222 CD223 CD224 CD225 CD226 CD227 CD228 CD229 CD230 CD233 CD234 CD235 a b CD236 CD238 CD239 CD240CE CD240D CD241 CD243 CD244 CD246 CD247 - CD248 CD249
251–300	CD252 CD253 CD254 CD256 CD257 CD258 CD261 CD262 CD263 CD264 CD265 CD266 CD267 CD268 CD269 CD271 CD272 CD273 CD274 CD275 CD276 CD278 CD279 CD280 CD281 CD282 CD283 CD284 CD286 CD288 CD289 CD290 CD292 CDw293 CD294 CD295 CD297 CD298 CD299
301–350	CD300A CD301 CD302 CD303 CD304 CD305 CD306 CD307 CD309 CD312 CD314 CD315 CD316 CD317 CD318 CD320 CD321 CD322 CD324 CD325 CD326 CD327 CD328 CD329 CD331 CD332 CD333 CD334 CD335 CD336 CD337 CD338 CD339 CD340 CD344 CD349 CD350

Function

Clinical significance

See also

References

External links

Further reading