1osx

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[[Image:1osx.png|left|200px]]
 
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{{STRUCTURE_1osx| PDB=1osx | SCENE= }}
{{STRUCTURE_1osx| PDB=1osx | SCENE= }}
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===Solution Structure of the Extracellular Domain of BLyS Receptor 3 (BR3)===
===Solution Structure of the Extracellular Domain of BLyS Receptor 3 (BR3)===
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{{ABSTRACT_PUBMED_12755599}}
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{{ABSTRACT_PUBMED_12755599}}
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==Disease==
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[[http://www.uniprot.org/uniprot/TR13C_HUMAN TR13C_HUMAN]] Defects in TNFRSF13C are the cause of immunodeficiency common variable type 4 (CVID4) [MIM:[http://omim.org/entry/613494 613494]]; also called antibody deficiency due to BAFFR defect. CVID4 is a primary immunodeficiency characterized by antibody deficiency, hypogammaglobulinemia, recurrent bacterial infections and an inability to mount an antibody response to antigen. The defect results from a failure of B-cell differentiation and impaired secretion of immunoglobulins; the numbers of circulating B-cells is usually in the normal range, but can be low.<ref>PMID:19666484</ref>
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==Function==
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[[http://www.uniprot.org/uniprot/TR13C_HUMAN TR13C_HUMAN]] B-cell receptor specific for TNFSF13B/TALL1/BAFF/BLyS. Promotes the survival of mature B-cells and the B-cell response.<ref>PMID:11591325</ref><ref>PMID:12387744</ref>
==About this Structure==
==About this Structure==
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==Reference==
==Reference==
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<ref group="xtra">PMID:012755599</ref><references group="xtra"/>
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<ref group="xtra">PMID:012755599</ref><references group="xtra"/><references/>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
[[Category: Cochran, A G.]]
[[Category: Cochran, A G.]]

Revision as of 20:21, 24 March 2013

Template:STRUCTURE 1osx

Contents

Solution Structure of the Extracellular Domain of BLyS Receptor 3 (BR3)

Template:ABSTRACT PUBMED 12755599

Disease

[TR13C_HUMAN] Defects in TNFRSF13C are the cause of immunodeficiency common variable type 4 (CVID4) [MIM:613494]; also called antibody deficiency due to BAFFR defect. CVID4 is a primary immunodeficiency characterized by antibody deficiency, hypogammaglobulinemia, recurrent bacterial infections and an inability to mount an antibody response to antigen. The defect results from a failure of B-cell differentiation and impaired secretion of immunoglobulins; the numbers of circulating B-cells is usually in the normal range, but can be low.[1]

Function

[TR13C_HUMAN] B-cell receptor specific for TNFSF13B/TALL1/BAFF/BLyS. Promotes the survival of mature B-cells and the B-cell response.[2][3]

About this Structure

1osx is a 1 chain structure with sequence from Homo sapiens. Full experimental information is available from OCA.

Reference

  • Gordon NC, Pan B, Hymowitz SG, Yin J, Kelley RF, Cochran AG, Yan M, Dixit VM, Fairbrother WJ, Starovasnik MA. BAFF/BLyS receptor 3 comprises a minimal TNF receptor-like module that encodes a highly focused ligand-binding site. Biochemistry. 2003 May 27;42(20):5977-83. PMID:12755599 doi:10.1021/bi034017g
  1. ↑ Warnatz K, Salzer U, Rizzi M, Fischer B, Gutenberger S, Bohm J, Kienzler AK, Pan-Hammarstrom Q, Hammarstrom L, Rakhmanov M, Schlesier M, Grimbacher B, Peter HH, Eibel H. B-cell activating factor receptor deficiency is associated with an adult-onset antibody deficiency syndrome in humans. Proc Natl Acad Sci U S A. 2009 Aug 18;106(33):13945-50. doi:, 10.1073/pnas.0903543106. Epub 2009 Aug 6. PMID:19666484 doi:10.1073/pnas.0903543106
  2. ↑ Yan M, Brady JR, Chan B, Lee WP, Hsu B, Harless S, Cancro M, Grewal IS, Dixit VM. Identification of a novel receptor for B lymphocyte stimulator that is mutated in a mouse strain with severe B cell deficiency. Curr Biol. 2001 Oct 2;11(19):1547-52. PMID:11591325
  3. ↑ Kayagaki N, Yan M, Seshasayee D, Wang H, Lee W, French DM, Grewal IS, Cochran AG, Gordon NC, Yin J, Starovasnik MA, Dixit VM. BAFF/BLyS receptor 3 binds the B cell survival factor BAFF ligand through a discrete surface loop and promotes processing of NF-kappaB2. Immunity. 2002 Oct;17(4):515-24. PMID:12387744

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