3kg5

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[[Image:3kg5.jpg|left|200px]]
 
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{{STRUCTURE_3kg5| PDB=3kg5 | SCENE= }}
{{STRUCTURE_3kg5| PDB=3kg5 | SCENE= }}
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===Crystal structure of human Ig-beta homodimer===
===Crystal structure of human Ig-beta homodimer===
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{{ABSTRACT_PUBMED_20696394}}
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==Disease==
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[[http://www.uniprot.org/uniprot/CD79B_HUMAN CD79B_HUMAN]] Autosomal agammaglobulinemia. Defects in CD79B are the cause of agammaglobulinemia type 6 (AGM6) [MIM:[http://omim.org/entry/612692 612692]]. It is a primary immunodeficiency characterized by profoundly low or absent serum antibodies and low or absent circulating B-cells due to an early block of B-cell development. Affected individuals develop severe infections in the first years of life.<ref>PMID:17675462</ref>
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==Function==
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[[http://www.uniprot.org/uniprot/CD79B_HUMAN CD79B_HUMAN]] Required in cooperation with CD79A for initiation of the signal transduction cascade activated by the B-cell antigen receptor complex (BCR) which leads to internalization of the complex, trafficking to late endosomes and antigen presentation. Enhances phosphorylation of CD79A, possibly by recruiting kinases which phosphorylate CD79A or by recruiting proteins which bind to CD79A and protect it from dephosphorylation.<ref>PMID:8617796</ref> <ref>PMID:9057631</ref> <ref>PMID:12097390</ref>
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(as it appears on PubMed at http://www.pubmed.gov), where 20696394 is the PubMed ID number.
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{{ABSTRACT_PUBMED_20696394}}
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==About this Structure==
==About this Structure==
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3KG5 is a 2 chains structure with sequences from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=3KG5 OCA].
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[[3kg5]] is a 2 chain structure with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=3KG5 OCA].
==Reference==
==Reference==
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<ref group="xtra">PMID:20696394</ref><references group="xtra"/>
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<ref group="xtra">PMID:020696394</ref><references group="xtra"/><references/>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
[[Category: Radaev, S.]]
[[Category: Radaev, S.]]
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[[Category: Immunoglobulin domain]]
[[Category: Immunoglobulin domain]]
[[Category: Protein binding]]
[[Category: Protein binding]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Wed Aug 25 08:55:52 2010''
 

Revision as of 02:44, 4 April 2013

Template:STRUCTURE 3kg5

Contents

Crystal structure of human Ig-beta homodimer

Template:ABSTRACT PUBMED 20696394

Disease

[CD79B_HUMAN] Autosomal agammaglobulinemia. Defects in CD79B are the cause of agammaglobulinemia type 6 (AGM6) [MIM:612692]. It is a primary immunodeficiency characterized by profoundly low or absent serum antibodies and low or absent circulating B-cells due to an early block of B-cell development. Affected individuals develop severe infections in the first years of life.[1]

Function

[CD79B_HUMAN] Required in cooperation with CD79A for initiation of the signal transduction cascade activated by the B-cell antigen receptor complex (BCR) which leads to internalization of the complex, trafficking to late endosomes and antigen presentation. Enhances phosphorylation of CD79A, possibly by recruiting kinases which phosphorylate CD79A or by recruiting proteins which bind to CD79A and protect it from dephosphorylation.[2] [3] [4]

About this Structure

3kg5 is a 2 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA.

Reference

  • Radaev S, Zou Z, Tolar P, Nguyen K, Nguyen A, Krueger PD, Stutzman N, Pierce S, Sun PD. Structural and functional studies of Igalphabeta and its assembly with the B cell antigen receptor. Structure. 2010 Aug 11;18(8):934-43. PMID:20696394 doi:10.1016/j.str.2010.04.019
  1. ↑ Dobbs AK, Yang T, Farmer D, Kager L, Parolini O, Conley ME. Cutting edge: a hypomorphic mutation in Igbeta (CD79b) in a patient with immunodeficiency and a leaky defect in B cell development. J Immunol. 2007 Aug 15;179(4):2055-9. PMID:17675462
  2. ↑ Luisiri P, Lee YJ, Eisfelder BJ, Clark MR. Cooperativity and segregation of function within the Ig-alpha/beta heterodimer of the B cell antigen receptor complex. J Biol Chem. 1996 Mar 1;271(9):5158-63. PMID:8617796
  3. ↑ Tseng J, Eisfelder BJ, Clark MR. B-cell antigen receptor-induced apoptosis requires both Ig alpha and Ig beta. Blood. 1997 Mar 1;89(5):1513-20. PMID:9057631
  4. ↑ Pelanda R, Braun U, Hobeika E, Nussenzweig MC, Reth M. B cell progenitors are arrested in maturation but have intact VDJ recombination in the absence of Ig-alpha and Ig-beta. J Immunol. 2002 Jul 15;169(2):865-72. PMID:12097390

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