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2h32

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[[Image:2h32.png|left|200px]]
 
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{{STRUCTURE_2h32| PDB=2h32 | SCENE= }}
{{STRUCTURE_2h32| PDB=2h32 | SCENE= }}
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===Crystal structure of the pre-B cell receptor===
===Crystal structure of the pre-B cell receptor===
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{{ABSTRACT_PUBMED_17431183}}
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{{ABSTRACT_PUBMED_17431183}}
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==Disease==
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[[http://www.uniprot.org/uniprot/IGLL1_HUMAN IGLL1_HUMAN]] Defects in IGLL1 are the cause of agammaglobulinemia type 2 (AGM2) [MIM:[http://omim.org/entry/613500 613500]]. 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.
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==Function==
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[[http://www.uniprot.org/uniprot/VPREB_HUMAN VPREB_HUMAN]] Associates with the Ig-mu chain to form a molecular complex that is expressed on the surface of pre-B-cells. This complex presumably regulates Ig gene rearrangements in the early steps of B-cell differentiation. [[http://www.uniprot.org/uniprot/IGLL1_HUMAN IGLL1_HUMAN]] Critical for B-cell development.<ref>PMID:9419212</ref>
==About this Structure==
==About this Structure==
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==Reference==
==Reference==
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<ref group="xtra">PMID:017431183</ref><references group="xtra"/>
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<ref group="xtra">PMID:017431183</ref><references group="xtra"/><references/>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
[[Category: Bankovich, A J.]]
[[Category: Bankovich, A J.]]

Revision as of 18:24, 24 March 2013

Template:STRUCTURE 2h32

Contents

Crystal structure of the pre-B cell receptor

Template:ABSTRACT PUBMED 17431183

Disease

[IGLL1_HUMAN] Defects in IGLL1 are the cause of agammaglobulinemia type 2 (AGM2) [MIM:613500]. 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.

Function

[VPREB_HUMAN] Associates with the Ig-mu chain to form a molecular complex that is expressed on the surface of pre-B-cells. This complex presumably regulates Ig gene rearrangements in the early steps of B-cell differentiation. [IGLL1_HUMAN] Critical for B-cell development.[1]

About this Structure

2h32 is a 3 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA.

Reference

  • Bankovich AJ, Raunser S, Juo ZS, Walz T, Davis MM, Garcia KC. Structural insight into pre-B cell receptor function. Science. 2007 Apr 13;316(5822):291-4. PMID:17431183 doi:http://dx.doi.org/316/5822/291
  1. ↑ Minegishi Y, Coustan-Smith E, Wang YH, Cooper MD, Campana D, Conley ME. Mutations in the human lambda5/14.1 gene result in B cell deficiency and agammaglobulinemia. J Exp Med. 1998 Jan 5;187(1):71-7. PMID:9419212

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OCA

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