2h32: Difference between revisions
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{{STRUCTURE_2h32| PDB=2h32 | SCENE= }} | {{STRUCTURE_2h32| PDB=2h32 | SCENE= }} | ||
===Crystal structure of the pre-B cell receptor=== | |||
{{ABSTRACT_PUBMED_17431183}} | |||
=== | ==Disease== | ||
[[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. | |||
==Function== | |||
[[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== | ||
<ref group="xtra">PMID:017431183</ref><references group="xtra"/> | <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
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:https://dx.doi.org/316/5822/291
