2h3n: Difference between revisions
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{{STRUCTURE_2h3n| PDB=2h3n | SCENE= }} | {{STRUCTURE_2h3n| PDB=2h3n | SCENE= }} | ||
===Crystal structure of a surrogate light chain (LAMBDA5 and VpreB) homodimer=== | |||
{{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.]] | ||
