1xu1

<StructureSection load='1xu1' size='340' side='right' caption='[[1xu1]], [[Resolution|resolution]] 1.90&Aring;' scene=''>

<table><tr><td colspan='2'>[[1xu1]] is a 6 chain structure with sequence from [http://en.wikipedia.org/wiki/Human Human] and [http://en.wikipedia.org/wiki/Lk3_transgenic_mice Lk3 transgenic mice]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1XU1 OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=1XU1 FirstGlance]. <br>

</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=NI:NICKEL+(II)+ION'>NI</scene></td></tr>

<tr id='related'><td class="sblockLbl"><b>[[Related_structure|Related:]]</b></td><td class="sblockDat">[[1u5x|1u5x]], [[1u5y|1u5y]], [[1u5z|1u5z]], [[1xu2|1xu2]], [[1xut|1xut]]</td></tr>

<tr id='gene'><td class="sblockLbl"><b>[[Gene|Gene:]]</b></td><td class="sblockDat">Tnfsf13, APRIL ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=10090 LK3 transgenic mice]), TNFRSF13B, TACI ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 HUMAN])</td></tr>

<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=1xu1 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1xu1 OCA], [http://pdbe.org/1xu1 PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=1xu1 RCSB], [http://www.ebi.ac.uk/pdbsum/1xu1 PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=1xu1 ProSAT]</span></td></tr>

== Disease ==

[[http://www.uniprot.org/uniprot/TR13B_HUMAN TR13B_HUMAN]] Defects in TNFRSF13B are the cause of immunodeficiency common variable type 2 (CVID2) [MIM:[http://omim.org/entry/240500 240500]]. CVID2 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:16007086</ref> Defects in TNFRSF13B are a cause of immunoglobulin A deficiency 2 (IGAD2) [MIM:[http://omim.org/entry/609529 609529]]. Selective deficiency of immunoglobulin A (IGAD) is the most common form of primary immunodeficiency, with an incidence of approximately 1 in 600 individuals in the western world. Individuals with symptomatic IGAD often have deficiency of IgG subclasses or decreased antibody response to carbohydrate antigens such as pneumococcal polysaccharide vaccine. Individuals with IGAD also suffer from recurrent sinopulmonary and gastrointestinal infections and have an increased incidence of autoimmune disorders and of lymphoid and non-lymphoid malignancies. In vitro studies have suggested that some individuals with IGAD have impaired isotype class switching to IgA and others may have a post-switch defect. IGAD and CVID have been known to coexist in families. Some individuals initially present with IGAD1 and then develop CVID. These observations suggest that some cases of IGAD and CVID may have a common etiology.<ref>PMID:16007086</ref>

[[http://www.uniprot.org/uniprot/TR13B_HUMAN TR13B_HUMAN]] Receptor for TNFSF13/APRIL and TNFSF13B/TALL1/BAFF/BLYS that binds both ligands with similar high affinity. Mediates calcineurin-dependent activation of NF-AT, as well as activation of NF-kappa-B and AP-1. Involved in the stimulation of B- and T-cell function and the regulation of humoral immunity.<ref>PMID:10956646</ref> <ref>PMID:10973284</ref>

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*[[Tumor necrosis factor ligand superfamily|Tumor necrosis factor ligand superfamily]]

*[[Tumor necrosis factor receptor|Tumor necrosis factor receptor]]

[[Category: Human]]

[[Category: Lk3 transgenic mice]]

[[Category: Gordon, N C]]

[[Category: Hymowitz, S G]]

[[Category: Kelley, R F]]

[[Category: Pan, B]]

[[Category: Patel, D R]]

[[Category: Runyon, S]]

[[Category: Shriver, S K]]

[[Category: Skelton, N J]]

[[Category: Starovasnik, M A]]

[[Category: Wallweber, H J.A]]

[[Category: Yan, M]]

[[Category: Yin, J]]

[[Category: Tnfsf]]