1t77: Difference between revisions

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{{STRUCTURE_1t77|  PDB=1t77  |  SCENE=  }}
===Crystal structure of the PH-BEACH domains of human LRBA/BGL===
{{ABSTRACT_PUBMED_15554694}}


==Disease==
==Crystal structure of the PH-BEACH domains of human LRBA/BGL==
[[http://www.uniprot.org/uniprot/LRBA_HUMAN LRBA_HUMAN]] Defects in LRBA are the cause of immunodeficiency, common variable, type 8, with autoimmunity (CVID8) [MIM:[http://omim.org/entry/614700 614700]]. An autosomal recessive immunologic disorder associated with defective B-cell differentiation and decreased or absent antibody production. Affected individuals have early-childhood onset of recurrent infections, particularly respiratory infections, and also develop variable autoimmune disorders, including idiopathic thrombocytopenic purpura, autoimmune hemolytic anemia, and inflammatory bowel disease.<ref>PMID:22608502</ref>  
<StructureSection load='1t77' size='340' side='right'caption='[[1t77]], [[Resolution|resolution]] 2.40&Aring;' scene=''>
 
== Structural highlights ==
==Function==
<table><tr><td colspan='2'>[[1t77]] is a 4 chain structure with sequence from [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1T77 OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=1T77 FirstGlance]. <br>
[[http://www.uniprot.org/uniprot/LRBA_HUMAN LRBA_HUMAN]] May be involved in coupling signal transduction and vesicle trafficking to enable polarized secretion and/or membrane deposition of immune effector molecules (By similarity).  
</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">X-ray diffraction, [[Resolution|Resolution]] 2.4&#8491;</td></tr>
 
<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=1t77 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1t77 OCA], [https://pdbe.org/1t77 PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=1t77 RCSB], [https://www.ebi.ac.uk/pdbsum/1t77 PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=1t77 ProSAT]</span></td></tr>
==About this Structure==
</table>
[[1t77]] is a 4 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=1T77 OCA].  
== Disease ==
 
[https://www.uniprot.org/uniprot/LRBA_HUMAN LRBA_HUMAN] Defects in LRBA are the cause of immunodeficiency, common variable, type 8, with autoimmunity (CVID8) [MIM:[https://omim.org/entry/614700 614700]. An autosomal recessive immunologic disorder associated with defective B-cell differentiation and decreased or absent antibody production. Affected individuals have early-childhood onset of recurrent infections, particularly respiratory infections, and also develop variable autoimmune disorders, including idiopathic thrombocytopenic purpura, autoimmune hemolytic anemia, and inflammatory bowel disease.<ref>PMID:22608502</ref>  
==Reference==
== Function ==
<ref group="xtra">PMID:015554694</ref><references group="xtra"/><references/>
[https://www.uniprot.org/uniprot/LRBA_HUMAN LRBA_HUMAN] May be involved in coupling signal transduction and vesicle trafficking to enable polarized secretion and/or membrane deposition of immune effector molecules (By similarity).
== Evolutionary Conservation ==
[[Image:Consurf_key_small.gif|200px|right]]
Check<jmol>
  <jmolCheckbox>
    <scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/t7/1t77_consurf.spt"</scriptWhenChecked>
    <scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview01.spt</scriptWhenUnchecked>
    <text>to colour the structure by Evolutionary Conservation</text>
  </jmolCheckbox>
</jmol>, as determined by [http://consurfdb.tau.ac.il/ ConSurfDB]. You may read the [[Conservation%2C_Evolutionary|explanation]] of the method and the full data available from [http://bental.tau.ac.il/new_ConSurfDB/main_output.php?pdb_ID=1t77 ConSurf].
<div style="clear:both"></div>
== References ==
<references/>
__TOC__
</StructureSection>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
[[Category: Gebauer, D.]]
[[Category: Large Structures]]
[[Category: Jogl, G.]]
[[Category: Gebauer D]]
[[Category: Li, J.]]
[[Category: Jogl G]]
[[Category: Myszka, D G.]]
[[Category: Li J]]
[[Category: Shen, Y.]]
[[Category: Myszka DG]]
[[Category: Tong, L.]]
[[Category: Shen Y]]
[[Category: Ph-beach domain]]
[[Category: Tong L]]
[[Category: Signal transduction]]
[[Category: Signaling protein]]
[[Category: Vesicle trafficking]]
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