1t77

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[[Image:1t77.gif|left|200px]]
 
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{{Structure
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==Crystal structure of the PH-BEACH domains of human LRBA/BGL==
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|PDB= 1t77 |SIZE=350|CAPTION= <scene name='initialview01'>1t77</scene>, resolution 2.40&Aring;
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<StructureSection load='1t77' size='340' side='right'caption='[[1t77]], [[Resolution|resolution]] 2.40&Aring;' scene=''>
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|SITE=
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== Structural highlights ==
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|LIGAND=
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<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>
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|ACTIVITY=
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</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>
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|GENE= LRBA, LBA, CDC4L, BGL ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 Homo sapiens])
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<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>
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}}
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</table>
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== Disease ==
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'''Crystal structure of the PH-BEACH domains of human LRBA/BGL'''
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[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>
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== Function ==
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[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).
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==Overview==
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== Evolutionary Conservation ==
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The beige and Chediak-Higashi syndrome (BEACH) domain defines a large family of eukaryotic proteins that have diverse cellular functions in vesicle trafficking, membrane dynamics, and receptor signaling. The domain is the only module that is highly conserved among all of these proteins, but the exact functions of this domain and the molecular basis for its actions are currently unknown. Our previous studies showed that the BEACH domain is preceded by a novel, weakly conserved pleckstrin homology (PH) domain. We report here the crystal structure at 2.4 A resolution of the PH-BEACH domain of human LRBA/BGL. The PH domain has the same backbone fold as canonical PH domains, despite sharing no sequence homology with them. However, our binding assays demonstrate that the PH domain in the BEACH proteins cannot bind phospholipids. The BEACH domain contains a core of several partially extended peptide segments that is flanked by helices on both sides. The structure suggests intimate association between the PH and the BEACH domains, and surface plasmon resonance studies confirm that the two domains of the protein FAN have high affinity for each other, with a K(d) of 120 nM.
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[[Image:Consurf_key_small.gif|200px|right]]
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Check<jmol>
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==About this Structure==
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<jmolCheckbox>
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1T77 is a [[Single protein]] structure of 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].
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<scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/t7/1t77_consurf.spt"</scriptWhenChecked>
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<scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview01.spt</scriptWhenUnchecked>
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==Reference==
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<text>to colour the structure by Evolutionary Conservation</text>
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Crystal structure of the PH-BEACH domains of human LRBA/BGL., Gebauer D, Li J, Jogl G, Shen Y, Myszka DG, Tong L, Biochemistry. 2004 Nov 30;43(47):14873-80. PMID:[http://www.ncbi.nlm.nih.gov/pubmed/15554694 15554694]
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</jmolCheckbox>
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</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].
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<div style="clear:both"></div>
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== References ==
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<references/>
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__TOC__
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</StructureSection>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Single protein]]
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[[Category: Large Structures]]
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[[Category: Gebauer, D.]]
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[[Category: Gebauer D]]
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[[Category: Jogl, G.]]
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[[Category: Jogl G]]
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[[Category: Li, J.]]
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[[Category: Li J]]
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[[Category: Myszka, D G.]]
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[[Category: Myszka DG]]
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[[Category: Shen, Y.]]
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[[Category: Shen Y]]
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[[Category: Tong, L.]]
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[[Category: Tong L]]
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[[Category: ph-beach domains; vesicle trafficking; signal transduction]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu Mar 20 14:14:44 2008''
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Current revision

Crystal structure of the PH-BEACH domains of human LRBA/BGL

PDB ID 1t77

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