3bh7

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[[Image:3bh7.jpg|left|200px]]
 
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==Crystal structure of the RP2-Arl3 complex bound to GDP-AlF4==
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The line below this paragraph, containing "STRUCTURE_3bh7", creates the "Structure Box" on the page.
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<StructureSection load='3bh7' size='340' side='right'caption='[[3bh7]], [[Resolution|resolution]] 1.90&Aring;' scene=''>
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You may change the PDB parameter (which sets the PDB file loaded into the applet)
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== Structural highlights ==
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or the SCENE parameter (which sets the initial scene displayed when the page is loaded),
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<table><tr><td colspan='2'>[[3bh7]] is a 2 chain structure with sequence from [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens] and [https://en.wikipedia.org/wiki/Mus_musculus Mus musculus]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=3BH7 OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=3BH7 FirstGlance]. <br>
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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]] 1.9&#8491;</td></tr>
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<tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=ALF:TETRAFLUOROALUMINATE+ION'>ALF</scene>, <scene name='pdbligand=GDP:GUANOSINE-5-DIPHOSPHATE'>GDP</scene>, <scene name='pdbligand=MG:MAGNESIUM+ION'>MG</scene></td></tr>
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{{STRUCTURE_3bh7| PDB=3bh7 | SCENE= }}
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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=3bh7 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3bh7 OCA], [https://pdbe.org/3bh7 PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=3bh7 RCSB], [https://www.ebi.ac.uk/pdbsum/3bh7 PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=3bh7 ProSAT]</span></td></tr>
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</table>
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== Function ==
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[https://www.uniprot.org/uniprot/ARL3_MOUSE ARL3_MOUSE] Small GTP-binding protein which cycles between an inactive GDP-bound and an active GTP-bound form, and the rate of cycling is regulated by guanine nucleotide exchange factors (GEF) and GTPase-activating proteins (GAP). Required for normal cytokinesis and cilia signaling. Required for targeting proteins such as NPHP3 to the ciliary membrane by releasing myristoylated NPHP3 from UNC119B cargo adapter into the cilium (By similarity). Requires assistance from GTPase-activating proteins (GAPs) like RP2 and PDE6D, in order to cycle between inactive GDP-bound and active GTP-bound forms.<ref>PMID:15979089</ref> <ref>PMID:18376416</ref>
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== Evolutionary Conservation ==
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[[Image:Consurf_key_small.gif|200px|right]]
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Check<jmol>
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<jmolCheckbox>
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<scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/bh/3bh7_consurf.spt"</scriptWhenChecked>
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<scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview01.spt</scriptWhenUnchecked>
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<text>to colour the structure by Evolutionary Conservation</text>
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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=3bh7 ConSurf].
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<div style="clear:both"></div>
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<div style="background-color:#fffaf0;">
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== Publication Abstract from PubMed ==
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The retinitis pigmentosa 2 (RP2) gene is responsible for a particular variant of X chromosome-linked eye disease. Previously, RP2 was shown to bind the GTP form of the small G protein Arf-like 3 (Arl3), thus qualifying as an effector. Here we present the Arl3-GppNHp-RP2 complex structure, which shows features resembling complexes with GTPase-activating proteins (GAPs). Biochemical analysis showing a 90,000-fold stimulation of the GTPase reaction together with the structure of an Arl3-GDP-AlF4--RP2 transition state complex showed that RP2 is an efficient GAP for Arl3, with structural features similar to other GAPs. Furthermore, the effect of mutations in patients with retinitis pigmentosa correlated with their effect on catalysis, in particular the mutation of the arginine finger of RP2. The cognate G protein-GAP pair is conserved in yeast as Cin4-Cin2, and the ability of RP2 to act as a GAP can be correlated with its ability to complement a CIN2-deletion phenotype.
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'''Crystal structure of the RP2-Arl3 complex bound to GDP-AlF4'''
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The retinitis pigmentosa 2 gene product is a GTPase-activating protein for Arf-like 3.,Veltel S, Gasper R, Eisenacher E, Wittinghofer A Nat Struct Mol Biol. 2008 Apr;15(4):373-80. Epub 2008 Mar 23. PMID:18376416<ref>PMID:18376416</ref>
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From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
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==Disease==
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</div>
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Known disease associated with this structure: Retinitis pigmentosa-2 OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=312600 312600]], Xp11.3 deletion syndrome OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=312600 312600]]
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<div class="pdbe-citations 3bh7" style="background-color:#fffaf0;"></div>
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== References ==
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==About this Structure==
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<references/>
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3BH7 is a [[Protein complex]] structure of sequences from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens] and [http://en.wikipedia.org/wiki/Mus_musculus Mus musculus]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=3BH7 OCA].
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__TOC__
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</StructureSection>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Large Structures]]
[[Category: Mus musculus]]
[[Category: Mus musculus]]
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[[Category: Protein complex]]
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[[Category: Gasper R]]
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[[Category: Gasper, R.]]
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[[Category: Veltel S]]
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[[Category: Veltel, S.]]
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[[Category: Wittinghofer A]]
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[[Category: Wittinghofer, A.]]
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[[Category: Disease mutation]]
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[[Category: Gtp-binding]]
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[[Category: Gtpase activating protein and gtpase]]
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[[Category: Lipoprotein]]
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[[Category: Membrane]]
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[[Category: Metal binding protein]]
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[[Category: Myristate]]
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[[Category: Nucleotide-binding]]
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[[Category: Palmitate]]
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[[Category: Phosphoprotein]]
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[[Category: Polymorphism]]
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[[Category: Protein-protein complex]]
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[[Category: Retinitis pigmentosa]]
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[[Category: Sensory transduction]]
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[[Category: Signaling protein]]
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[[Category: Vision]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Sun May 4 20:45:33 2008''
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Current revision

Crystal structure of the RP2-Arl3 complex bound to GDP-AlF4

PDB ID 3bh7

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