3bh7

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==Crystal structure of the RP2-Arl3 complex bound to GDP-AlF4==
==Crystal structure of the RP2-Arl3 complex bound to GDP-AlF4==
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<StructureSection load='3bh7' size='340' side='right' caption='[[3bh7]], [[Resolution|resolution]] 1.90&Aring;' scene=''>
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<StructureSection load='3bh7' size='340' side='right'caption='[[3bh7]], [[Resolution|resolution]] 1.90&Aring;' scene=''>
== Structural highlights ==
== Structural highlights ==
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<table><tr><td colspan='2'>[[3bh7]] is a 2 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=3BH7 OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=3BH7 FirstGlance]. <br>
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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><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><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><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><td class="sblockLbl"><b>[[Related_structure|Related:]]</b></td><td class="sblockDat">[[2bx6|2bx6]], [[3bh6|3bh6]]</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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<tr><td class="sblockLbl"><b>[[Gene|Gene:]]</b></td><td class="sblockDat">ARL3 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=10090 LK3 transgenic mice]), RP2 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 HUMAN])</td></tr>
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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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<tr><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=3bh7 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3bh7 OCA], [http://www.rcsb.org/pdb/explore.do?structureId=3bh7 RCSB], [http://www.ebi.ac.uk/pdbsum/3bh7 PDBsum]</span></td></tr>
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</table>
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<table>
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== Disease ==
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[[http://www.uniprot.org/uniprot/XRP2_HUMAN XRP2_HUMAN]] Defects in RP2 are the cause of retinitis pigmentosa type 2 (RP2) [MIM:[http://omim.org/entry/312600 312600]]; also known as X-linked retinitis pigmentosa 2 (XLRP-2). RP leads to degeneration of retinal photoreceptor cells. Patients typically have night vision blindness and loss of midperipheral visual field. As their condition progresses, they lose their far peripheral visual field and eventually central vision as well.<ref>PMID:11847227</ref> <ref>PMID:16472755</ref> <ref>PMID:10942419</ref> <ref>PMID:9697692</ref> <ref>PMID:10090907</ref> <ref>PMID:10520237</ref> <ref>PMID:10634633</ref> <ref>PMID:10937588</ref> <ref>PMID:11462235</ref> <ref>PMID:11992260</ref> <ref>PMID:14564670</ref> <ref>PMID:12657579</ref> <ref>PMID:22334370</ref>
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== Function ==
== Function ==
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[[http://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> [[http://www.uniprot.org/uniprot/XRP2_HUMAN XRP2_HUMAN]] Acts as a GTPase-activating protein (GAP) involved in trafficking between the Golgi and the ciliary membrane. Involved in localization of proteins, such as NPHP3, to the cilium membrane by inducing hydrolysis of GTP ARL3, leading to the release of UNC119 (or UNC119B). Acts as a GTPase-activating protein (GAP) for tubulin in concert with tubulin-specific chaperone C, but does not enhance tubulin heterodimerization. Acts as guanine nucleotide dissociation inhibitor towards ADP-ribosylation factor-like proteins.<ref>PMID:11847227</ref> <ref>PMID:20106869</ref> <ref>PMID:22085962</ref> <ref>PMID:18376416</ref>
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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>
== Evolutionary Conservation ==
== Evolutionary Conservation ==
[[Image:Consurf_key_small.gif|200px|right]]
[[Image:Consurf_key_small.gif|200px|right]]
Check<jmol>
Check<jmol>
<jmolCheckbox>
<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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<scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/bh/3bh7_consurf.spt"</scriptWhenChecked>
<scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview01.spt</scriptWhenUnchecked>
<scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview01.spt</scriptWhenUnchecked>
<text>to colour the structure by Evolutionary Conservation</text>
<text>to colour the structure by Evolutionary Conservation</text>
</jmolCheckbox>
</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/chain_selection.php?pdb_ID=2ata ConSurf].
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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].
<div style="clear:both"></div>
<div style="clear:both"></div>
<div style="background-color:#fffaf0;">
<div style="background-color:#fffaf0;">
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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>
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®/PubMed®, a database of the U.S. National Library of Medicine.<br>
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From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
</div>
</div>
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<div class="pdbe-citations 3bh7" style="background-color:#fffaf0;"></div>
== References ==
== References ==
<references/>
<references/>
__TOC__
__TOC__
</StructureSection>
</StructureSection>
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[[Category: Human]]
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[[Category: Homo sapiens]]
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[[Category: Lk3 transgenic mice]]
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[[Category: Large Structures]]
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[[Category: Gasper, R.]]
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[[Category: Mus musculus]]
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[[Category: Veltel, S.]]
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[[Category: Gasper R]]
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[[Category: Wittinghofer, A.]]
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[[Category: Veltel S]]
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[[Category: Disease mutation]]
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[[Category: Wittinghofer A]]
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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: 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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Current revision

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

PDB ID 3bh7

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