2ppi

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[[Image:2ppi.jpg|left|200px]]<br /><applet load="2ppi" size="350" color="white" frame="true" align="right" spinBox="true"
 
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caption="2ppi, resolution 2.40&Aring;" />
 
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'''Structure of the BTB (Tramtrack and Bric a brac) domain of human Gigaxonin'''<br />
 
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==About this Structure==
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==Structure of the BTB (Tramtrack and Bric a brac) domain of human Gigaxonin==
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2PPI is a [http://en.wikipedia.org/wiki/Single_protein 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=2PPI OCA].
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<StructureSection load='2ppi' size='340' side='right'caption='[[2ppi]], [[Resolution|resolution]] 2.40&Aring;' scene=''>
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[[Category: Homo sapiens]]
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== Structural highlights ==
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[[Category: Single protein]]
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<table><tr><td colspan='2'>[[2ppi]] is a 1 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=2PPI OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=2PPI FirstGlance]. <br>
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[[Category: Amos, A.]]
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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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[[Category: Arrowsmith, C.H.]]
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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=2ppi FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2ppi OCA], [https://pdbe.org/2ppi PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=2ppi RCSB], [https://www.ebi.ac.uk/pdbsum/2ppi PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=2ppi ProSAT]</span></td></tr>
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[[Category: Bullock, A.]]
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</table>
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[[Category: Burgess-Brown, N.]]
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== Disease ==
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[[Category: Debreczeni, J.E.]]
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[https://www.uniprot.org/uniprot/GAN_HUMAN GAN_HUMAN] Defects in GAN are the cause of giant axonal neuropathy (GAN) [MIM:[https://omim.org/entry/256850 256850]. GAN is a severe autosomal recessive sensorimotor neuropathy affecting both the peripheral nerves and the central nervous system. It is characterized by neurofilament accumulation, leading to segmental distention of axons.<ref>PMID:16303566</ref> <ref>PMID:11062483</ref> <ref>PMID:11971098</ref> <ref>PMID:12655563</ref> <ref>PMID:17578852</ref> <ref>PMID:17587580</ref>
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[[Category: Delft, F.von.]]
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== Function ==
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[[Category: Edwards, A.]]
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[https://www.uniprot.org/uniprot/GAN_HUMAN GAN_HUMAN] Probable cytoskeletal component that directly or indirectly plays an important role in neurofilament architecture. Substrate-specific adapter of an E3 ubiquitin-protein ligase complex which mediates the ubiquitination and subsequent proteasomal degradation of target proteins. Controls degradation of TBCB. Controls degradation of MAP1B and MAP1S, and is critical for neuronal maintenance and survival.<ref>PMID:12147674</ref> <ref>PMID:14528312</ref> <ref>PMID:16303566</ref> <ref>PMID:15983046</ref> <ref>PMID:16227972</ref>
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[[Category: Keates, T.]]
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== Evolutionary Conservation ==
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[[Category: Knapp, S.]]
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[[Image:Consurf_key_small.gif|200px|right]]
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[[Category: Papagrigoriou, E.]]
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Check<jmol>
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[[Category: Pike, A.C.W.]]
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<jmolCheckbox>
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[[Category: SGC, Structural.Genomics.Consortium.]]
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<scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/pp/2ppi_consurf.spt"</scriptWhenChecked>
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[[Category: Savitsky, P.]]
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<scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview01.spt</scriptWhenUnchecked>
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[[Category: Sundstrom, M.]]
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<text>to colour the structure by Evolutionary Conservation</text>
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[[Category: Tickle, J.]]
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</jmolCheckbox>
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[[Category: Turnbull, A.P.]]
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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=2ppi ConSurf].
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[[Category: Ugochukwu, E.]]
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<div style="clear:both"></div>
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[[Category: Umeano, C.]]
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[[Category: Weigelt, J.]]
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[[Category: btb domain]]
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[[Category: protein degradation]]
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[[Category: sgc]]
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[[Category: structural genomics]]
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[[Category: structural genomics consortium]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Wed Jan 23 15:22:57 2008''
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==See Also==
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*[[Kelch-like protein 3D structures|Kelch-like protein 3D structures]]
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== References ==
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<references/>
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__TOC__
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</StructureSection>
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[[Category: Homo sapiens]]
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[[Category: Large Structures]]
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[[Category: Amos A]]
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[[Category: Arrowsmith CH]]
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[[Category: Bullock A]]
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[[Category: Burgess-Brown N]]
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[[Category: Debreczeni JE]]
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[[Category: Edwards A]]
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[[Category: Keates T]]
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[[Category: Knapp S]]
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[[Category: Papagrigoriou E]]
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[[Category: Pike ACW]]
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[[Category: Savitsky P]]
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[[Category: Sundstrom M]]
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[[Category: Tickle J]]
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[[Category: Turnbull AP]]
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[[Category: Ugochukwu E]]
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[[Category: Umeano C]]
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[[Category: Weigelt J]]
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[[Category: Von Delft F]]

Current revision

Structure of the BTB (Tramtrack and Bric a brac) domain of human Gigaxonin

PDB ID 2ppi

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OCA

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