2vd6

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[[Image:2vd6.png|left|200px]]
 
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==Human adenylosuccinate lyase in complex with its substrate N6-(1,2- Dicarboxyethyl)-AMP, and its products AMP and fumarate.==
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The line below this paragraph, containing "STRUCTURE_2vd6", creates the "Structure Box" on the page.
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<StructureSection load='2vd6' size='340' side='right'caption='[[2vd6]], [[Resolution|resolution]] 2.00&Aring;' scene=''>
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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'>[[2vd6]] 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=2VD6 OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=2VD6 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]] 2&#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=2SA:2-[9-(3,4-DIHYDROXY-5-PHOSPHONOOXYMETHYL-TETRAHYDRO-FURAN-2-YL)-9H-PURIN-6-YLAMINO]-SUCCINIC+ACID'>2SA</scene>, <scene name='pdbligand=AMP:ADENOSINE+MONOPHOSPHATE'>AMP</scene>, <scene name='pdbligand=CL:CHLORIDE+ION'>CL</scene>, <scene name='pdbligand=FUM:FUMARIC+ACID'>FUM</scene>, <scene name='pdbligand=GOL:GLYCEROL'>GOL</scene></td></tr>
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{{STRUCTURE_2vd6| PDB=2vd6 | 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=2vd6 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2vd6 OCA], [https://pdbe.org/2vd6 PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=2vd6 RCSB], [https://www.ebi.ac.uk/pdbsum/2vd6 PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=2vd6 ProSAT]</span></td></tr>
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</table>
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== Disease ==
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[https://www.uniprot.org/uniprot/PUR8_HUMAN PUR8_HUMAN] Defects in ADSL are the cause of adenylosuccinase deficiency (ADSL deficiency) [MIM:[https://omim.org/entry/103050 103050]. ADSL deficiency is an autosomal recessive disorder characterized by the accumulation in the body fluids of succinylaminoimidazole-carboxamide riboside (SAICA-riboside) and succinyladenosine (S-Ado). Most children display marked psychomotor delay, often accompanied by epilepsy or autistic features, or both, although some patients may be less profoundly retarded. Occasionally, growth retardation and muscular wasting are also present.
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== Function ==
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[https://www.uniprot.org/uniprot/PUR8_HUMAN PUR8_HUMAN]
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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/vd/2vd6_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=2vd6 ConSurf].
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<div style="clear:both"></div>
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===HUMAN ADENYLOSUCCINATE LYASE IN COMPLEX WITH ITS SUBSTRATE N6-(1,2-DICARBOXYETHYL)-AMP, AND ITS PRODUCTS AMP AND FUMARATE.===
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==See Also==
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*[[Adenylosuccinate lyase 3D structures|Adenylosuccinate lyase 3D structures]]
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__TOC__
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</StructureSection>
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The line below this paragraph, {{ABSTRACT_PUBMED_018469177}}, adds the Publication Abstract to the page
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(as it appears on PubMed at http://www.pubmed.gov), where 018469177 is the PubMed ID number.
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{{ABSTRACT_PUBMED_018469177}}
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==About this Structure==
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[[2vd6]] 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=2VD6 OCA].
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==Reference==
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<ref group="xtra">PMID:018469177</ref><references group="xtra"/>
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[[Category: Adenylosuccinate lyase]]
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[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Arrowsmith, C.]]
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[[Category: Large Structures]]
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[[Category: Berg, S Van Den.]]
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[[Category: Arrowsmith C]]
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[[Category: Berglund, H.]]
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[[Category: Berglund H]]
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[[Category: Busam, R.]]
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[[Category: Busam R]]
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[[Category: Collins, R.]]
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[[Category: Collins R]]
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[[Category: Dahlgren, L G.]]
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[[Category: Dahlgren LG]]
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[[Category: Edwards, A.]]
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[[Category: Edwards A]]
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[[Category: Flodin, S.]]
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[[Category: Flodin S]]
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[[Category: Flores, A.]]
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[[Category: Flores A]]
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[[Category: Graslund, S.]]
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[[Category: Graslund S]]
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[[Category: Hallberg, B M.]]
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[[Category: Hallberg BM]]
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[[Category: Hammarstrom, M.]]
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[[Category: Hammarstrom M]]
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[[Category: Holmberg-Schiavone, L.]]
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[[Category: Holmberg-schiavone L]]
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[[Category: Johansson, I.]]
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[[Category: Johansson I]]
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[[Category: Kallas, A.]]
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[[Category: Kallas A]]
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[[Category: Karlberg, T.]]
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[[Category: Karlberg T]]
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[[Category: Kotenyova, T.]]
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[[Category: Kotenyova T]]
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[[Category: Lehtio, L.]]
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[[Category: Lehtio L]]
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[[Category: Moche, M.]]
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[[Category: Moche M]]
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[[Category: Nilsson, M.]]
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[[Category: Nilsson M]]
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[[Category: Nordlund, P.]]
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[[Category: Nordlund P]]
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[[Category: Nyman, T.]]
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[[Category: Nyman T]]
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[[Category: Ogg, D.]]
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[[Category: Ogg D]]
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[[Category: Persson, C.]]
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[[Category: Persson C]]
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[[Category: Sagemark, J.]]
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[[Category: Sagemark J]]
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[[Category: Sgc, Structural Genomics Consortium.]]
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[[Category: Stenmark P]]
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[[Category: Stenmark, P.]]
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[[Category: Sundstrom M]]
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[[Category: Sundstrom, M.]]
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[[Category: Thorsell AG]]
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[[Category: Thorsell, A G.]]
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[[Category: Tresaugues L]]
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[[Category: Tresaugues, L.]]
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[[Category: Weigelt J]]
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[[Category: Weigelt, J.]]
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[[Category: Welin M]]
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[[Category: Welin, M.]]
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[[Category: Van den Berg S]]
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[[Category: Disease mutation]]
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[[Category: Epilepsy]]
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[[Category: Lyase]]
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[[Category: Purine biosynthesis]]
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[[Category: Purine metabolism]]
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Current revision

Human adenylosuccinate lyase in complex with its substrate N6-(1,2- Dicarboxyethyl)-AMP, and its products AMP and fumarate.

PDB ID 2vd6

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OCA

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