2j91

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[[Image:2j91.gif|left|200px]]<br /><applet load="2j91" size="350" color="white" frame="true" align="right" spinBox="true"
 
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caption="2j91, resolution 1.80&Aring;" />
 
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'''CRYSTAL STRUCTURE OF HUMAN ADENYLOSUCCINATE LYASE IN COMPLEX WITH AMP'''<br />
 
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==Disease==
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==Crystal structure of Human Adenylosuccinate Lyase in complex with AMP==
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Known disease associated with this structure: Adenylosuccinase deficiency OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=608222 608222]]
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<StructureSection load='2j91' size='340' side='right'caption='[[2j91]], [[Resolution|resolution]] 1.80&Aring;' scene=''>
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== Structural highlights ==
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<table><tr><td colspan='2'>[[2j91]] is a 4 chain structure with sequence from [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. This structure supersedes the now removed PDB entry [http://oca.weizmann.ac.il/oca-bin/send-pdb?obs=1&id=2j84 2j84]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2J91 OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=2J91 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]] 1.8&#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=AMP:ADENOSINE+MONOPHOSPHATE'>AMP</scene>, <scene name='pdbligand=CL:CHLORIDE+ION'>CL</scene>, <scene name='pdbligand=GOL:GLYCEROL'>GOL</scene></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=2j91 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2j91 OCA], [https://pdbe.org/2j91 PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=2j91 RCSB], [https://www.ebi.ac.uk/pdbsum/2j91 PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=2j91 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/j9/2j91_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=2j91 ConSurf].
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<div style="clear:both"></div>
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==About this Structure==
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==See Also==
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2J91 is a [http://en.wikipedia.org/wiki/Single_protein Single protein] structure of sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens] with <scene name='pdbligand=CL:'>CL</scene>, <scene name='pdbligand=AMP:'>AMP</scene> and <scene name='pdbligand=GOL:'>GOL</scene> as [http://en.wikipedia.org/wiki/ligands ligands]. This structure supersedes the now removed PDB entry 2J84. Active as [http://en.wikipedia.org/wiki/Adenylosuccinate_lyase Adenylosuccinate lyase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=4.3.2.2 4.3.2.2] Known structural/functional Site: <scene name='pdbsite=AC1:Gol+Binding+Site+For+Chain+D'>AC1</scene>. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2J91 OCA].
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*[[Adenylosuccinate lyase 3D structures|Adenylosuccinate lyase 3D structures]]
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[[Category: Adenylosuccinate lyase]]
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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: Arrowsmith, C.]]
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[[Category: Arrowsmith C]]
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[[Category: Berg, S Van Den.]]
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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: Edwards A]]
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[[Category: Edwards, A.]]
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[[Category: Ericsson UB]]
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[[Category: Ericsson, U B.]]
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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 BM]]
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[[Category: Hallberg, B M.]]
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[[Category: Hammarstrom M]]
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[[Category: Hammarstrom, M.]]
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[[Category: Hogbom M]]
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[[Category: Hogbom, M.]]
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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: Karlberg, T.]]
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[[Category: Karlberg T]]
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[[Category: Kosinska, U.]]
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[[Category: Kosinska U]]
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[[Category: Kotenyova, T.]]
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[[Category: Kotenyova T]]
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[[Category: Magnusdottir, A.]]
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[[Category: Magnusdottir A]]
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[[Category: Moche, M.]]
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[[Category: Moche M]]
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[[Category: Nilsson, M E.]]
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[[Category: Nilsson ME]]
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[[Category: Nilsson-Ehle, P.]]
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[[Category: Nilsson-Ehle P]]
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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: Schiavone, L Holmberg.]]
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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: Uppenberg J]]
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[[Category: Uppenberg, J.]]
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[[Category: Uppsten M]]
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[[Category: Uppsten, M.]]
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[[Category: Wallden K]]
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[[Category: Wallden, K.]]
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[[Category: Weigelt J]]
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[[Category: Weigelt, J.]]
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[[Category: Van Den Berg S]]
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[[Category: AMP]]
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[[Category: CL]]
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[[Category: GOL]]
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[[Category: adenylosuccinase]]
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[[Category: adenylosuccinase deficiency]]
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[[Category: adenylosuccinate lyase]]
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[[Category: adsl]]
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[[Category: alternative splicing]]
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[[Category: amp]]
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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]]
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[[Category: purine biosynthesis]]
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[[Category: s-amp]]
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[[Category: saicar]]
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[[Category: succino amp-lyase]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu Feb 21 18:00:35 2008''
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Current revision

Crystal structure of Human Adenylosuccinate Lyase in complex with AMP

PDB ID 2j91

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