1z6z

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[[Image:1z6z.gif|left|200px]]<br /><applet load="1z6z" size="350" color="white" frame="true" align="right" spinBox="true"
 
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caption="1z6z, resolution 2.50&Aring;" />
 
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'''Crystal Structure of Human Sepiapterin Reductase in complex with NADP+'''<br />
 
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==About this Structure==
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==Crystal Structure of Human Sepiapterin Reductase in complex with NADP+==
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1Z6Z 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=SO4:'>SO4</scene>, <scene name='pdbligand=CL:'>CL</scene> and <scene name='pdbligand=NAP:'>NAP</scene> as [http://en.wikipedia.org/wiki/ligands ligands]. Active as [http://en.wikipedia.org/wiki/Sepiapterin_reductase Sepiapterin reductase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=1.1.1.153 1.1.1.153] Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1Z6Z OCA].
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<StructureSection load='1z6z' size='340' side='right'caption='[[1z6z]], [[Resolution|resolution]] 2.50&Aring;' scene=''>
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== Structural highlights ==
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<table><tr><td colspan='2'>[[1z6z]] is a 6 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=1Z6Z OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=1Z6Z 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]] 2.5&#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=CL:CHLORIDE+ION'>CL</scene>, <scene name='pdbligand=NAP:NADP+NICOTINAMIDE-ADENINE-DINUCLEOTIDE+PHOSPHATE'>NAP</scene>, <scene name='pdbligand=SO4:SULFATE+ION'>SO4</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=1z6z FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1z6z OCA], [https://pdbe.org/1z6z PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=1z6z RCSB], [https://www.ebi.ac.uk/pdbsum/1z6z PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=1z6z ProSAT]</span></td></tr>
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</table>
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== Disease ==
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[https://www.uniprot.org/uniprot/SPRE_HUMAN SPRE_HUMAN] Defects in SPR are the cause of dystonia DOPA-responsive due to sepiapterin reductase deficiency (DRDSPRD) [MIM:[https://omim.org/entry/612716 612716]. In the majority of cases, patients manifest progressive psychomotor retardation, dystonia and spasticity. Cognitive anomalies are also often present. The disease is due to severe dopamine and serotonin deficiencies in the central nervous system caused by a defect in BH4 synthesis. Dystonia is defined by the presence of sustained involuntary muscle contractions, often leading to abnormal postures.<ref>PMID:11443547</ref> <ref>PMID:16650784</ref> <ref>PMID:17159114</ref>
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== Function ==
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[https://www.uniprot.org/uniprot/SPRE_HUMAN SPRE_HUMAN] Catalyzes the final one or two reductions in tetra-hydrobiopterin biosynthesis to form 5,6,7,8-tetrahydrobiopterin.
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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/z6/1z6z_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=1z6z ConSurf].
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<div style="clear:both"></div>
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== References ==
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<references/>
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__TOC__
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</StructureSection>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Sepiapterin reductase]]
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[[Category: Large Structures]]
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[[Category: Single protein]]
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[[Category: Arrowsmith C]]
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[[Category: Arrowsmith, C.]]
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[[Category: Edwards A]]
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[[Category: Delft, F von.]]
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[[Category: Kavanagh K]]
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[[Category: Edwards, A.]]
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[[Category: Ng S]]
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[[Category: Kavanagh, K.]]
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[[Category: Oppermann U]]
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[[Category: Ng, S.]]
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[[Category: Sundstrom M]]
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[[Category: Oppermann, U.]]
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[[Category: Ugochukwu E]]
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[[Category: SGC, Structural Genomics Consortium.]]
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[[Category: Von Delft F]]
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[[Category: Sundstrom, M.]]
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[[Category: Ugochukwu, E.]]
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[[Category: CL]]
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[[Category: NAP]]
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[[Category: SO4]]
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[[Category: human]]
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[[Category: sepiapterin reductase]]
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[[Category: sgc]]
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[[Category: short-chain dehydrogenase/reductase]]
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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 Thu Feb 21 16:12:44 2008''
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Current revision

Crystal Structure of Human Sepiapterin Reductase in complex with NADP+

PDB ID 1z6z

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