1z6z

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{{Seed}}
 
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[[Image:1z6z.png|left|200px]]
 
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==Crystal Structure of Human Sepiapterin Reductase in complex with NADP+==
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The line below this paragraph, containing "STRUCTURE_1z6z", creates the "Structure Box" on the page.
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<StructureSection load='1z6z' size='340' side='right'caption='[[1z6z]], [[Resolution|resolution]] 2.50&Aring;' scene=''>
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You may change the PDB parameter (which sets the PDB file loaded into the applet)
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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'>[[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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or leave the SCENE parameter empty for the default display.
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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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{{STRUCTURE_1z6z| PDB=1z6z | 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=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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===Crystal Structure of Human Sepiapterin Reductase in complex with NADP+===
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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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==Disease==
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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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Known disease associated with this structure: Sepiapterin reductase deficiency OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=182125 182125]]
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== Evolutionary Conservation ==
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[[Image:Consurf_key_small.gif|200px|right]]
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==About this Structure==
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Check<jmol>
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1Z6Z is a 6 chains structure of sequences 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=1Z6Z OCA].
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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: Arrowsmith, C.]]
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[[Category: Arrowsmith C]]
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[[Category: Delft, F von.]]
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[[Category: Edwards A]]
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[[Category: Edwards, A.]]
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[[Category: Kavanagh K]]
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[[Category: Kavanagh, K.]]
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[[Category: Ng S]]
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[[Category: Ng, S.]]
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[[Category: Oppermann U]]
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[[Category: Oppermann, U.]]
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[[Category: Sundstrom M]]
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[[Category: SGC, Structural Genomics Consortium.]]
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[[Category: Ugochukwu E]]
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[[Category: Sundstrom, M.]]
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[[Category: Von Delft F]]
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[[Category: Ugochukwu, E.]]
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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 genomic]]
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[[Category: Structural genomics consortium]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Tue Feb 17 08:50:40 2009''
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Current revision

Crystal Structure of Human Sepiapterin Reductase in complex with NADP+

PDB ID 1z6z

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