1z6z

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[[Image:1z6z.gif|left|200px]]
 
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{{Structure
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==Crystal Structure of Human Sepiapterin Reductase in complex with NADP+==
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|PDB= 1z6z |SIZE=350|CAPTION= <scene name='initialview01'>1z6z</scene>, resolution 2.50&Aring;
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<StructureSection load='1z6z' size='340' side='right'caption='[[1z6z]], [[Resolution|resolution]] 2.50&Aring;' scene=''>
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|SITE=
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== Structural highlights ==
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|LIGAND= <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>
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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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|ACTIVITY= <span class='plainlinks'>[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] </span>
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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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|GENE= SPR ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 Homo sapiens])
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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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|DOMAIN=
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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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|RELATEDENTRY=[[1sep|1SEP]]
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</table>
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|RESOURCES=<span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=1z6z FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1z6z OCA], [http://www.ebi.ac.uk/pdbsum/1z6z PDBsum], [http://www.rcsb.org/pdb/explore.do?structureId=1z6z RCSB]</span>
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== Disease ==
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}}
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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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'''Crystal Structure of Human Sepiapterin Reductase in complex with NADP+'''
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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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==Disease==
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Check<jmol>
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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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<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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==About this Structure==
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<scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview01.spt</scriptWhenUnchecked>
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1Z6Z is a [[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=1Z6Z OCA].
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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: 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 Mon Mar 31 01:30:17 2008''
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Current revision

Crystal Structure of Human Sepiapterin Reductase in complex with NADP+

PDB ID 1z6z

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