3i2b

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{{STRUCTURE_3i2b| PDB=3i2b | SCENE= }}
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==The crystal structure of human 6 Pyruvoyl Tetrahydrobiopterin Synthase==
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===The crystal structure of human 6 Pyruvoyl Tetrahydrobiopterin Synthase===
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<StructureSection load='3i2b' size='340' side='right' caption='[[3i2b]], [[Resolution|resolution]] 2.30&Aring;' scene=''>
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== Structural highlights ==
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==Disease==
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<table><tr><td colspan='2'>[[3i2b]] is a 12 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=3I2B OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=3I2B FirstGlance]. <br>
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[[http://www.uniprot.org/uniprot/PTPS_HUMAN PTPS_HUMAN]] Defects in PTS are the cause of BH4-deficient hyperphenylalaninemia type A (HPABH4A) [MIM:[http://omim.org/entry/261640 261640]]; also called 6-pyruvoyl-tetrahydropterin synthase deficiency (PTS deficiency) or hyperphenylalaninemia tetrahydrobiopterin-deficient due to PTS deficiency. HPABH4A is an autosomal recessive disorder characterized by depletion of the neurotransmitters dopamine and serotonin, and clinically by severe neurological symptoms unresponsive to the classic phenylalanine-low diet.<ref>PMID:10531334</ref><ref>PMID:7698774</ref><ref>PMID:8178819</ref><ref>PMID:7493990</ref><ref>PMID:8707300</ref><ref>PMID:9222757</ref><ref>PMID:9159737</ref><ref>PMID:9450907</ref><ref>PMID:10585341</ref><ref>PMID:10220141</ref><ref>PMID:10874306</ref><ref>PMID:11388593</ref>
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</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=EDO:1,2-ETHANEDIOL'>EDO</scene>, <scene name='pdbligand=MG:MAGNESIUM+ION'>MG</scene>, <scene name='pdbligand=NI:NICKEL+(II)+ION'>NI</scene>, <scene name='pdbligand=PEG:DI(HYDROXYETHYL)ETHER'>PEG</scene></td></tr>
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<tr id='related'><td class="sblockLbl"><b>[[Related_structure|Related:]]</b></td><td class="sblockDat">[[1gtq|1gtq]]</td></tr>
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==Function==
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<tr id='gene'><td class="sblockLbl"><b>[[Gene|Gene:]]</b></td><td class="sblockDat">PTS ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 Homo sapiens])</td></tr>
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<tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/6-pyruvoyltetrahydropterin_synthase 6-pyruvoyltetrahydropterin synthase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=4.2.3.12 4.2.3.12] </span></td></tr>
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<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=3i2b FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3i2b OCA], [http://www.rcsb.org/pdb/explore.do?structureId=3i2b RCSB], [http://www.ebi.ac.uk/pdbsum/3i2b PDBsum]</span></td></tr>
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</table>
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== Disease ==
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[[http://www.uniprot.org/uniprot/PTPS_HUMAN PTPS_HUMAN]] Defects in PTS are the cause of BH4-deficient hyperphenylalaninemia type A (HPABH4A) [MIM:[http://omim.org/entry/261640 261640]]; also called 6-pyruvoyl-tetrahydropterin synthase deficiency (PTS deficiency) or hyperphenylalaninemia tetrahydrobiopterin-deficient due to PTS deficiency. HPABH4A is an autosomal recessive disorder characterized by depletion of the neurotransmitters dopamine and serotonin, and clinically by severe neurological symptoms unresponsive to the classic phenylalanine-low diet.<ref>PMID:10531334</ref> <ref>PMID:7698774</ref> <ref>PMID:8178819</ref> <ref>PMID:7493990</ref> <ref>PMID:8707300</ref> <ref>PMID:9222757</ref> <ref>PMID:9159737</ref> <ref>PMID:9450907</ref> <ref>PMID:10585341</ref> <ref>PMID:10220141</ref> <ref>PMID:10874306</ref> <ref>PMID:11388593</ref>
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== Function ==
[[http://www.uniprot.org/uniprot/PTPS_HUMAN PTPS_HUMAN]] Involved in the biosynthesis of tetrahydrobiopterin, an essential cofactor of aromatic amino acid hydroxylases. Catalyzes the transformation of 7,8-dihydroneopterin triphosphate into 6-pyruvoyl tetrahydropterin.
[[http://www.uniprot.org/uniprot/PTPS_HUMAN PTPS_HUMAN]] Involved in the biosynthesis of tetrahydrobiopterin, an essential cofactor of aromatic amino acid hydroxylases. Catalyzes the transformation of 7,8-dihydroneopterin triphosphate into 6-pyruvoyl tetrahydropterin.
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== Evolutionary Conservation ==
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==About this Structure==
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[[Image:Consurf_key_small.gif|200px|right]]
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[[3i2b]] is a 12 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=3I2B OCA].
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Check<jmol>
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<jmolCheckbox>
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==Reference==
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<scriptWhenChecked>select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/i2/3i2b_consurf.spt"</scriptWhenChecked>
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<references group="xtra"/><references/>
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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/chain_selection.php?pdb_ID=2ata 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: 6-pyruvoyltetrahydropterin synthase]]
[[Category: 6-pyruvoyltetrahydropterin synthase]]
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Arrowsmith, C H.]]
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[[Category: Arrowsmith, C H]]
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[[Category: Bountra, C.]]
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[[Category: Bountra, C]]
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[[Category: Bray, J E.]]
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[[Category: Bray, J E]]
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[[Category: Chaikuad, A.]]
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[[Category: Chaikuad, A]]
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[[Category: Cocking, R.]]
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[[Category: Cocking, R]]
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[[Category: Delft, F von.]]
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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: Krojer, T.]]
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[[Category: Krojer, T]]
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[[Category: Muniz, J.]]
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[[Category: Muniz, J]]
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[[Category: Oppermann, U.]]
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[[Category: Oppermann, U]]
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[[Category: Pilka, E.]]
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[[Category: Pilka, E]]
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[[Category: SGC, Structural Genomics Consortium.]]
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[[Category: Structural genomic]]
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[[Category: Ugochukwu, E.]]
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[[Category: Ugochukwu, E]]
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[[Category: Weigelt, J.]]
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[[Category: Weigelt, J]]
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[[Category: Yue, W W.]]
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[[Category: Yue, W W]]
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[[Category: 6 pyruvoyl tetrahydrobiopterin synthase]]
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[[Category: Pyruvoyl tetrahydrobiopterin synthase]]
[[Category: Disease mutation]]
[[Category: Disease mutation]]
[[Category: Lyase]]
[[Category: Lyase]]
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[[Category: Pt]]
[[Category: Pt]]
[[Category: Sgc]]
[[Category: Sgc]]
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[[Category: Structural genomic]]
 
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[[Category: Structural genomics consortium]]
 
[[Category: Tetrahydrobiopterin biosynthesis]]
[[Category: Tetrahydrobiopterin biosynthesis]]

Revision as of 17:33, 18 December 2014

The crystal structure of human 6 Pyruvoyl Tetrahydrobiopterin Synthase

3i2b, resolution 2.30Å

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