View source for 3isq

==Crystal structure of human 4-Hydroxyphenylpyruvate dioxygenase==
<StructureSection load='3isq' size='340' side='right' caption='[[3isq]], [[Resolution|resolution]] 1.75&Aring;' scene=''>
== Structural highlights ==
<table><tr><td colspan='2'>[[3isq]] is a 1 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=3ISQ OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=3ISQ FirstGlance]. <br>
</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=CL:CHLORIDE+ION'>CL</scene>, <scene name='pdbligand=CO:COBALT+(II)+ION'>CO</scene>, <scene name='pdbligand=EDO:1,2-ETHANEDIOL'>EDO</scene>, <scene name='pdbligand=NA:SODIUM+ION'>NA</scene></td></tr>
<tr id='gene'><td class="sblockLbl"><b>[[Gene|Gene:]]</b></td><td class="sblockDat">HPD ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 Homo sapiens])</td></tr>
<tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/4-hydroxyphenylpyruvate_dioxygenase 4-hydroxyphenylpyruvate dioxygenase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=1.13.11.27 1.13.11.27] </span></td></tr>
<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=3isq FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3isq OCA], [http://www.rcsb.org/pdb/explore.do?structureId=3isq RCSB], [http://www.ebi.ac.uk/pdbsum/3isq PDBsum]</span></td></tr>
</table>
== Disease ==
[[http://www.uniprot.org/uniprot/HPPD_HUMAN HPPD_HUMAN]] Defects in HPD are the cause of tyrosinemia type 3 (TYRO3) [MIM:[http://omim.org/entry/276710 276710]]. TYRO3 is an inborn error of metabolism characterized by elevations of tyrosine in the blood and urine, seizures and mild mental retardation.<ref>PMID:10942115</ref> <ref>PMID:11073718</ref>   Defects in HPD are a cause of hawkinsinuria (HAWK) [MIM:[http://omim.org/entry/140350 140350]]. HAWK is an inborn error of tyrosine metabolism characterized by failure to thrive, persistent metabolic acidosis, fine and sparse hair, and excretion of the unusual cyclic amino acid metabolite, hawkinsin, in the urine.<ref>PMID:11073718</ref>  
== Function ==
[[http://www.uniprot.org/uniprot/HPPD_HUMAN HPPD_HUMAN]] Key enzyme in the degradation of tyrosine. 
== Evolutionary Conservation ==
[[Image:Consurf_key_small.gif|200px|right]]
Check<jmol>
  <jmolCheckbox>
    <scriptWhenChecked>select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/is/3isq_consurf.spt"</scriptWhenChecked>
    <scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview01.spt</scriptWhenUnchecked>
    <text>to colour the structure by Evolutionary Conservation</text>
  </jmolCheckbox>
</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].
<div style="clear:both"></div>

==See Also==
*[[Dioxygenase|Dioxygenase]]
== References ==
<references/>
__TOC__
</StructureSection>
[[Category: 4-hydroxyphenylpyruvate dioxygenase]]
[[Category: Homo sapiens]]
[[Category: Arrowsmith, C H]]
[[Category: Bountra, C]]
[[Category: Bray, J E]]
[[Category: Cocking, R]]
[[Category: Delft, F von]]
[[Category: Edwards, A]]
[[Category: Kavanagh, K L]]
[[Category: Krojer, T]]
[[Category: Oppermann, U]]
[[Category: Pike, A C.W]]
[[Category: Pilka, E S]]
[[Category: Structural genomic]]
[[Category: Shafqat, N]]
[[Category: Weigelt, J]]
[[Category: Yue, W W]]
[[Category: Dioxygenase]]
[[Category: Disease mutation]]
[[Category: Hydroxyphenylpyruvate]]
[[Category: Iron]]
[[Category: Mental retardation]]
[[Category: Metal-binding]]
[[Category: Oxidoreductase]]
[[Category: Phenylalanine catabolism]]
[[Category: Phosphoprotein]]
[[Category: Sgc]]
[[Category: Tyrosine catabolism]]
[[Category: Tyrosine metabolism]]