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==Crystal structure of human 4-Hydroxyphenylpyruvate dioxygenase==
==Crystal structure of human 4-Hydroxyphenylpyruvate dioxygenase==
<StructureSection load='3isq' size='340' side='right' caption='[[3isq]], [[Resolution|resolution]] 1.75&Aring;' scene=''>
<StructureSection load='3isq' size='340' side='right'caption='[[3isq]], [[Resolution|resolution]] 1.75&Aring;' scene=''>
== Structural highlights ==
== 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>
<table><tr><td colspan='2'>[[3isq]] is a 1 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=3ISQ OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/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>
</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">X-ray diffraction, [[Resolution|Resolution]] 1.75&#8491;</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='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=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='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'>[https://proteopedia.org/fgij/fg.htm?mol=3isq FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3isq OCA], [https://pdbe.org/3isq PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=3isq RCSB], [https://www.ebi.ac.uk/pdbsum/3isq PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=3isq ProSAT]</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>
</table>
== Disease ==
== 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>
[https://www.uniprot.org/uniprot/HPPD_HUMAN HPPD_HUMAN] Defects in HPD are the cause of tyrosinemia type 3 (TYRO3) [MIM:[https://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:[https://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 ==
== Function ==
[[http://www.uniprot.org/uniprot/HPPD_HUMAN HPPD_HUMAN]] Key enzyme in the degradation of tyrosine.  
[https://www.uniprot.org/uniprot/HPPD_HUMAN HPPD_HUMAN] Key enzyme in the degradation of tyrosine.
== Evolutionary Conservation ==
== Evolutionary Conservation ==
[[Image:Consurf_key_small.gif|200px|right]]
[[Image:Consurf_key_small.gif|200px|right]]
Check<jmol>
Check<jmol>
   <jmolCheckbox>
   <jmolCheckbox>
     <scriptWhenChecked>select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/is/3isq_consurf.spt"</scriptWhenChecked>
     <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>
     <scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview01.spt</scriptWhenUnchecked>
     <text>to colour the structure by Evolutionary Conservation</text>
     <text>to colour the structure by Evolutionary Conservation</text>
   </jmolCheckbox>
   </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].
</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=3isq ConSurf].
<div style="clear:both"></div>
<div style="clear:both"></div>


==See Also==
==See Also==
*[[Dioxygenase|Dioxygenase]]
*[[Dioxygenase 3D structures|Dioxygenase 3D structures]]
== References ==
== References ==
<references/>
<references/>
__TOC__
__TOC__
</StructureSection>
</StructureSection>
[[Category: 4-hydroxyphenylpyruvate dioxygenase]]
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
[[Category: Arrowsmith, C H]]
[[Category: Large Structures]]
[[Category: Bountra, C]]
[[Category: Arrowsmith CH]]
[[Category: Bray, J E]]
[[Category: Bountra C]]
[[Category: Cocking, R]]
[[Category: Bray JE]]
[[Category: Delft, F von]]
[[Category: Cocking R]]
[[Category: Edwards, A]]
[[Category: Edwards A]]
[[Category: Kavanagh, K L]]
[[Category: Kavanagh KL]]
[[Category: Krojer, T]]
[[Category: Krojer T]]
[[Category: Oppermann, U]]
[[Category: Oppermann U]]
[[Category: Pike, A C.W]]
[[Category: Pike ACW]]
[[Category: Pilka, E S]]
[[Category: Pilka ES]]
[[Category: Structural genomic]]
[[Category: Shafqat N]]
[[Category: Shafqat, N]]
[[Category: Weigelt J]]
[[Category: Weigelt, J]]
[[Category: Yue WW]]
[[Category: Yue, W W]]
[[Category: Von Delft F]]
[[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]]

Latest revision as of 19:02, 1 November 2023

Crystal structure of human 4-Hydroxyphenylpyruvate dioxygenaseCrystal structure of human 4-Hydroxyphenylpyruvate dioxygenase

Structural highlights

3isq is a 1 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA. For a guided tour on the structure components use FirstGlance.
Method:X-ray diffraction, Resolution 1.75Å
Ligands:, , ,
Resources:FirstGlance, OCA, PDBe, RCSB, PDBsum, ProSAT

Disease

HPPD_HUMAN Defects in HPD are the cause of tyrosinemia type 3 (TYRO3) [MIM:276710. TYRO3 is an inborn error of metabolism characterized by elevations of tyrosine in the blood and urine, seizures and mild mental retardation.[1] [2] Defects in HPD are a cause of hawkinsinuria (HAWK) [MIM: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.[3]

Function

HPPD_HUMAN Key enzyme in the degradation of tyrosine.

Evolutionary Conservation

Check, as determined by ConSurfDB. You may read the explanation of the method and the full data available from ConSurf.

See Also

References

  1. ↑ Ruetschi U, Cerone R, Perez-Cerda C, Schiaffino MC, Standing S, Ugarte M, Holme E. Mutations in the 4-hydroxyphenylpyruvate dioxygenase gene (HPD) in patients with tyrosinemia type III. Hum Genet. 2000 Jun;106(6):654-62. PMID:10942115
  2. ↑ Tomoeda K, Awata H, Matsuura T, Matsuda I, Ploechl E, Milovac T, Boneh A, Scott CR, Danks DM, Endo F. Mutations in the 4-hydroxyphenylpyruvic acid dioxygenase gene are responsible for tyrosinemia type III and hawkinsinuria. Mol Genet Metab. 2000 Nov;71(3):506-10. PMID:11073718 doi:10.1006/mgme.2000.3085
  3. ↑ Tomoeda K, Awata H, Matsuura T, Matsuda I, Ploechl E, Milovac T, Boneh A, Scott CR, Danks DM, Endo F. Mutations in the 4-hydroxyphenylpyruvic acid dioxygenase gene are responsible for tyrosinemia type III and hawkinsinuria. Mol Genet Metab. 2000 Nov;71(3):506-10. PMID:11073718 doi:10.1006/mgme.2000.3085

3isq, resolution 1.75Å

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