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New page: left|200px<br /> <applet load="2hqq" size="450" color="white" frame="true" align="right" spinBox="true" caption="2hqq, resolution 1.86Å" /> '''Crystal structure o...
 
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[[Image:2hqq.gif|left|200px]]<br />
<applet load="2hqq" size="450" color="white" frame="true" align="right" spinBox="true"
caption="2hqq, resolution 1.86&Aring;" />
'''Crystal structure of human ketohexokinase complexed to different sugar molecules'''<br />


==Disease==
==Crystal structure of human ketohexokinase complexed to different sugar molecules==
Known disease associated with this structure: Fructosuria OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=229800 229800]]
<StructureSection load='2hqq' size='340' side='right'caption='[[2hqq]], [[Resolution|resolution]] 1.86&Aring;' scene=''>
== Structural highlights ==
<table><tr><td colspan='2'>[[2hqq]] 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=2HQQ OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=2HQQ FirstGlance]. <br>
</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">X-ray diffraction, [[Resolution|Resolution]] 1.86&#8491;</td></tr>
<tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene></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=2hqq FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2hqq OCA], [https://pdbe.org/2hqq PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=2hqq RCSB], [https://www.ebi.ac.uk/pdbsum/2hqq PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=2hqq ProSAT]</span></td></tr>
</table>
== Disease ==
[https://www.uniprot.org/uniprot/KHK_HUMAN KHK_HUMAN] Defects in KHK are the cause of fructosuria (FRUCT) [MIM:[https://omim.org/entry/229800 229800]. Benign defect of intermediary metabolism.<ref>PMID:19237742</ref> <ref>PMID:7833921</ref>
== Function ==
[https://www.uniprot.org/uniprot/KHK_HUMAN KHK_HUMAN]
== 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/hq/2hqq_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/main_output.php?pdb_ID=2hqq ConSurf].
<div style="clear:both"></div>
<div style="background-color:#fffaf0;">
== Publication Abstract from PubMed ==
A molecular understanding of the unique aspects of dietary fructose metabolism may be the key to understanding and controlling the current epidemic of fructose-related obesity, diabetes and related adverse metabolic states in Western populations. Fructose catabolism is initiated by its phosphorylation to fructose 1-phosphate, which is performed by ketohexokinase (KHK). Here, the crystal structures of the two alternatively spliced isoforms of human ketohexokinase, hepatic KHK-C and the peripheral isoform KHK-A, and of the ternary complex of KHK-A with the substrate fructose and AMP-PNP are reported. The structure of the KHK-A ternary complex revealed an active site with both the substrate fructose and the ATP analogue in positions ready for phosphorylation following a reaction mechanism similar to that of the pfkB family of carbohydrate kinases. Hepatic KHK deficiency causes the benign disorder essential fructosuria. The effects of the disease-causing mutations (Gly40Arg and Ala43Thr) have been modelled in the context of the KHK structure.


==About this Structure==
Structures of alternatively spliced isoforms of human ketohexokinase.,Trinh CH, Asipu A, Bonthron DT, Phillips SE Acta Crystallogr D Biol Crystallogr. 2009 Mar;65(Pt 3):201-11. Epub 2009, Feb 20. PMID:19237742<ref>PMID:19237742</ref>
2HQQ is a [http://en.wikipedia.org/wiki/Single_protein Single protein] structure of sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens] with SO4 as [http://en.wikipedia.org/wiki/ligand ligand]. Active as [http://en.wikipedia.org/wiki/Ketohexokinase Ketohexokinase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=2.7.1.3 2.7.1.3] Full crystallographic information is available from [http://ispc.weizmann.ac.il/oca-bin/ocashort?id=2HQQ OCA].
 
From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
</div>
<div class="pdbe-citations 2hqq" style="background-color:#fffaf0;"></div>
 
==See Also==
*[[Ketohexokinase|Ketohexokinase]]
== References ==
<references/>
__TOC__
</StructureSection>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
[[Category: Ketohexokinase]]
[[Category: Large Structures]]
[[Category: Single protein]]
[[Category: Asipu A]]
[[Category: Asipu, A.]]
[[Category: Bonthron DT]]
[[Category: Bonthron, D.T.]]
[[Category: Phillips SEV]]
[[Category: Phillips, S.E.V.]]
[[Category: Trinh CH]]
[[Category: Trinh, C.H.]]
[[Category: SO4]]
[[Category: fructose kinase]]
 
''Page seeded by [http://ispc.weizmann.ac.il/oca OCA ] on Mon Nov 12 22:35:07 2007''

Latest revision as of 10:00, 30 August 2023

Crystal structure of human ketohexokinase complexed to different sugar molecules

2hqq, resolution 1.86Å

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