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6nn5

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'''Unreleased structure'''
 
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The entry 6nn5 is ON HOLD until Paper Publication
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==The structure of human liver pyruvate kinase, hLPYK-W527H==
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<StructureSection load='6nn5' size='340' side='right'caption='[[6nn5]], [[Resolution|resolution]] 2.26&Aring;' scene=''>
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Authors: McFarlane, J.S., Ronnebaum, T.A., Meneely, K.M., Fenton, A.W., Lamb, A.L.
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== Structural highlights ==
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<table><tr><td colspan='2'>[[6nn5]] is a 4 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=6NN5 OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=6NN5 FirstGlance]. <br>
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Description: The structure of human liver pyruvate kinase, hLPYK-W527H
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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=GOL:GLYCEROL'>GOL</scene></td></tr>
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[[Category: Unreleased Structures]]
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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/Pyruvate_kinase Pyruvate kinase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=2.7.1.40 2.7.1.40] </span></td></tr>
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[[Category: Lamb, A.L]]
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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=6nn5 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6nn5 OCA], [http://pdbe.org/6nn5 PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=6nn5 RCSB], [http://www.ebi.ac.uk/pdbsum/6nn5 PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=6nn5 ProSAT]</span></td></tr>
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[[Category: Fenton, A.W]]
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</table>
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[[Category: Meneely, K.M]]
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== Disease ==
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[[Category: Mcfarlane, J.S]]
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[[http://www.uniprot.org/uniprot/KPYR_HUMAN KPYR_HUMAN]] Defects in PKLR are the cause of pyruvate kinase hyperactivity (PKHYP) [MIM:[http://omim.org/entry/102900 102900]]; also known as high red cell ATP syndrome. This autosomal dominant phenotype is characterized by increase of red blood cell ATP.<ref>PMID:9090535</ref> Defects in PKLR are the cause of pyruvate kinase deficiency of red cells (PKRD) [MIM:[http://omim.org/entry/266200 266200]]. A frequent cause of hereditary non-spherocytic hemolytic anemia. Clinically, pyruvate kinase-deficient patients suffer from a highly variable degree of chronic hemolysis, ranging from severe neonatal jaundice and fatal anemia at birth, severe transfusion-dependent chronic hemolysis, moderate hemolysis with exacerbation during infection, to a fully compensated hemolysis without apparent anemia.
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[[Category: Ronnebaum, T.A]]
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== Function ==
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[[http://www.uniprot.org/uniprot/KPYR_HUMAN KPYR_HUMAN]] Plays a key role in glycolysis (By similarity).
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== References ==
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<references/>
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__TOC__
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</StructureSection>
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[[Category: Large Structures]]
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[[Category: Pyruvate kinase]]
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[[Category: Fenton, A W]]
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[[Category: Lamb, A L]]
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[[Category: McFarlane, J S]]
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[[Category: Meneely, K M]]
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[[Category: Ronnebaum, T A]]
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[[Category: Allosteric]]
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[[Category: Glycolysis]]
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[[Category: Transferase]]

Revision as of 06:03, 19 June 2019

The structure of human liver pyruvate kinase, hLPYK-W527H

PDB ID 6nn5

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