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6nn8
From Proteopedia
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| - | '''Unreleased structure''' | ||
| - | The | + | ==The structure of human liver pyruvate kinase, hLPYK-S531E== |
| - | + | <StructureSection load='6nn8' size='340' side='right'caption='[[6nn8]], [[Resolution|resolution]] 2.42Å' scene=''> | |
| - | + | == Structural highlights == | |
| - | + | <table><tr><td colspan='2'>[[6nn8]] is a 8 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=6NN8 OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=6NN8 FirstGlance]. <br> | |
| - | + | </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></td></tr> | |
| - | [[Category: | + | <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> |
| - | [[Category: Fenton, A | + | <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=6nn8 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6nn8 OCA], [http://pdbe.org/6nn8 PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=6nn8 RCSB], [http://www.ebi.ac.uk/pdbsum/6nn8 PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=6nn8 ProSAT]</span></td></tr> |
| - | [[Category: | + | </table> |
| - | [[Category: | + | == Disease == |
| - | [[Category: Meneely, K | + | [[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. |
| - | [[Category: | + | == Function == |
| + | [[http://www.uniprot.org/uniprot/KPYR_HUMAN KPYR_HUMAN]] Plays a key role in glycolysis (By similarity). | ||
| + | == References == | ||
| + | <references/> | ||
| + | __TOC__ | ||
| + | </StructureSection> | ||
| + | [[Category: Large Structures]] | ||
| + | [[Category: Pyruvate kinase]] | ||
| + | [[Category: Fenton, A W]] | ||
| + | [[Category: Lamb, A L]] | ||
| + | [[Category: McFarlane, J S]] | ||
| + | [[Category: Meneely, K M]] | ||
| + | [[Category: Ronnebaum, T A]] | ||
| + | [[Category: Allosteric]] | ||
| + | [[Category: Glycolysis]] | ||
| + | [[Category: Transferase]] | ||
Revision as of 06:03, 19 June 2019
The structure of human liver pyruvate kinase, hLPYK-S531E
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