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6h00
From Proteopedia
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| - | '''Unreleased structure''' | ||
| - | + | ==Crystal structure of human pyridoxine 5-phophate oxidase, R116Q variant== | |
| - | + | <StructureSection load='6h00' size='340' side='right' caption='[[6h00]], [[Resolution|resolution]] 1.66Å' scene=''> | |
| - | + | == Structural highlights == | |
| - | + | <table><tr><td colspan='2'>[[6h00]] is a 1 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=6H00 OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=6H00 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>, <scene name='pdbligand=FMN:FLAVIN+MONONUCLEOTIDE'>FMN</scene>, <scene name='pdbligand=SO4:SULFATE+ION'>SO4</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/Pyridoxal_5'-phosphate_synthase Pyridoxal 5'-phosphate synthase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=1.4.3.5 1.4.3.5] </span></td></tr> |
| - | [[Category: | + | <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=6h00 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6h00 OCA], [http://pdbe.org/6h00 PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=6h00 RCSB], [http://www.ebi.ac.uk/pdbsum/6h00 PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=6h00 ProSAT]</span></td></tr> |
| + | </table> | ||
| + | == Disease == | ||
| + | [[http://www.uniprot.org/uniprot/PNPO_HUMAN PNPO_HUMAN]] Defects in PNPO are the cause of pyridoxine-5'-phosphate oxidase deficiency (PNPO deficiency) [MIM:[http://omim.org/entry/610090 610090]]; also known as PNPO-related neonatal epileptic encephalopathy. The main feature of neonatal epileptic encephalopathy is the onset within hours of birth of a severe seizure disorder that does not respond to anticonvulsant drugs and can be fatal. Seizures can cease with the administration of PLP, being resistant to treatment with pyridoxine. | ||
| + | == Function == | ||
| + | [[http://www.uniprot.org/uniprot/PNPO_HUMAN PNPO_HUMAN]] Catalyzes the oxidation of either pyridoxine 5'-phosphate (PNP) or pyridoxamine 5'-phosphate (PMP) into pyridoxal 5'-phosphate (PLP).<ref>PMID:12824491</ref> | ||
| + | == References == | ||
| + | <references/> | ||
| + | __TOC__ | ||
| + | </StructureSection> | ||
| + | [[Category: Pyridoxal 5'-phosphate synthase]] | ||
| + | [[Category: Arrowsmith, C H]] | ||
| + | [[Category: Bezerra, G A]] | ||
| + | [[Category: Bountra, C]] | ||
| + | [[Category: Clayton, P T]] | ||
| + | [[Category: Edwards, A]] | ||
| + | [[Category: Fox, N]] | ||
[[Category: Mackinnon, S]] | [[Category: Mackinnon, S]] | ||
| + | [[Category: Mills, P B]] | ||
[[Category: Newman, J]] | [[Category: Newman, J]] | ||
| - | [[Category: Clayton, P.T]] | ||
| - | [[Category: Bountra, C]] | ||
| - | [[Category: Wilson, M.P]] | ||
| - | [[Category: Fox, N]] | ||
| - | [[Category: Arrowsmith, C.H]] | ||
[[Category: Shrestha, L]] | [[Category: Shrestha, L]] | ||
| - | [[Category: Bezerra, G.A]] | ||
| - | [[Category: Edwards, A]] | ||
| - | [[Category: Mills, P.B]] | ||
[[Category: Sorrell, F]] | [[Category: Sorrell, F]] | ||
| + | [[Category: Wilson, M P]] | ||
| + | [[Category: Yue, W W]] | ||
| + | [[Category: Fmn-binding]] | ||
| + | [[Category: Oxidoreductase]] | ||
| + | [[Category: Pnp-oxidase activity]] | ||
| + | [[Category: Pnpo]] | ||
Current revision
Crystal structure of human pyridoxine 5-phophate oxidase, R116Q variant
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