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6qh4

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==Crystal structure of human Methylmalonyl-CoA epimerase (MCEE) p.Arg143Cys variant==
==Crystal structure of human Methylmalonyl-CoA epimerase (MCEE) p.Arg143Cys variant==
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<StructureSection load='6qh4' size='340' side='right' caption='[[6qh4]], [[Resolution|resolution]] 1.92&Aring;' scene=''>
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<StructureSection load='6qh4' size='340' side='right'caption='[[6qh4]], [[Resolution|resolution]] 1.92&Aring;' scene=''>
== Structural highlights ==
== Structural highlights ==
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<table><tr><td colspan='2'>[[6qh4]] is a 4 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=6QH4 OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=6QH4 FirstGlance]. <br>
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<table><tr><td colspan='2'>[[6qh4]] is a 4 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=6QH4 OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=6QH4 FirstGlance]. <br>
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</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=CO:COBALT+(II)+ION'>CO</scene></td></tr>
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</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">X-ray diffraction, [[Resolution|Resolution]] 1.922&#8491;</td></tr>
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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/Methylmalonyl-CoA_epimerase Methylmalonyl-CoA epimerase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=5.1.99.1 5.1.99.1] </span></td></tr>
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<tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=CO:COBALT+(II)+ION'>CO</scene></td></tr>
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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=6qh4 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6qh4 OCA], [http://pdbe.org/6qh4 PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=6qh4 RCSB], [http://www.ebi.ac.uk/pdbsum/6qh4 PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=6qh4 ProSAT]</span></td></tr>
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<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=6qh4 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6qh4 OCA], [https://pdbe.org/6qh4 PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=6qh4 RCSB], [https://www.ebi.ac.uk/pdbsum/6qh4 PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=6qh4 ProSAT]</span></td></tr>
</table>
</table>
== Disease ==
== Disease ==
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[[http://www.uniprot.org/uniprot/MCEE_HUMAN MCEE_HUMAN]] Vitamin B12-unresponsive methylmalonic acidemia. Methylmalonyl-CoA epimerase deficiency (MCEED) [MIM:[http://omim.org/entry/251120 251120]]: Autosomal recessive inborn error of amino acid metabolism, involving valine, threonine, isoleucine and methionine. This organic aciduria may present in the neonatal period with life-threatening metabolic acidosis, hyperammonemia, feeding difficulties, pancytopenia and coma. Note=The disease is caused by mutations affecting the gene represented in this entry.<ref>PMID:16752391</ref>
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[https://www.uniprot.org/uniprot/MCEE_HUMAN MCEE_HUMAN] Vitamin B12-unresponsive methylmalonic acidemia. Methylmalonyl-CoA epimerase deficiency (MCEED) [MIM:[https://omim.org/entry/251120 251120]: Autosomal recessive inborn error of amino acid metabolism, involving valine, threonine, isoleucine and methionine. This organic aciduria may present in the neonatal period with life-threatening metabolic acidosis, hyperammonemia, feeding difficulties, pancytopenia and coma. Note=The disease is caused by mutations affecting the gene represented in this entry.<ref>PMID:16752391</ref>
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== Function ==
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[https://www.uniprot.org/uniprot/MCEE_HUMAN MCEE_HUMAN]
== References ==
== References ==
<references/>
<references/>
__TOC__
__TOC__
</StructureSection>
</StructureSection>
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[[Category: Methylmalonyl-CoA epimerase]]
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[[Category: Homo sapiens]]
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[[Category: Bailey, H J]]
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[[Category: Large Structures]]
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[[Category: Bountra, C]]
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[[Category: Bailey HJ]]
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[[Category: Chaikuid, A]]
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[[Category: Bountra C]]
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[[Category: Diaz-Saez, L]]
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[[Category: Chaikuid A]]
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[[Category: Edwards, A M]]
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[[Category: Diaz-Saez L]]
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[[Category: Froese, D S]]
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[[Category: Edwards AM]]
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[[Category: Kennedy, E]]
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[[Category: Froese DS]]
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[[Category: Krysztofinska, E]]
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[[Category: Kennedy E]]
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[[Category: Sorrell, F J]]
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[[Category: Krysztofinska E]]
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[[Category: Yue, W W]]
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[[Category: Sorrell FJ]]
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[[Category: Isomerase]]
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[[Category: Yue WW]]
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[[Category: Methylmalonyl-coa epimerase]]
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[[Category: Mitochondrial]]
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Current revision

Crystal structure of human Methylmalonyl-CoA epimerase (MCEE) p.Arg143Cys variant

PDB ID 6qh4

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