4ja8

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{{STRUCTURE_4ja8| PDB=4ja8 | SCENE= }}
 
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===Complex of Mitochondrial Isocitrate Dehydrogenase R140Q Mutant with AGI-6780 Inhibitor===
 
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{{ABSTRACT_PUBMED_23558173}}
 
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==About this Structure==
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==Complex of Mitochondrial Isocitrate Dehydrogenase R140Q Mutant with AGI-6780 Inhibitor==
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[[4ja8]] is a 2 chain structure with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4JA8 OCA].
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<StructureSection load='4ja8' size='340' side='right'caption='[[4ja8]], [[Resolution|resolution]] 1.55&Aring;' scene=''>
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== Structural highlights ==
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<table><tr><td colspan='2'>[[4ja8]] is a 2 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=4JA8 OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=4JA8 FirstGlance]. <br>
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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]] 1.55&#8491;</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=1K9:1-[5-(CYCLOPROPYLSULFAMOYL)-2-THIOPHEN-3-YL-PHENYL]-3-[3-(TRIFLUOROMETHYL)PHENYL]UREA'>1K9</scene>, <scene name='pdbligand=CA:CALCIUM+ION'>CA</scene>, <scene name='pdbligand=GOL:GLYCEROL'>GOL</scene>, <scene name='pdbligand=NDP:NADPH+DIHYDRO-NICOTINAMIDE-ADENINE-DINUCLEOTIDE+PHOSPHATE'>NDP</scene></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=4ja8 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4ja8 OCA], [https://pdbe.org/4ja8 PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=4ja8 RCSB], [https://www.ebi.ac.uk/pdbsum/4ja8 PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=4ja8 ProSAT]</span></td></tr>
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</table>
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== Disease ==
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[https://www.uniprot.org/uniprot/IDHP_HUMAN IDHP_HUMAN] D-2-hydroxyglutaric aciduria. Defects in IDH2 are the cause of D-2-hydroxyglutaric aciduria type 2 (D2HGA2) [MIM:[https://omim.org/entry/613657 613657]. D2HGA2 is a neurometabolic disorder causing developmental delay, epilepsy, hypotonia, and dysmorphic features. Both a mild and a severe phenotype exist. The severe phenotype is homogeneous and is characterized by early infantile-onset epileptic encephalopathy and cardiomyopathy. The mild phenotype has a more variable clinical presentation. Diagnosis is based on the presence of an excess of D-2-hydroxyglutaric acid in the urine.<ref>PMID:20847235</ref>
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== Function ==
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[https://www.uniprot.org/uniprot/IDHP_HUMAN IDHP_HUMAN] Plays a role in intermediary metabolism and energy production. It may tightly associate or interact with the pyruvate dehydrogenase complex.
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==See Also==
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*[[Isocitrate dehydrogenase 3D structures|Isocitrate dehydrogenase 3D structures]]
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== References ==
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<references/>
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__TOC__
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</StructureSection>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Chen, L.]]
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[[Category: Large Structures]]
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[[Category: DeLaBarre, B.]]
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[[Category: Chen L]]
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[[Category: Jiang, F.]]
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[[Category: DeLaBarre B]]
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[[Category: Qian, K.]]
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[[Category: Jiang F]]
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[[Category: Travins, J.]]
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[[Category: Qian K]]
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[[Category: Wei, W.]]
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[[Category: Travins J]]
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[[Category: Wu, M.]]
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[[Category: Wei W]]
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[[Category: Metabolic enzyme oncometabolite]]
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[[Category: Wu M]]
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[[Category: Oxidoreductase-oxidoreductase inhibitor complex]]
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[[Category: Protein-inhibitor complex]]
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Current revision

Complex of Mitochondrial Isocitrate Dehydrogenase R140Q Mutant with AGI-6780 Inhibitor

PDB ID 4ja8

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