8f5f
From Proteopedia
(Difference between revisions)
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| - | '''Unreleased structure''' | ||
| - | + | ==human branched chain ketoacid dehydrogenase kinase in complex with inhibitors== | |
| - | + | <StructureSection load='8f5f' size='340' side='right'caption='[[8f5f]], [[Resolution|resolution]] 3.15Å' scene=''> | |
| - | + | == Structural highlights == | |
| - | + | <table><tr><td colspan='2'>[[8f5f]] 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=8F5F OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=8F5F FirstGlance]. <br> | |
| - | + | </td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">X-ray diffraction, [[Resolution|Resolution]] 3.149Å</td></tr> | |
| - | [[Category: | + | <tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=ADP:ADENOSINE-5-DIPHOSPHATE'>ADP</scene>, <scene name='pdbligand=MG:MAGNESIUM+ION'>MG</scene>, <scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene>, <scene name='pdbligand=XGG:(2P)-2-[(4P)-4-{6-[(1-ethylcyclopropyl)methoxy]pyridin-3-yl}-1,3-thiazol-2-yl]benzoic+acid'>XGG</scene></td></tr> |
| - | [[Category: | + | <tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=8f5f FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=8f5f OCA], [https://pdbe.org/8f5f PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=8f5f RCSB], [https://www.ebi.ac.uk/pdbsum/8f5f PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=8f5f ProSAT]</span></td></tr> |
| - | [[Category: | + | </table> |
| - | [[Category: | + | == Disease == |
| - | [[Category: | + | [https://www.uniprot.org/uniprot/BCKD_HUMAN BCKD_HUMAN] Autism-epilepsy syndrome due to branched chain ketoacid dehydrogenase kinase deficiency. The disease is caused by variants affecting the gene represented in this entry. A diet enriched in branched amino acids (BCAAs) allows to normalize plasma BCAA levels. This suggests that it may be possible to treat patients with mutations in BCKDK with BCAA supplementation. |
| + | == Function == | ||
| + | [https://www.uniprot.org/uniprot/BCKD_HUMAN BCKD_HUMAN] Catalyzes the phosphorylation and inactivation of the branched-chain alpha-ketoacid dehydrogenase complex, the key regulatory enzyme of the valine, leucine and isoleucine catabolic pathways. Key enzyme that regulate the activity state of the BCKD complex.<ref>PMID:24449431</ref> | ||
| + | == References == | ||
| + | <references/> | ||
| + | __TOC__ | ||
| + | </StructureSection> | ||
| + | [[Category: Homo sapiens]] | ||
| + | [[Category: Large Structures]] | ||
| + | [[Category: Bollinger E]] | ||
| + | [[Category: Filipski K]] | ||
| + | [[Category: Liu S]] | ||
| + | [[Category: Roth Flach R]] | ||
Current revision
human branched chain ketoacid dehydrogenase kinase in complex with inhibitors
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