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1rx0

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{{STRUCTURE_1rx0| PDB=1rx0 | SCENE= }}
 
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===Crystal structure of isobutyryl-CoA dehydrogenase complexed with substrate/ligand.===
 
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{{ABSTRACT_PUBMED_14752098}}
 
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==Disease==
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==Crystal structure of isobutyryl-CoA dehydrogenase complexed with substrate/ligand.==
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[[http://www.uniprot.org/uniprot/ACAD8_HUMAN ACAD8_HUMAN]] Defects in ACAD8 are the cause of isobutyryl-CoA dehydrogenase deficiency (IBDD) [MIM:[http://omim.org/entry/611283 611283]]. The symptoms of IBDD generally appear until late in infancy or in childhood and can include poor feeding and growth (failure to thrive), a weakened and enlarged heart (dilated cardiomyopathy), seizures, and low numbers of red blood cells (anemia).<ref>PMID:12359132</ref><ref>PMID:15505379</ref><ref>PMID:16857760</ref>
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<StructureSection load='1rx0' size='340' side='right'caption='[[1rx0]], [[Resolution|resolution]] 1.77&Aring;' scene=''>
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== Structural highlights ==
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<table><tr><td colspan='2'>[[1rx0]] 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=1RX0 OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=1RX0 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]] 1.77&#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=2MC:METHACRYLYL-COENZYME+A'>2MC</scene>, <scene name='pdbligand=ACY:ACETIC+ACID'>ACY</scene>, <scene name='pdbligand=EDO:1,2-ETHANEDIOL'>EDO</scene>, <scene name='pdbligand=FAD:FLAVIN-ADENINE+DINUCLEOTIDE'>FAD</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=1rx0 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1rx0 OCA], [https://pdbe.org/1rx0 PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=1rx0 RCSB], [https://www.ebi.ac.uk/pdbsum/1rx0 PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=1rx0 ProSAT]</span></td></tr>
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</table>
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== Disease ==
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[https://www.uniprot.org/uniprot/ACAD8_HUMAN ACAD8_HUMAN] Defects in ACAD8 are the cause of isobutyryl-CoA dehydrogenase deficiency (IBDD) [MIM:[https://omim.org/entry/611283 611283]. The symptoms of IBDD generally appear until late in infancy or in childhood and can include poor feeding and growth (failure to thrive), a weakened and enlarged heart (dilated cardiomyopathy), seizures, and low numbers of red blood cells (anemia).<ref>PMID:12359132</ref> <ref>PMID:15505379</ref> <ref>PMID:16857760</ref>
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== Function ==
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[https://www.uniprot.org/uniprot/ACAD8_HUMAN ACAD8_HUMAN] Has very high activity toward isobutyryl-CoA. Is an isobutyryl-CoA dehydrogenase that functions in valine catabolism. Plays a role in transcriptional coactivation within the ARC complex.<ref>PMID:11013134</ref> <ref>PMID:12359132</ref>
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== Evolutionary Conservation ==
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[[Image:Consurf_key_small.gif|200px|right]]
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Check<jmol>
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<jmolCheckbox>
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<scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/rx/1rx0_consurf.spt"</scriptWhenChecked>
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<scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview01.spt</scriptWhenUnchecked>
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<text>to colour the structure by Evolutionary Conservation</text>
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</jmolCheckbox>
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</jmol>, as determined by [http://consurfdb.tau.ac.il/ ConSurfDB]. You may read the [[Conservation%2C_Evolutionary|explanation]] of the method and the full data available from [http://bental.tau.ac.il/new_ConSurfDB/main_output.php?pdb_ID=1rx0 ConSurf].
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<div style="clear:both"></div>
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==Function==
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==See Also==
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[[http://www.uniprot.org/uniprot/ACAD8_HUMAN ACAD8_HUMAN]] Has very high activity toward isobutyryl-CoA. Is an isobutyryl-CoA dehydrogenase that functions in valine catabolism. Plays a role in transcriptional coactivation within the ARC complex.<ref>PMID:11013134</ref><ref>PMID:12359132</ref>
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*[[Acyl-CoA dehydrogenase 3D structures|Acyl-CoA dehydrogenase 3D structures]]
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== References ==
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==About this Structure==
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<references/>
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[[1rx0]] is a 4 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=1RX0 OCA].
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__TOC__
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</StructureSection>
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==Reference==
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<ref group="xtra">PMID:014752098</ref><references group="xtra"/><references/>
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[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Battaile, K P.]]
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[[Category: Large Structures]]
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[[Category: Kim, J J.]]
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[[Category: Battaile KP]]
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[[Category: Nguyen, T V.]]
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[[Category: Kim JJ]]
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[[Category: Vockley, J.]]
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[[Category: Nguyen TV]]
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[[Category: Coenzyme some]]
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[[Category: Vockley J]]
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[[Category: Dehydrogenase]]
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[[Category: Flavoprotein]]
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[[Category: Oxidoreductase]]
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Current revision

Crystal structure of isobutyryl-CoA dehydrogenase complexed with substrate/ligand.

PDB ID 1rx0

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