1siq

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{{STRUCTURE_1siq| PDB=1siq | SCENE= }}
 
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===The Crystal Structure and Mechanism of Human Glutaryl-CoA Dehydrogenase===
 
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{{ABSTRACT_PUBMED_15274622}}
 
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==Disease==
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==The Crystal Structure and Mechanism of Human Glutaryl-CoA Dehydrogenase==
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[[http://www.uniprot.org/uniprot/GCDH_HUMAN GCDH_HUMAN]] Defects in GCDH are the cause of glutaric aciduria type 1 (GA1) [MIM:[http://omim.org/entry/231670 231670]]. GA1 is an autosomal recessive metabolic disorder characterized by progressive dystonia and athetosis due to gliosis and neuronal loss in the basal ganglia.<ref>PMID:18775954</ref><ref>PMID:8541831</ref><ref>PMID:9600243</ref><ref>PMID:8900227</ref><ref>PMID:8900228</ref><ref>PMID:14707522</ref>
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<StructureSection load='1siq' size='340' side='right'caption='[[1siq]], [[Resolution|resolution]] 2.10&Aring;' scene=''>
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== Structural highlights ==
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<table><tr><td colspan='2'>[[1siq]] is a 1 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=1SIQ OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=1SIQ 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]] 2.1&#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=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=1siq FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1siq OCA], [https://pdbe.org/1siq PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=1siq RCSB], [https://www.ebi.ac.uk/pdbsum/1siq PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=1siq ProSAT]</span></td></tr>
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</table>
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== Disease ==
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[https://www.uniprot.org/uniprot/GCDH_HUMAN GCDH_HUMAN] Defects in GCDH are the cause of glutaric aciduria type 1 (GA1) [MIM:[https://omim.org/entry/231670 231670]. GA1 is an autosomal recessive metabolic disorder characterized by progressive dystonia and athetosis due to gliosis and neuronal loss in the basal ganglia.<ref>PMID:18775954</ref> <ref>PMID:8541831</ref> <ref>PMID:9600243</ref> <ref>PMID:8900227</ref> <ref>PMID:8900228</ref> <ref>PMID:14707522</ref>
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== Function ==
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[https://www.uniprot.org/uniprot/GCDH_HUMAN GCDH_HUMAN] Catalyzes the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and CO(2) in the degradative pathway of L-lysine, L-hydroxylysine, and L-tryptophan metabolism. It uses electron transfer flavoprotein as its electron acceptor. Isoform Short is inactive.
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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/si/1siq_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=1siq 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/GCDH_HUMAN GCDH_HUMAN]] Catalyzes the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and CO(2) in the degradative pathway of L-lysine, L-hydroxylysine, and L-tryptophan metabolism. It uses electron transfer flavoprotein as its electron acceptor. Isoform Short is inactive.
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*[[Acyl-CoA dehydrogenase 3D structures|Acyl-CoA dehydrogenase 3D structures]]
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*[[Glutaryl-CoA dehydrogenase|Glutaryl-CoA dehydrogenase]]
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==About this Structure==
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== References ==
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[[1siq]] is a 1 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=1SIQ OCA].
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<references/>
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__TOC__
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==Reference==
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</StructureSection>
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<ref group="xtra">PMID:015274622</ref><references group="xtra"/><references/>
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[[Category: Glutaryl-CoA dehydrogenase]]
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[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Frerman, F E.]]
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[[Category: Large Structures]]
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[[Category: Fu, Z.]]
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[[Category: Frerman FE]]
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[[Category: Goodman, S.]]
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[[Category: Fu Z]]
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[[Category: Kim, J J.]]
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[[Category: Goodman S]]
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[[Category: Paschke, R.]]
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[[Category: Kim JJ]]
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[[Category: Wang, M.]]
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[[Category: Paschke R]]
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[[Category: Acyl-coa dehydrogenase]]
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[[Category: Wang M]]
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[[Category: Decarboxylation]]
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[[Category: Flavin protein]]
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[[Category: Oxidoreductase]]
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Current revision

The Crystal Structure and Mechanism of Human Glutaryl-CoA Dehydrogenase

PDB ID 1siq

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