3b96

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[[Image:3b96.png|left|200px]]
 
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{{STRUCTURE_3b96| PDB=3b96 | SCENE= }}
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==Structural Basis for Substrate Fatty-Acyl Chain Specificity: Crystal Structure of Human Very-Long-Chain Acyl-CoA Dehydrogenase==
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<StructureSection load='3b96' size='340' side='right'caption='[[3b96]], [[Resolution|resolution]] 1.91&Aring;' scene=''>
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===Structural Basis for Substrate Fatty-Acyl Chain Specificity: Crystal Structure of Human Very-Long-Chain Acyl-CoA Dehydrogenase===
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== Structural highlights ==
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<table><tr><td colspan='2'>[[3b96]] 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=3B96 OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=3B96 FirstGlance]. <br>
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{{ABSTRACT_PUBMED_18227065}}
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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]] 1.91&#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>, <scene name='pdbligand=MYA:TETRADECANOYL-COA'>MYA</scene></td></tr>
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==About this Structure==
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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=3b96 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3b96 OCA], [https://pdbe.org/3b96 PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=3b96 RCSB], [https://www.ebi.ac.uk/pdbsum/3b96 PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=3b96 ProSAT]</span></td></tr>
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[[3b96]] 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=3B96 OCA].
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</table>
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== Disease ==
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[https://www.uniprot.org/uniprot/ACADV_HUMAN ACADV_HUMAN] Defects in ACADVL are the cause of acyl-CoA dehydrogenase very long chain deficiency (ACADVLD) [MIM:[https://omim.org/entry/201475 201475]. ACADVLD is an autosomal recessive disease which leads to impaired long-chain fatty acid beta-oxidation. It is clinically heterogeneous, with three major phenotypes: a severe childhood form, with early onset, high mortality, and high incidence of cardiomyopathy; a milder childhood form, with later onset, usually with hypoketotic hypoglycemia as the main presenting feature, low mortality, and rare cardiomyopathy; and an adult form, with isolated skeletal muscle involvement, rhabdomyolysis, and myoglobinuria, usually triggered by exercise or fasting.<ref>PMID:8554073</ref> <ref>PMID:9546340</ref> <ref>PMID:10077518</ref>
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== Function ==
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[https://www.uniprot.org/uniprot/ACADV_HUMAN ACADV_HUMAN] Active toward esters of long-chain and very long chain fatty acids such as palmitoyl-CoA, mysritoyl-CoA and stearoyl-CoA. Can accommodate substrate acyl chain lengths as long as 24 carbons, but shows little activity for substrates of less than 12 carbons.<ref>PMID:18227065</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/b9/3b96_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=3b96 ConSurf].
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<div style="clear:both"></div>
==See Also==
==See Also==
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*[[Acyl-CoA dehydrogenase|Acyl-CoA dehydrogenase]]
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*[[Acyl-CoA dehydrogenase 3D structures|Acyl-CoA dehydrogenase 3D structures]]
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== References ==
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==Reference==
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<references/>
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<ref group="xtra">PMID:018227065</ref><references group="xtra"/>
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__TOC__
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</StructureSection>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: He, M.]]
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[[Category: Large Structures]]
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[[Category: Kim, J J.]]
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[[Category: He M]]
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[[Category: McAndrew, R P.]]
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[[Category: Kim JJ]]
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[[Category: Mohsen, A W.]]
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[[Category: McAndrew RP]]
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[[Category: Vockley, J.]]
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[[Category: Mohsen AW]]
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[[Category: Wang, Y.]]
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[[Category: Vockley J]]
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[[Category: Acyl-coa]]
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[[Category: Wang Y]]
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[[Category: Cardiomyopathy]]
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[[Category: Dehydrogenase]]
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[[Category: Disease mutation]]
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[[Category: Fad]]
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[[Category: Fatty acid beta-oxidation]]
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[[Category: Fatty acid metabolism]]
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[[Category: Flavoprotein]]
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[[Category: Lipid metabolism]]
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[[Category: Membrane]]
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[[Category: Mitochondria]]
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[[Category: Mitochondrion]]
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[[Category: Oxidoreductase]]
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[[Category: Transit peptide]]
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[[Category: Very long chain]]
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Current revision

Structural Basis for Substrate Fatty-Acyl Chain Specificity: Crystal Structure of Human Very-Long-Chain Acyl-CoA Dehydrogenase

PDB ID 3b96

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