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2uxw

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==Crystal structure of human very long chain acyl-CoA dehydrogenase (ACADVL)==
==Crystal structure of human very long chain acyl-CoA dehydrogenase (ACADVL)==
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<StructureSection load='2uxw' size='340' side='right' caption='[[2uxw]], [[Resolution|resolution]] 1.45&Aring;' scene=''>
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<StructureSection load='2uxw' size='340' side='right'caption='[[2uxw]], [[Resolution|resolution]] 1.45&Aring;' scene=''>
== Structural highlights ==
== Structural highlights ==
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<table><tr><td colspan='2'>[[2uxw]] is a 1 chain structure with sequence from [http://en.wikipedia.org/wiki/Human Human]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2UXW OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=2UXW FirstGlance]. <br>
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<table><tr><td colspan='2'>[[2uxw]] 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=2UXW OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=2UXW FirstGlance]. <br>
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</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=EDO:1,2-ETHANEDIOL'>EDO</scene>, <scene name='pdbligand=FAD:FLAVIN-ADENINE+DINUCLEOTIDE'>FAD</scene>, <scene name='pdbligand=TH3:TRANS+DELTA2+PALMITENOYL-COENZYMEA'>TH3</scene></td></tr>
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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]] 1.45&#8491;</td></tr>
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<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=2uxw FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2uxw OCA], [http://pdbe.org/2uxw PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=2uxw RCSB], [http://www.ebi.ac.uk/pdbsum/2uxw PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=2uxw ProSAT]</span></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=EDO:1,2-ETHANEDIOL'>EDO</scene>, <scene name='pdbligand=FAD:FLAVIN-ADENINE+DINUCLEOTIDE'>FAD</scene>, <scene name='pdbligand=TH3:TRANS+DELTA2+PALMITENOYL-COENZYMEA'>TH3</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=2uxw FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2uxw OCA], [https://pdbe.org/2uxw PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=2uxw RCSB], [https://www.ebi.ac.uk/pdbsum/2uxw PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=2uxw ProSAT]</span></td></tr>
</table>
</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>
== Evolutionary Conservation ==
== Evolutionary Conservation ==
[[Image:Consurf_key_small.gif|200px|right]]
[[Image:Consurf_key_small.gif|200px|right]]
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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=2uxw ConSurf].
</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=2uxw ConSurf].
<div style="clear:both"></div>
<div style="clear:both"></div>
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==See Also==
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*[[Acyl-CoA dehydrogenase 3D structures|Acyl-CoA dehydrogenase 3D structures]]
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== References ==
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<references/>
__TOC__
__TOC__
</StructureSection>
</StructureSection>
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[[Category: Human]]
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[[Category: Homo sapiens]]
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[[Category: Arrowsmith, C H]]
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[[Category: Large Structures]]
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[[Category: Berridge, G]]
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[[Category: Arrowsmith CH]]
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[[Category: Bunkoczi, G]]
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[[Category: Berridge G]]
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[[Category: Burgess, N]]
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[[Category: Bunkoczi G]]
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[[Category: Delft, F von]]
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[[Category: Burgess N]]
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[[Category: Edwards, A]]
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[[Category: Edwards A]]
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[[Category: Hozjan, V]]
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[[Category: Hozjan V]]
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[[Category: Oppermann, U]]
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[[Category: Oppermann U]]
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[[Category: Pike, A C.W]]
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[[Category: Pike ACW]]
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[[Category: Salah, E]]
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[[Category: Salah E]]
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[[Category: Smee, C]]
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[[Category: Smee C]]
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[[Category: Sundstrom, M]]
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[[Category: Sundstrom M]]
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[[Category: Ugochukwu, E]]
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[[Category: Ugochukwu E]]
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[[Category: Uppenberg, J]]
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[[Category: Uppenberg J]]
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[[Category: Weigelt, J]]
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[[Category: Weigelt J]]
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[[Category: Cardiomyopathy]]
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[[Category: Von Delft F]]
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[[Category: Coenzyme a dehydrogenase]]
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[[Category: Disease mutation]]
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[[Category: Fad]]
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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: Mitochondrial fatty acid beta-oxidation]]
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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 fatty acid]]
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Current revision

Crystal structure of human very long chain acyl-CoA dehydrogenase (ACADVL)

PDB ID 2uxw

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