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2uxw

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[[Image:2uxw.jpg|left|200px]]<br /><applet load="2uxw" size="350" color="white" frame="true" align="right" spinBox="true"
 
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caption="2uxw, resolution 1.45&Aring;" />
 
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'''CRYSTAL STRUCTURE OF HUMAN VERY LONG CHAIN ACYL-COA DEHYDROGENASE (ACADVL)'''<br />
 
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==Disease==
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==Crystal structure of human very long chain acyl-CoA dehydrogenase (ACADVL)==
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Known diseases associated with this structure: VLCAD deficiency OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=609575 609575]]
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<StructureSection load='2uxw' size='340' side='right'caption='[[2uxw]], [[Resolution|resolution]] 1.45&Aring;' scene=''>
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== Structural highlights ==
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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='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='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>
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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/ux/2uxw_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=2uxw ConSurf].
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<div style="clear:both"></div>
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==About this Structure==
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==See Also==
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2UXW is a [http://en.wikipedia.org/wiki/Single_protein Single protein] structure of sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens] with <scene name='pdbligand=EDO:'>EDO</scene>, <scene name='pdbligand=FAD:'>FAD</scene> and <scene name='pdbligand=TH3:'>TH3</scene> as [http://en.wikipedia.org/wiki/ligands ligands]. Known structural/functional Site: <scene name='pdbsite=AC1:Edo+Binding+Site+For+Chain+A'>AC1</scene>. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2UXW OCA].
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*[[Acyl-CoA dehydrogenase 3D structures|Acyl-CoA dehydrogenase 3D structures]]
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== References ==
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<references/>
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__TOC__
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</StructureSection>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Single protein]]
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[[Category: Large Structures]]
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[[Category: Arrowsmith, C.H.]]
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[[Category: Arrowsmith CH]]
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[[Category: Berridge, G.]]
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[[Category: Berridge G]]
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[[Category: Bunkoczi, G.]]
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[[Category: Bunkoczi G]]
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[[Category: Burgess, N.]]
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[[Category: Burgess N]]
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[[Category: Delft, F.Von.]]
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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 ACW]]
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[[Category: Pike, A.C.W.]]
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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: Von Delft F]]
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[[Category: EDO]]
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[[Category: FAD]]
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[[Category: TH3]]
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[[Category: acetylation]]
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[[Category: alternative splicing]]
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[[Category: cardiomyopathy]]
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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: polymorphism]]
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[[Category: transit peptide]]
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[[Category: very long chain fatty acids]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Sun Feb 3 10:48:13 2008''
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Current revision

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

PDB ID 2uxw

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