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[[Image:3b96.jpg|left|200px]]


{{Structure
==Structural Basis for Substrate Fatty-Acyl Chain Specificity: Crystal Structure of Human Very-Long-Chain Acyl-CoA Dehydrogenase==
|PDB= 3b96 |SIZE=350|CAPTION= <scene name='initialview01'>3b96</scene>, resolution 1.910&Aring;
<StructureSection load='3b96' size='340' side='right'caption='[[3b96]], [[Resolution|resolution]] 1.91&Aring;' scene=''>
|SITE= <scene name='pdbsite=AC1:Mya+Binding+Site+For+Residue+A+1'>AC1</scene> and <scene name='pdbsite=AC2:Fad+Binding+Site+For+Residue+A+616'>AC2</scene>
== Structural highlights ==
|LIGAND= <scene name='pdbligand=MYA:TETRADECANOYL-COA'>MYA</scene> and <scene name='pdbligand=FAD:FLAVIN-ADENINE DINUCLEOTIDE'>FAD</scene>
<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>
|ACTIVITY=  
</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>
|GENE= ACADVL, VLCAD ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 Homo sapiens])
<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>
}}
<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>
</table>
== Disease ==
[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>
== Function ==
[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 ==
[[Image:Consurf_key_small.gif|200px|right]]
Check<jmol>
  <jmolCheckbox>
    <scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/b9/3b96_consurf.spt"</scriptWhenChecked>
    <scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview01.spt</scriptWhenUnchecked>
    <text>to colour the structure by Evolutionary Conservation</text>
  </jmolCheckbox>
</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].
<div style="clear:both"></div>


'''Structural Basis for Substrate Fatty-Acyl Chain Specificity: Crystal Structure of Human Very-Long-Chain Acyl-CoA Dehydrogenase'''
==See Also==
 
*[[Acyl-CoA dehydrogenase 3D structures|Acyl-CoA dehydrogenase 3D structures]]
 
== References ==
==About this Structure==
<references/>
3B96 is a [[Single protein]] structure of 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].
__TOC__
</StructureSection>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
[[Category: Single protein]]
[[Category: Large Structures]]
[[Category: He, M.]]
[[Category: He M]]
[[Category: Kim, J J.]]
[[Category: Kim JJ]]
[[Category: McAndrew, R P.]]
[[Category: McAndrew RP]]
[[Category: Mohsen, A W.]]
[[Category: Mohsen AW]]
[[Category: Vockley, J.]]
[[Category: Vockley J]]
[[Category: Wang, Y.]]
[[Category: Wang Y]]
[[Category: FAD]]
[[Category: MYA]]
[[Category: acetylation]]
[[Category: acyl-coa]]
[[Category: alternative splicing]]
[[Category: cardiomyopathy]]
[[Category: dehydrogenase]]
[[Category: disease mutation]]
[[Category: fad]]
[[Category: fatty acid beta-oxidation]]
[[Category: fatty acid metabolism]]
[[Category: flavoprotein]]
[[Category: lipid metabolism]]
[[Category: membrane]]
[[Category: mitochondria]]
[[Category: mitochondrion]]
[[Category: oxidoreductase]]
[[Category: polymorphism]]
[[Category: transit peptide]]
[[Category: ubl conjugation]]
[[Category: very long chain]]
 
''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu Mar 20 18:57:15 2008''
Retrieved from "https://proteopedia.org/3b96"