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1egd

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{{STRUCTURE_1egd| PDB=1egd | SCENE= }}
 
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===STRUCTURE OF T255E, E376G MUTANT OF HUMAN MEDIUM CHAIN ACYL-COA DEHYDROGENASE===
 
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{{ABSTRACT_PUBMED_8823176}}
 
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==Disease==
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==STRUCTURE OF T255E, E376G MUTANT OF HUMAN MEDIUM CHAIN ACYL-COA DEHYDROGENASE==
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[[http://www.uniprot.org/uniprot/ACADM_HUMAN ACADM_HUMAN]] Defects in ACADM are the cause of acyl-CoA dehydrogenase medium-chain deficiency (ACADMD) [MIM:[http://omim.org/entry/201450 201450]]. It is an autosomal recessive disease which causes fasting hypoglycemia, hepatic dysfunction, and encephalopathy, often resulting in death in infancy.<ref>PMID:2393404</ref><ref>PMID:2394825</ref><ref>PMID:2251268</ref><ref>PMID:1684086</ref><ref>PMID:1902818</ref><ref>PMID:1671131</ref><ref>PMID:8198141</ref><ref>PMID:7603790</ref><ref>PMID:7929823</ref><ref>PMID:9158144</ref><ref>PMID:9882619</ref><ref>PMID:10767181</ref><ref>PMID:11349232</ref><ref>PMID:11409868</ref><ref>PMID:11486912</ref>
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<StructureSection load='1egd' size='340' side='right'caption='[[1egd]], [[Resolution|resolution]] 2.40&Aring;' scene=''>
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== Structural highlights ==
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==Function==
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<table><tr><td colspan='2'>[[1egd]] is a 4 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=1EGD OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=1EGD FirstGlance]. <br>
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[[http://www.uniprot.org/uniprot/ACADM_HUMAN ACADM_HUMAN]] This enzyme is specific for acyl chain lengths of 4 to 16.
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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]] 2.4&#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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==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=1egd FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1egd OCA], [https://pdbe.org/1egd PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=1egd RCSB], [https://www.ebi.ac.uk/pdbsum/1egd PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=1egd ProSAT]</span></td></tr>
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[[1egd]] is a 4 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=1EGD OCA].
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</table>
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== Disease ==
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[https://www.uniprot.org/uniprot/ACADM_HUMAN ACADM_HUMAN] Defects in ACADM are the cause of acyl-CoA dehydrogenase medium-chain deficiency (ACADMD) [MIM:[https://omim.org/entry/201450 201450]. It is an autosomal recessive disease which causes fasting hypoglycemia, hepatic dysfunction, and encephalopathy, often resulting in death in infancy.<ref>PMID:2393404</ref> <ref>PMID:2394825</ref> <ref>PMID:2251268</ref> <ref>PMID:1684086</ref> <ref>PMID:1902818</ref> <ref>PMID:1671131</ref> <ref>PMID:8198141</ref> <ref>PMID:7603790</ref> <ref>PMID:7929823</ref> <ref>PMID:9158144</ref> <ref>PMID:9882619</ref> <ref>PMID:10767181</ref> <ref>PMID:11349232</ref> <ref>PMID:11409868</ref> <ref>PMID:11486912</ref>
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== Function ==
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[https://www.uniprot.org/uniprot/ACADM_HUMAN ACADM_HUMAN] This enzyme is specific for acyl chain lengths of 4 to 16.
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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/eg/1egd_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=1egd 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:008823176</ref><references group="xtra"/><references/>
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__TOC__
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</StructureSection>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Oxidoreductase]]
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[[Category: Large Structures]]
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[[Category: Ghisla, S.]]
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[[Category: Ghisla S]]
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[[Category: Kim, J P.]]
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[[Category: Kim JP]]
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[[Category: Lee, H J.]]
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[[Category: Lee HJ]]
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[[Category: Nandy, A.]]
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[[Category: Nandy A]]
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[[Category: Paschke, R.]]
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[[Category: Paschke R]]
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[[Category: Wang, M.]]
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[[Category: Wang M]]
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[[Category: Acyl-coa dehydrogenase]]
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[[Category: Electron transfer]]
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[[Category: Flavoprotein]]
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Current revision

STRUCTURE OF T255E, E376G MUTANT OF HUMAN MEDIUM CHAIN ACYL-COA DEHYDROGENASE

PDB ID 1egd

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