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1ivh

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{{STRUCTURE_1ivh| PDB=1ivh | SCENE= }}
 
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===STRUCTURE OF HUMAN ISOVALERYL-COA DEHYDROGENASE AT 2.6 ANGSTROMS RESOLUTION: STRUCTURAL BASIS FOR SUBSTRATE SPECIFICITY===
 
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{{ABSTRACT_PUBMED_9214289}}
 
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==Disease==
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==STRUCTURE OF HUMAN ISOVALERYL-COA DEHYDROGENASE AT 2.6 ANGSTROMS RESOLUTION: STRUCTURAL BASIS FOR SUBSTRATE SPECIFICITY==
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[[http://www.uniprot.org/uniprot/IVD_HUMAN IVD_HUMAN]] Defects in IVD are the cause of isovaleric acidemia (IVA) [MIM:[http://omim.org/entry/243500 243500]]. IVA is characterized by retarded psychomotor development, a peculiar odor resembling sweaty feet, an aversion to dietary protein, and pernicious vomiting, leading to acidosis and coma. The acute neonatal form leads to massive metabolic acidosis from the first days of life and rapid death.<ref>PMID:2063866</ref><ref>PMID:9665741</ref>
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<StructureSection load='1ivh' size='340' side='right'caption='[[1ivh]], [[Resolution|resolution]] 2.60&Aring;' scene=''>
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== Structural highlights ==
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<table><tr><td colspan='2'>[[1ivh]] 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=1IVH OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=1IVH 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]] 2.6&#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=COS:COENZYME+A+PERSULFIDE'>COS</scene>, <scene name='pdbligand=FAD:FLAVIN-ADENINE+DINUCLEOTIDE'>FAD</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=1ivh FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1ivh OCA], [https://pdbe.org/1ivh PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=1ivh RCSB], [https://www.ebi.ac.uk/pdbsum/1ivh PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=1ivh ProSAT]</span></td></tr>
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</table>
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== Disease ==
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[https://www.uniprot.org/uniprot/IVD_HUMAN IVD_HUMAN] Defects in IVD are the cause of isovaleric acidemia (IVA) [MIM:[https://omim.org/entry/243500 243500]. IVA is characterized by retarded psychomotor development, a peculiar odor resembling sweaty feet, an aversion to dietary protein, and pernicious vomiting, leading to acidosis and coma. The acute neonatal form leads to massive metabolic acidosis from the first days of life and rapid death.<ref>PMID:2063866</ref> <ref>PMID:9665741</ref>
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== Function ==
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[https://www.uniprot.org/uniprot/IVD_HUMAN IVD_HUMAN]
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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/iv/1ivh_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=1ivh 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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[[1ivh]] 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=1IVH OCA].
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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:009214289</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: Isovaleryl-CoA dehydrogenase]]
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[[Category: Large Structures]]
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[[Category: Kim, J J.P.]]
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[[Category: Kim JJP]]
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[[Category: Mohsen, A W.A.]]
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[[Category: Mohsen A-WA]]
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[[Category: Paschke, R.]]
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[[Category: Paschke R]]
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[[Category: Roberts, D L.]]
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[[Category: Roberts DL]]
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[[Category: Tiffany, K A.]]
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[[Category: Tiffany KA]]
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[[Category: Vockley, J.]]
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[[Category: Vockley J]]
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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: Flavoprotein]]
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[[Category: Isovaleric acidemia]]
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[[Category: Isovaleryl-coa]]
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[[Category: Oxidoreductase]]
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Current revision

STRUCTURE OF HUMAN ISOVALERYL-COA DEHYDROGENASE AT 2.6 ANGSTROMS RESOLUTION: STRUCTURAL BASIS FOR SUBSTRATE SPECIFICITY

PDB ID 1ivh

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