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1ivh

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{{Seed}}
 
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[[Image:1ivh.png|left|200px]]
 
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==STRUCTURE OF HUMAN ISOVALERYL-COA DEHYDROGENASE AT 2.6 ANGSTROMS RESOLUTION: STRUCTURAL BASIS FOR SUBSTRATE SPECIFICITY==
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The line below this paragraph, containing "STRUCTURE_1ivh", creates the "Structure Box" on the page.
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<StructureSection load='1ivh' size='340' side='right'caption='[[1ivh]], [[Resolution|resolution]] 2.60&Aring;' scene=''>
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You may change the PDB parameter (which sets the PDB file loaded into the applet)
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== Structural highlights ==
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or the SCENE parameter (which sets the initial scene displayed when the page is loaded),
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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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or leave the SCENE parameter empty for the default display.
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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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{{STRUCTURE_1ivh| PDB=1ivh | SCENE= }}
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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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===STRUCTURE OF HUMAN ISOVALERYL-COA DEHYDROGENASE AT 2.6 ANGSTROMS RESOLUTION: STRUCTURAL BASIS FOR SUBSTRATE SPECIFICITY===
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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/>
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The line below this paragraph, {{ABSTRACT_PUBMED_9214289}}, adds the Publication Abstract to the page
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__TOC__
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(as it appears on PubMed at http://www.pubmed.gov), where 9214289 is the PubMed ID number.
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</StructureSection>
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{{ABSTRACT_PUBMED_9214289}}
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==Disease==
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Known disease associated with this structure: Isovaleric acidemia OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=607036 607036]]
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==About this Structure==
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1IVH is a 4 chains structure of sequences 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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==Reference==
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<ref group="xtra">PMID:9214289</ref><references group="xtra"/>
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[[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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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Wed Feb 18 08:58:07 2009''
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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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