8xl6

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m (Protected "8xl6" [edit=sysop:move=sysop])
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'''Unreleased structure'''
 
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The entry 8xl6 is ON HOLD
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==Structure of human 3-methylcrotonyl-CoA carboxylase at apo-state (MCC-Apo)==
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<StructureSection load='8xl6' size='340' side='right'caption='[[8xl6]], [[Resolution|resolution]] 2.29&Aring;' scene=''>
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Authors: Zhou, F.Y., Zhang, Y.Y., Zhou, Q., Hu, Q.
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== Structural highlights ==
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<table><tr><td colspan='2'>[[8xl6]] is a 12 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=8XL6 OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=8XL6 FirstGlance]. <br>
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Description: Structure of human 3-methylcrotonyl-CoA carboxylase at apo-state (MCC-Apo)
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</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">Electron Microscopy, [[Resolution|Resolution]] 2.29&#8491;</td></tr>
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[[Category: Unreleased Structures]]
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<tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=BTN:BIOTIN'>BTN</scene></td></tr>
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[[Category: Hu, Q]]
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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=8xl6 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=8xl6 OCA], [https://pdbe.org/8xl6 PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=8xl6 RCSB], [https://www.ebi.ac.uk/pdbsum/8xl6 PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=8xl6 ProSAT]</span></td></tr>
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[[Category: Zhou, F.Y]]
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</table>
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[[Category: Zhang, Y.Y]]
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== Disease ==
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[[Category: Zhou, Q]]
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[https://www.uniprot.org/uniprot/MCCA_HUMAN MCCA_HUMAN] Defects in MCCC1 are the cause of methylcrotonoyl-CoA carboxylase 1 deficiency (MCC1D) [MIM:[https://omim.org/entry/210200 210200]. An autosomal recessive disorder of leucine catabolism. The phenotype is variable, ranging from neonatal onset with severe neurological involvement to asymptomatic adults. There is a characteristic organic aciduria with massive excretion of 3-hydroxyisovaleric acid and 3-methylcrotonylglycine, usually in combination with a severe secondary carnitine deficiency.<ref>PMID:11170888</ref> <ref>PMID:11406611</ref> <ref>PMID:11181649</ref> <ref>PMID:22150417</ref>
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== Function ==
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[https://www.uniprot.org/uniprot/MCCA_HUMAN MCCA_HUMAN]
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== References ==
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<references/>
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__TOC__
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</StructureSection>
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[[Category: Homo sapiens]]
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[[Category: Large Structures]]
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[[Category: Hu Q]]
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[[Category: Zhang YY]]
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[[Category: Zhou FY]]
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[[Category: Zhou Q]]

Current revision

Structure of human 3-methylcrotonyl-CoA carboxylase at apo-state (MCC-Apo)

PDB ID 8xl6

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