9uh6

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(New page: '''Unreleased structure''' The entry 9uh6 is ON HOLD Authors: Description: Category: Unreleased Structures)
Current revision (07:52, 19 November 2025) (edit) (undo)
 
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'''Unreleased structure'''
 
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The entry 9uh6 is ON HOLD
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==BCCP-CT Conformation of ADP-bound hPCC==
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<StructureSection load='9uh6' size='340' side='right'caption='[[9uh6]], [[Resolution|resolution]] 3.10&Aring;' scene=''>
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== Structural highlights ==
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<table><tr><td colspan='2'>[[9uh6]] is a 3 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=9UH6 OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=9UH6 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">Electron Microscopy, [[Resolution|Resolution]] 3.1&#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=1VU:PROPIONYL+COENZYME+A'>1VU</scene>, <scene name='pdbligand=ADP:ADENOSINE-5-DIPHOSPHATE'>ADP</scene>, <scene name='pdbligand=BTN:BIOTIN'>BTN</scene>, <scene name='pdbligand=MG:MAGNESIUM+ION'>MG</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=9uh6 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=9uh6 OCA], [https://pdbe.org/9uh6 PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=9uh6 RCSB], [https://www.ebi.ac.uk/pdbsum/9uh6 PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=9uh6 ProSAT]</span></td></tr>
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</table>
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== Disease ==
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[https://www.uniprot.org/uniprot/PCCA_HUMAN PCCA_HUMAN] Defects in PCCA are the cause of propionic acidemia type I (PA-1) [MIM:[https://omim.org/entry/606054 606054]. PA-1 is a life-threatening disease characterized by episodic vomiting, lethargy and ketosis, neutropenia, periodic thrombocytopenia, hypogammaglobulinemia, developmental retardation, and intolerance to protein.<ref>PMID:10101253</ref> <ref>PMID:12559849</ref> <ref>PMID:15059621</ref> <ref>PMID:10329019</ref>
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== Function ==
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[https://www.uniprot.org/uniprot/PCCA_HUMAN PCCA_HUMAN]
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<div style="background-color:#fffaf0;">
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== Publication Abstract from PubMed ==
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Propionyl-CoA carboxylase (PCC) is a biotin-dependent mitochondrial enzyme responsible for propionyl-CoA catabolism. Deficiencies in human PCC (hPCC) cause propionic acidemia, a severe metabolic disorder driven by toxic metabolite accumulation. Despite its therapeutic relevance, the structural basis of hPCC's catalytic function remains unresolved. Here, we present high-resolution cryo-EM structures of hPCC in four distinct states, unliganded, ADP-, AMPPNP-, and ATP-bound/substrate-bound, capturing the full trajectory of the biotin carboxyl carrier protein (BCCP) domain as it translocates between active sites. Our results reinforce the crucial role of nucleotide-gated B-lid subdomain in synchronizing catalysis through coupling with BCCP movement. Structural and biochemical analysis of 10 disease-associated variants reveals how mutations disrupt key domain interfaces and dynamic motions required for activity. These new insights define the mechanistic principles governing hPCC functions, establish a structural framework for understanding PCC-related disorders, and lay the groundwork for future efforts to engineer functional replacements or modulators for metabolic therapy.
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Authors:
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Nanoscale conformational dynamics of human propionyl-CoA carboxylase.,Yan H, Ni F, Wang Q, Ma J Structure. 2025 Nov 5:S0969-2126(25)00396-X. doi: 10.1016/j.str.2025.10.009. PMID:41197621<ref>PMID:41197621</ref>
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Description:
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From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
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[[Category: Unreleased Structures]]
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</div>
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<div class="pdbe-citations 9uh6" style="background-color:#fffaf0;"></div>
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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: Ma JP]]
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[[Category: Ni FY]]
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[[Category: Wang QH]]
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[[Category: Yan HF]]

Current revision

BCCP-CT Conformation of ADP-bound hPCC

PDB ID 9uh6

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