6pce: Difference between revisions
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The entry | ==Human Coa6== | ||
<StructureSection load='6pce' size='340' side='right'caption='[[6pce]], [[Resolution|resolution]] 1.65Å' scene=''> | |||
== Structural highlights == | |||
<table><tr><td colspan='2'>[[6pce]] is a 2 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=6PCE OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=6PCE FirstGlance]. <br> | |||
</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene></td></tr> | |||
<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=6pce FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6pce OCA], [http://pdbe.org/6pce PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=6pce RCSB], [http://www.ebi.ac.uk/pdbsum/6pce PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=6pce ProSAT]</span></td></tr> | |||
</table> | |||
== Disease == | |||
[[http://www.uniprot.org/uniprot/COA6_HUMAN COA6_HUMAN]] Fatal infantile cytochrome C oxidase deficiency. The disease is caused by mutations affecting the gene represented in this entry. | |||
== Function == | |||
[[http://www.uniprot.org/uniprot/COA6_HUMAN COA6_HUMAN]] Involved in the maturation of the mitochondrial respiratory chain complex IV subunit MT-CO2/COX2. Thereby, may regulate early steps of complex IV assembly. Mitochondrial respiratory chain complex IV or cytochrome c oxidase is the component of the respiratory chain that catalyzes the transfer of electrons from intermembrane space cytochrome c to molecular oxygen in the matrix and as a consequence contributes to the proton gradient involved in mitochondrial ATP synthesis. May also be required for efficient formation of respiratory supercomplexes comprised of complexes III and IV.<ref>PMID:24549041</ref> <ref>PMID:25959673</ref> <ref>PMID:26160915</ref> | |||
<div style="background-color:#fffaf0;"> | |||
== Publication Abstract from PubMed == | |||
Assembly factors play key roles in the biogenesis of many multi-subunit protein complexes regulating their stability, activity, and the incorporation of essential cofactors. The human assembly factor Coa6 participates in the biogenesis of the CuA site in complex IV (cytochrome c oxidase, COX). Patients with mutations in Coa6 suffer from mitochondrial disease due to complex IV deficiency. Here, we present the crystal structures of human Coa6 and the pathogenic (W59C)Coa6-mutant protein. These structures show that Coa6 has a 3-helical bundle structure, with the first 2 helices tethered by disulfide bonds, one of which likely provides the copper-binding site. Disulfide-mediated oligomerization of the (W59C)Coa6 protein provides a structural explanation for the loss-of-function mutation. | |||
Structural and functional characterization of the mitochondrial complex IV assembly factor Coa6.,Maghool S, Cooray NDG, Stroud DA, Aragao D, Ryan MT, Maher MJ Life Sci Alliance. 2019 Sep 12;2(5). pii: 2/5/e201900458. doi:, 10.26508/lsa.201900458. Print 2019 Oct. PMID:31515291<ref>PMID:31515291</ref> | |||
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.<br> | |||
[[Category: | </div> | ||
<div class="pdbe-citations 6pce" style="background-color:#fffaf0;"></div> | |||
== References == | |||
<references/> | |||
__TOC__ | |||
</StructureSection> | |||
[[Category: Large Structures]] | |||
[[Category: Maghool, S]] | |||
[[Category: Maher, M J]] | |||
[[Category: Coa6 copper cytochrome c oxidase mitochondria structure]] | |||
[[Category: Metal binding protein]] | |||
