View source for 7mqz


==Cytochrome c oxidase assembly factor 7==
<StructureSection load='7mqz' size='340' side='right'caption='[[7mqz]], [[Resolution|resolution]] 2.39&Aring;' scene=''>
== Structural highlights ==
<table><tr><td colspan='2'>[[7mqz]] is a 1 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=7MQZ OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=7MQZ FirstGlance]. <br>
</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">X-ray diffraction, [[Resolution|Resolution]] 2.39&#8491;</td></tr>
<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=7mqz FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=7mqz OCA], [https://pdbe.org/7mqz PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=7mqz RCSB], [https://www.ebi.ac.uk/pdbsum/7mqz PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=7mqz ProSAT]</span></td></tr>
</table>
== Disease ==
[https://www.uniprot.org/uniprot/COA7_HUMAN COA7_HUMAN] The disease is caused by variants affecting the gene represented in this entry.
== Function ==
[https://www.uniprot.org/uniprot/COA7_HUMAN COA7_HUMAN] Required for assembly of mitochondrial respiratory chain complex I and complex IV.<ref>PMID:24333015</ref> 
<div style="background-color:#fffaf0;">
== Publication Abstract from PubMed ==
Cytochrome c oxidase (COX) assembly factor 7 (COA7) is a metazoan-specific assembly factor, critical for the biogenesis of mitochondrial complex IV (cytochrome c oxidase). Although mutations in COA7 have been linked to complex IV assembly defects and neurological conditions such as peripheral neuropathy, ataxia, and leukoencephalopathy, the precise role COA7 plays in the biogenesis of complex IV is not known. Here, we show that loss of COA7 blocks complex IV assembly after the initial step where the COX1 module is built, progression from which requires the incorporation of copper and addition of the COX2 and COX3 modules. The crystal structure of COA7, determined to 2.4 A resolution, reveals a banana-shaped molecule composed of five helix-turn-helix (alpha/alpha) repeats, tethered by disulfide bonds. COA7 interacts transiently with the copper metallochaperones SCO1 and SCO2 and catalyzes the reduction of disulfide bonds within these proteins, which are crucial for copper relay to COX2. COA7 binds heme with micromolar affinity, through axial ligation to the central iron atom by histidine and methionine residues. We therefore propose that COA7 is a heme-binding disulfide reductase for regenerating the copper relay system that underpins complex IV assembly.

Mitochondrial COA7 is a heme-binding protein with disulfide reductase activity, which acts in the early stages of complex IV assembly.,Formosa LE, Maghool S, Sharpe AJ, Reljic B, Muellner-Wong L, Stroud DA, Ryan MT, Maher MJ Proc Natl Acad Sci U S A. 2022 Mar 1;119(9). pii: 2110357119. doi:, 10.1073/pnas.2110357119. PMID:35210360<ref>PMID:35210360</ref>

From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
</div>
<div class="pdbe-citations 7mqz" style="background-color:#fffaf0;"></div>
== References ==
<references/>
__TOC__
</StructureSection>
[[Category: Homo sapiens]]
[[Category: Large Structures]]
[[Category: Maghool S]]
[[Category: Maher MJ]]