Article
COA6 Facilitates Cytochrome c Oxidase Biogenesis as Thiol-reductase for Copper Metallochaperones in Mitochondria.
Journal of molecular biology - 27 Mar 2020
Pacheu-Grau David, Wasilewski Michał, Oeljeklaus Silke, Gibhardt Christine Silvia, Aich Abhishek, Chudenkova Margarita, Dennerlein Sven, Deckers Markus, Bogeski Ivan, Warscheid Bettina, Chacinska Agnieszka, Rehling Peter
Abstract excerpt
The mitochondrial cytochrome c oxidase, the terminal enzyme of the respiratory chain, contains heme and copper centers for electron transfer. The conserved COX2 subunit contains the CuA site, a binuclear copper center. The copper chaperones SCO1, SCO2, and COA6, are required for CuA center formation. Loss of function of these chaperones and the concomitant cytochrome c oxidase deficiency cause severe human...
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