Metallochaperone inserts Cu2+ into MT-CO1

Stable Identifier
R-HSA-9865449
Type
Reaction [transition]
Species
Homo sapiens
Compartment
ReviewStatus
5/5
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A metallochaperone complex consisting of nine subunits (COA3, COA5, COX10, dimeric COX11, COX15, COX16, COX19, SCO1, SCO2) carries a copper cation from the outer side of the inner mitochondrial membrane, where it was delivered by the copper chaperone COX17, to the pre-assembled Complex IV on the inner side of the membrane, where it inserts the cation into the MT-CO1 subunit. This copper ion is frequently called CuB (Leary et al., 2013; Bode et al., 2015; Nývltová et al., 2022; reviewed in Ruiz et al., 2021).
Literature References
PubMed ID Title Journal Year
25926683 Redox-regulated dynamic interplay between Cox19 and the copper-binding protein Cox11 in the intermembrane space of mitochondria facilitates biogenesis of cytochrome c oxidase

Jung, M, Bode, M, Zimmermann, R, Schroda, M, Bohnert, M, Woellhaf, MW, van der Laan, M, Herrmann, JM, Sommer, F

Mol Biol Cell 2015
34504870 Role of Copper on Mitochondrial Function and Metabolism

Ruiz, LM, Elorza, AA, Libedinsky, A

Front Mol Biosci 2021
23345593 COX19 mediates the transduction of a mitochondrial redox signal from SCO1 that regulates ATP7A-mediated cellular copper efflux

de Coo, R, de Krijger, R, Shoubridge, EA, Winge, DR, Verdijk, RM, Nishimura, T, Cobine, PA, Leary, SC, Tarnopolsky, MA

Mol Biol Cell 2013
35750769 Coordination of metal center biogenesis in human cytochrome c oxidase

Dietz, JV, Nývltová, E, Seravalli, J, Barrientos, A, Khalimonchuk, O

Nat Commun 2022
Participants
Participates
Catalyst Activity

copper chaperone activity of metallochaperone complex [mitochondrial inner membrane]

Orthologous Events
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