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  Cooperation between COA6 and SCO2 in COX2 maturation during cytochrome c oxidase assembly links two mitochondrial cardiomyopathies.

Pacheu-Grau, D., Bareth, B., Dudek, J., Juris, L., Vogtle, F. N., Wissel, M., et al. (2015). Cooperation between COA6 and SCO2 in COX2 maturation during cytochrome c oxidase assembly links two mitochondrial cardiomyopathies. Cell Metabolism, 21(6), 823-833. doi:10.1016/j.cmet.2015.04.012.

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Item Permalink: http://hdl.handle.net/11858/00-001M-0000-0027-A787-4 Version Permalink: http://hdl.handle.net/11858/00-001M-0000-002A-51AB-E
Genre: Journal Article

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 Creators:
Pacheu-Grau, D., Author
Bareth, B., Author
Dudek, J., Author
Juris, L., Author
Vogtle, F. N., Author
Wissel, M., Author
Leary, S. C., Author
Dennerlein, S., Author
Rehling, P.1, Author              
Deckers, M., Author
Affiliations:
1Max Planck Fellow Peter Rehling, ou_1298545              

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 Abstract: Three mitochondria-encoded subunits form the catalytic core of cytochrome c oxidase, the terminal enzyme of the respiratory chain. COX1 and COX2 contain heme and copper redox centers, which are integrated during assembly of the enzyme. Defects in this process lead to an enzyme deficiency and manifest as mitochondrial disorders in humans. Here we demonstrate that COA6 is specifically required for COX2 biogenesis. Absence of COA6 leads to fast turnover of newly synthesized COX2 and a concomitant reduction in cytochrome c oxidase levels. COA6 interacts transiently with the copper-containing catalytic domain of newly synthesized COX2. Interestingly, similar to the copper metallochaperone SCO2, loss of COA6 causes cardiomyopathy in humans. We show that COA6 and SCO2 interact and that corresponding pathogenic mutations in each protein affect complex formation. Our analyses define COA6 as a constituent of the mitochondrial copper relay system, linking defects in COX2 metallation to cardiac cytochrome c oxidase deficiency.

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Language(s): eng - English
 Dates: 2015-05-072015-06-02
 Publication Status: Published in print
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 Rev. Method: Peer
 Identifiers: DOI: 10.1016/j.cmet.2015.04.012
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Title: Cell Metabolism
Source Genre: Journal
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Pages: - Volume / Issue: 21 (6) Sequence Number: - Start / End Page: 823 - 833 Identifier: -