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The Human Cytochrome c Oxidase Assembly Factors SCO1 and SCO2 Have Regulatory Roles in the Maintenance of Cellular Copper Homeostasis
Open accessAbstract
Human SCO1 and SCO2 are metallochaperones that are essential for the assembly of the catalytic core of cytochrome c oxidase (COX). Here we show that they have additional, unexpected roles in cellular copper homeostasis. Mutations in either SCO result in a cellular copper deficiency that is both tissue and allele specific. This phenotype can be dissociated from the defects in COX assembly and is suppressed by overexpression of SCO2, but not SCO1. Overexpression of a SCO1 mutant in control cells in which wild-type SCO1 levels were reduced by shRNA recapitulates the copper-deficiency phenotype in SCO1 patient cells. The copper-deficiency phenotype reflects not a change in high-affinity copper uptake but rather a proportional increase in copper efflux. These results suggest a mitochondrial pathway for the regulation of cellular copper content that involves signaling through SCO1 and SCO2, perhaps by their thiol redox or metal-binding state.
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Citations
Cited by
Copper and iron disorders of the brain
Madsen, E., & Gitlin, J. D. (2007) · Annual Review of Neuroscience · doi:10.1146/annurev.neuro.30.051606.094232 · Cited by 557
Mitochondrial copper metabolism and delivery to cytochrome c oxidase
Horn, D., & Barrientos, A. (2008) · IUBMB Life · doi:10.1002/iub.50 · Cited by 283
Assembly of mammalian oxidative phosphorylation complexes I–V and supercomplexes
Signes, A., & Fernandez-Vizarra, E. (2018) · Essays in Biochemistry · doi:10.1042/ebc20170098 · Cited by 267
Fernández-Vizarra, E., Tiranti, V., & Zeviani, M. (2009) · Biochimica et Biophysica Acta (BBA) - Molecular Cell Research · doi:10.1016/j.bbamcr.2008.05.028 · Cited by 214
Ikeda, K., Shiba, S., Horie-Inoue, K., Shimokata, K., & Inoue, S. (2013) · Nature Communications · doi:10.1038/ncomms3147 · Cited by 152
Metadata
- Authors
- Leary, S. C., Cobine, P. A., Kaufman, B. A., Guercin, G.-H., Mattman, A., Palaty, J., Lockitch, G., Winge, D. R., Rustin, P., Horvath, R., & Shoubridge, E. A.
- Venue
- Cell Metabolism
- Publisher
- Elsevier BV
- Published
- 2007-01
- Volume
- 5
- Issue
- 1
- Pages
- 9-20
- ISSN
- 1550-4131 (ISSN-L)
- OpenAlex
- W1984164475
- Cited by
- 225
- References
- 0
- FWCI
- 14.25
- Type
- journal article
- Language
- en
- Open access
- bronze
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- CoChat. (2026). Citation check: The Human Cytochrome c Oxidase Assembly Factors SCO1 and SCO2 Have Regulatory Roles in the Maintenance of Cellular Copper Homeostasis. https://app.cochat.ai/papers/10.1016/j.cmet.2006.12.001
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- Leary, S. C., Cobine, P. A., Kaufman, B. A., Guercin, G.-H., Mattman, A., Palaty, J., Lockitch, G., Winge, D. R., Rustin, P., Horvath, R., & Shoubridge, E. A. (2007). The Human Cytochrome c Oxidase Assembly Factors SCO1 and SCO2 Have Regulatory Roles in the Maintenance of Cellular Copper Homeostasis. Cell Metabolism. https://doi.org/10.1016/j.cmet.2006.12.001
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