Gene Symbol: COX17
Description: copper metallochaperone COX17
Alias: copper metallochaperone COX17
Species: Saccharomyces cerevisiae S288c
Products:     COX17

Top Publications

  1. Glerum D, Shtanko A, Tzagoloff A. Characterization of COX17, a yeast gene involved in copper metabolism and assembly of cytochrome oxidase. J Biol Chem. 1996;271:14504-9 pubmed
    Mutations in the COX17 gene of Saccharomyces cerevisiae cause a respiratory deficiency due to a block in the production of a functional cytochrome oxidase complex...
  2. Chacinska A, Pfannschmidt S, Wiedemann N, Kozjak V, Sanjuán Szklarz L, Schulze Specking A, et al. Essential role of Mia40 in import and assembly of mitochondrial intermembrane space proteins. EMBO J. 2004;23:3735-46 pubmed
    ..We conclude that Mia40 is a central component of the protein import and assembly machinery of the mitochondrial IMS. ..
  3. Olivas W, Parker R. The Puf3 protein is a transcript-specific regulator of mRNA degradation in yeast. EMBO J. 2000;19:6602-11 pubmed
    ..Examination of COX17 mRNA indicates that Puf3p specifically promotes decay of this mRNA by enhancing the rate of deadenylation and ..
  4. Mesecke N, Terziyska N, Kozany C, Baumann F, Neupert W, Hell K, et al. A disulfide relay system in the intermembrane space of mitochondria that mediates protein import. Cell. 2005;121:1059-69 pubmed
    ..We suggest that this process reflects the evolutionary origin of the IMS from the periplasmic space of the prokaryotic ancestors of mitochondria. ..
  5. Zhu D, Stumpf C, Krahn J, Wickens M, Hall T. A 5' cytosine binding pocket in Puf3p specifies regulation of mitochondrial mRNAs. Proc Natl Acad Sci U S A. 2009;106:20192-7 pubmed publisher
    ..Yeast Puf3p binds its own RNAs because they possess a -2C and is excluded from those of Puf4p which contain an additional nucleotide in the core-binding site. ..
  6. Beers J, Glerum D, Tzagoloff A. Purification, characterization, and localization of yeast Cox17p, a mitochondrial copper shuttle. J Biol Chem. 1997;272:33191-6 pubmed
    ..In the present study COX17 has been placed under the control of the GAL10 promoter in an autonomously replicating plasmid...
  7. Arnesano F, Balatri E, Banci L, Bertini I, Winge D. Folding studies of Cox17 reveal an important interplay of cysteine oxidation and copper binding. Structure. 2005;13:713-22 pubmed
    b>Cox17 is a key mitochondrial copper chaperone involved in the assembly of cytochrome c oxidase (COX)...
  8. Srinivasan C, Posewitz M, George G, Winge D. Characterization of the copper chaperone Cox17 of Saccharomyces cerevisiae. Biochemistry. 1998;37:7572-7 pubmed
    Assembly of functional cytochrome oxidase in yeast requires Cox17, which has been postulated to deliver copper ions to the mitochondrion for insertion into the enzyme...
  9. Heaton D, George G, Garrison G, Winge D. The mitochondrial copper metallochaperone Cox17 exists as an oligomeric, polycopper complex. Biochemistry. 2001;40:743-51 pubmed
    b>Cox17 is the candidate copper metallochaperone for delivery of copper ions to the mitochondrion for assembly of cytochrome c oxidase...

More Information


  1. Bragoszewski P, Gornicka A, Sztolsztener M, Chacinska A. The ubiquitin-proteasome system regulates mitochondrial intermembrane space proteins. Mol Cell Biol. 2013;33:2136-48 pubmed publisher
    ..Thus, the ubiquitin-proteasome system plays an important role in determining the levels of proteins targeted to the intermembrane space of mitochondria. ..
  2. Koch J, Schmid F. Mia40 targets cysteines in a hydrophobic environment to direct oxidative protein folding in the mitochondria. Nat Commun. 2014;5:3041 pubmed publisher
    ..Here, we employ Cox17 to elucidate the molecular determinants of this reaction...
  3. Miller M, Russo J, Fischer A, Lopez Leban F, Olivas W. Carbon source-dependent alteration of Puf3p activity mediates rapid changes in the stabilities of mRNAs involved in mitochondrial function. Nucleic Acids Res. 2014;42:3954-70 pubmed publisher
    ..However, only COX17 has been established as a target of Puf3p-mediated deadenylation and decapping...
  4. Sideris D, Petrakis N, Katrakili N, Mikropoulou D, Gallo A, Ciofi Baffoni S, et al. A novel intermembrane space-targeting signal docks cysteines onto Mia40 during mitochondrial oxidative folding. J Cell Biol. 2009;187:1007-22 pubmed publisher
  5. Chojnacka M, Gornicka A, Oeljeklaus S, Warscheid B, Chacinska A. Cox17 Protein Is an Auxiliary Factor Involved in the Control of the Mitochondrial Contact Site and Cristae Organizing System. J Biol Chem. 2015;290:15304-12 pubmed publisher
    ..Here, we report a direct link between Cox17, a protein involved in the assembly of cytochrome c oxidase, and the MICOS complex...
  6. Yosefzon Y, Koh Y, Chritton J, Lande A, Leibovich L, Barziv L, et al. Divergent RNA binding specificity of yeast Puf2p. RNA. 2011;17:1479-88 pubmed publisher
    ..This study expands the repertoire of cis elements bound by PUF proteins and suggests new modes by which PUF proteins recognize their mRNA targets. ..
  7. Rigby K, Cobine P, Khalimonchuk O, Winge D. Mapping the functional interaction of Sco1 and Cox2 in cytochrome oxidase biogenesis. J Biol Chem. 2008;283:15015-22 pubmed publisher
    ..The structural dynamics of loop 8 in Sco1 suggests it may be an interface for interactions with Cox17, the Cu(I) donor and/or Cox2...
  8. Glerum D, Shtanko A, Tzagoloff A. SCO1 and SCO2 act as high copy suppressors of a mitochondrial copper recruitment defect in Saccharomyces cerevisiae. J Biol Chem. 1996;271:20531-5 pubmed
    ..defective mutant of Saccharomyces cerevisiae arrested in cytochrome oxidase assembly due to a mutation in COX17, a nuclear gene encoding a low molecular weight cytoplasmic protein proposed to function in mitochondrial copper ..
  9. Huang Z, Chen K, Xu T, Zhang J, Li Y, Li W, et al. Sampangine inhibits heme biosynthesis in both yeast and human. Eukaryot Cell. 2011;10:1536-44 pubmed publisher
    ..This study also reveals a surprising essential role for the interaction between the mitochondrial ATP synthase and the electron transport chain. ..
  10. Horng Y, Cobine P, Maxfield A, Carr H, Winge D. Specific copper transfer from the Cox17 metallochaperone to both Sco1 and Cox11 in the assembly of yeast cytochrome C oxidase. J Biol Chem. 2004;279:35334-40 pubmed
    The assembly of the copper sites in cytochrome c oxidase involves a series of accessory proteins, including Cox11, Cox17, and Sco1...