COX17

Summary

Gene Symbol: COX17
Description: COX17, cytochrome c oxidase copper chaperone
Alias: cytochrome c oxidase copper chaperone, COX17 cytochrome c oxidase assembly homolog, cytochrome c oxidase 17 copper chaperone, cytochrome c oxidase assembly homolog 17, human homolog of yeast mitochondrial copper recruitment
Species: human
Products:     COX17

Top Publications

  1. Oswald C, Krause Buchholz U, Rödel G. Knockdown of human COX17 affects assembly and supramolecular organization of cytochrome c oxidase. J Mol Biol. 2009;389:470-9 pubmed publisher
    ..Here, we investigate the role of COX17 in the biogenesis of the respiratory chain in HeLa cells...
  2. Buapet P, Mohammadi N, Pernice M, Kumar M, Kuzhiumparambil U, Ralph P. Excess copper promotes photoinhibition and modulates the expression of antioxidant-related genes in Zostera muelleri. Aquat Toxicol. 2019;207:91-100 pubmed publisher
    ..expression level of genes encoding metallothionein type 2 (mt2), metallothionein type 3 (mt3) and cytochrome c oxidase copper chaperone (cox17)...
  3. Zhang Y, Zhang R, Sun H, Chen Q, Yu X, Zhang T, et al. Copper inhibits hatching of fish embryos via inducing reactive oxygen species and down-regulating Wnt signaling. Aquat Toxicol. 2018;205:156-164 pubmed publisher
    ..was observed between the control and Cu2+-treated group at 72 hpf (hours post fertilization) in cox17 mutant embryos, in which little ROS was producd after copper stimulation...
  4. NÃ¥bo L, Madsen C, Jensen K, Kongsted J, Astakhova K. Ultramild protein-mediated click chemistry creates efficient oligonucleotide probes for targeting and detecting nucleic acids. Chembiochem. 2015;16:1163-7 pubmed publisher
    ..For the first time, human copper(I)-binding chaperon Cox17 was applied to effectively catalyze click labeling of oligonucleotides...
  5. Choveaux D, Krause R, Przyborski J, Goldring J. Identification and initial characterisation of a Plasmodium falciparum Cox17 copper metallochaperone. Exp Parasitol. 2015;148:30-9 pubmed publisher
    ..A P. falciparum Cox17 ortholog (PfCox17) was recombinantly expressed and the purified protein bound reduced copper in vitro...
  6. Murtha K, Hwang M, Peccarelli M, Scott T, Kebaara B. The nonsense-mediated mRNA decay (NMD) pathway differentially regulates COX17, COX19 and COX23 mRNAs. Curr Genet. 2019;65:507-521 pubmed publisher
    The differential regulation of COX17, COX19 and COX23 mRNAs by the nonsense-mediated mRNA decay (NMD) pathway was investigated. The NMD pathway regulates mRNAs that aberrantly terminate translation...
  7. Kong P, Lei P, Zhang S, Li D, Zhao J, Zhang B. Integrated microarray analysis provided a new insight of the pathogenesis of Parkinson's disease. Neurosci Lett. 2018;662:51-58 pubmed publisher
    ..Several DEGs including SNCA, COX17, COX4I1, COX7B, COX6A1 and ATP5J targeted by Pax-4, Oct-1 and Evi-1 may involve in the neurodegeneration and ..
  8. 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...
  9. Garcia L, Welchen E, Gey U, Arce A, Steinebrunner I, Gonzalez D. The cytochrome c oxidase biogenesis factor AtCOX17 modulates stress responses in Arabidopsis. Plant Cell Environ. 2016;39:628-44 pubmed publisher
    b>COX17 is a soluble protein from the mitochondrial intermembrane space that participates in the transfer of copper for cytochrome c oxidase (COX) assembly in eukaryotic organisms...

More Information

Publications39

  1. Koch J, Schmid F. Mia40 combines thiol oxidase and disulfide isomerase activity to efficiently catalyze oxidative folding in mitochondria. J Mol Biol. 2014;426:4087-98 pubmed publisher
    ..By using Cox17 (a mitochondrial copper-binding protein) as a natural substrate, we discovered that, in the presence of Mia40, the ..
  2. Zhong Y, Zhang J, Yu H, Zhang J, Sun X, Chen W, et al. Characterization and sub-cellular localization of GalNAc-binding proteins isolated from human hepatic stellate cells. Biochem Biophys Res Commun. 2015;468:906-12 pubmed publisher
    ..For example, the GNBPs (i.e. BTF3, COX17, and ATP5A1) responsible for the regulation of protein binding were up-regulated, and those (i.e...
  3. Yin W, Li R, Feng X, James Kang Y. The Involvement of Cytochrome c Oxidase in Mitochondrial Fusion in Primary Cultures of Neonatal Rat Cardiomyocytes. Cardiovasc Toxicol. 2018;18:365-373 pubmed publisher
    ..Depression of CCO activity by siRNA targeting CCO assembly homolog 17 (COX17), a copper chaperone for CCO, led to fragmentation of mitochondria, which was not recoverable by copper ..
  4. Smirnova J, Kabin E, Järving I, Bragina O, Tougu V, Plitz T, et al. Copper(I)-binding properties of de-coppering drugs for the treatment of Wilson disease. α-Lipoic acid as a potential anti-copper agent. Sci Rep. 2018;8:1463 pubmed publisher
    ..ability to extract Cu(I) ions from two Cu(I)-binding proteins, the copper chaperone for cytochrome c oxidase, Cox17, and metallothionein...
  5. Schikora Tamarit M, Toscano Ochoa C, Domingo Espinós J, Espinar L, Carey L. A synthetic gene circuit for measuring autoregulatory feedback control. Integr Biol (Camb). 2016;8:546-55 pubmed publisher
    ..participate in any direct or indirect feedback regulation, but that replacing the native 3'UTR with that of COX17 generates an auto-regulatory negative feedback loop which reduces gene expression noise...
  6. Banci L, Bertini I, Cefaro C, Ciofi Baffoni S, Gallo A, Martinelli M, et al. MIA40 is an oxidoreductase that catalyzes oxidative protein folding in mitochondria. Nat Struct Mol Biol. 2009;16:198-206 pubmed publisher
    ..MIA40 represents a thioredoxin-unrelated, minimal oxidoreductase, with a facile CPC redox active site that ensures its catalytic function in oxidative folding in mitochondria. ..
  7. Horvath R, Lochmuller H, Stucka R, Yao J, Shoubridge E, Kim S, et al. Characterization of human SCO1 and COX17 genes in mitochondrial cytochrome-c-oxidase deficiency. Biochem Biophys Res Commun. 2000;276:530-3 pubmed
    ..Here we report on the genomic organization of SCO1 and COX17 on human chromosomes 17 and 3 respectively, and the complete sequence analysis of COX17 and SCO1 in 30 patients ..
  8. Kako K, Tsumori K, Ohmasa Y, Takahashi Y, Munekata E. The expression of Cox17p in rodent tissues and cells. Eur J Biochem. 2000;267:6699-707 pubmed
    ..These findings suggested that Cox17p is not only part of the respiratory chain but also involved in brain and endocrine functions. ..
  9. Szklarczyk R, Wanschers B, Cuypers T, Esseling J, Riemersma M, van den Brand M, et al. Iterative orthology prediction uncovers new mitochondrial proteins and identifies C12orf62 as the human ortholog of COX14, a protein involved in the assembly of cytochrome c oxidase. Genome Biol. 2012;13:R12 pubmed publisher
    ..Divergent homologs can often only be detected by comparing sequence profiles and profile-based hidden Markov models. The Ortho-Profile method takes advantage of these techniques in the quest for orthologs. ..
  10. Amaravadi R, Glerum D, Tzagoloff A. Isolation of a cDNA encoding the human homolog of COX17, a yeast gene essential for mitochondrial copper recruitment. Hum Genet. 1997;99:329-33 pubmed
    The COX17 gene of Saccharomyces cerevisiae codes for a cytoplasmic protein essential for the expression of functional cytochrome oxidase. This protein has been implicated in targeting copper to mitochondria...
  11. Sotgia F, Fiorillo M, Lisanti M. Mitochondrial markers predict recurrence, metastasis and tamoxifen-resistance in breast cancer patients: Early detection of treatment failure with companion diagnostics. Oncotarget. 2017;8:68730-68745 pubmed publisher
    ..In addition, we combined 4 mitochondrial proteins (HSPD1, UQCRB, MRPL15, COX17), to generate a compact mitochondrial gene signature, associated with a HR of 5.34 (p=1e-09)...
  12. 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...
  13. Zhang H, Wu X, Mehmood K, Chang Z, Li K, Jiang X, et al. Intestinal epithelial cell injury induced by copper containing nanoparticles in piglets. Environ Toxicol Pharmacol. 2017;56:151-156 pubmed publisher
    ..were assayed by differential expression levels of SOD, MDA and Metallothionein (MT) in addition to CTR1, SOD1, COX17, MT and ATOX1 genes expression...
  14. Suzuki C, Daigo Y, Kikuchi T, Katagiri T, Nakamura Y. Identification of COX17 as a therapeutic target for non-small cell lung cancer. Cancer Res. 2003;63:7038-41 pubmed
    ..Here we report evidence that the cytochrome c oxidase (CCO) assembly protein COX17 is a potential molecular target for treatment of lung cancers...
  15. Punter F, Adams D, Glerum D. Characterization and localization of human COX17, a gene involved in mitochondrial copper transport. Hum Genet. 2000;107:69-74 pubmed
    ..b>COX17 is such an assembly protein and is thought to recruit copper to mitochondria for incorporation into the COX ..
  16. Li L, Guo W, Wu K, Wu X, Zhao L, Zhao Y, et al. A comparative study on the interactions of human copper chaperone Cox17 with anticancer organoruthenium(II) complexes and cisplatin by mass spectrometry. J Inorg Biochem. 2016;161:99-106 pubmed publisher
    ..en=ethylenediamine, arene=p-cymene (1) or biphenyl (2)), and the human copper chaperone protein Cox17 by mass spectrometry with cisplatin as a reference...
  17. Mercatelli E, Barbieri L, Luchinat E, Banci L. Direct structural evidence of protein redox regulation obtained by in-cell NMR. Biochim Biophys Acta. 2016;1863:198-204 pubmed publisher
    ..Here, by in-cell NMR, we directly observe the redox state of three human proteins, Cox17, Mia40 and SOD1, in the cytoplasm of human and bacterial cells...
  18. Kako K, Takehara A, Arai H, Onodera T, Takahashi Y, Hanagata H, et al. A selective requirement for copper-dependent activation of cytochrome c oxidase by Cox17p. Biochem Biophys Res Commun. 2004;324:1379-85 pubmed
    ..required for mitochondrial oxidative phosphorylation but also is essential for embryonic growth and development in COX17 gene-deficient mice...
  19. Dong D, Xu X, Yin W, Kang Y. Changes in copper concentrations affect the protein levels but not the mRNA levels of copper chaperones in human umbilical vein endothelial cells. Metallomics. 2014;6:554-9 pubmed publisher
    ..and copper chaperone for superoxide dismutase 1 (CCS-1), but decreased copper chaperone for cytochrome c oxidase (COX17). In contrast, copper supplementation decreased the protein levels of ATOX1 and CCS-1 and increased COX17...
  20. Voronova A, Meyer Klaucke W, Meyer T, Rompel A, Krebs B, Kazantseva J, et al. Oxidative switches in functioning of mammalian copper chaperone Cox17. Biochem J. 2007;408:139-48 pubmed
    b>Cox17, a copper chaperone for cytochrome-c oxidase, is an essential and highly conserved protein in eukaryotic organisms...
  21. Zhao L, Wang Z, Wu H, Xi Z, Liu Y. Glutathione selectively modulates the binding of platinum drugs to human copper chaperone Cox17. Biochem J. 2015;472:217-23 pubmed publisher
    The copper chaperone Cox17 (cytochrome c oxidase copper chaperone) has been shown to facilitate the delivery of cisplatin to mitochondria, which contributes to the overall cytotoxicity of the drug [Zhao et al. (2014) Chem. Commun...
  22. Cheng J, Luo Z, Chen G, Wei C, Zhuo M. Identification of eight copper (Cu) uptake related genes from yellow catfish Pelteobagrus fulvidraco, and their tissue expression and transcriptional responses to dietborne Cu exposure. J Trace Elem Med Biol. 2017;44:256-265 pubmed publisher
    ..including two isoforms of copper transporter genes (ctr1 and ctr2), three copper chaperone genes (atox1, ccs and cox17), two Cu-ATPase genes (atp7a and atp7b) and divalent metal ion transporter 1 (dmt1), were cloned and characterized ..
  23. 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)...
  24. Cobine P, Pierrel F, Leary S, Sasarman F, Horng Y, Shoubridge E, et al. The P174L mutation in human Sco1 severely compromises Cox17-dependent metallation but does not impair copper binding. J Biol Chem. 2006;281:12270-6 pubmed
    ..The mutant protein retained the ability to bind Cu(I) and Cu(II) normally when expressed in bacteria, but Cox17-mediated copper transfer was severely compromised both in vitro and in a yeast cytoplasmic assay...
  25. Fraga H, Pujols J, Gil Garcia M, Roque A, Bernardo Seisdedos G, Santambrogio C, et al. Disulfide driven folding for a conditionally disordered protein. Sci Rep. 2017;7:16994 pubmed publisher
    ..Here we characterize the disorder-to-order transition of a Mia40 substrate, the human small copper chaperone Cox17. Using an integrated real-time approach, including chromatography, fluorescence, CD, FTIR, SAXS, NMR, and MS ..
  26. Banci L, Bertini I, Cefaro C, Ciofi Baffoni S, Gallo A. Functional role of two interhelical disulfide bonds in human Cox17 protein from a structural perspective. J Biol Chem. 2011;286:34382-90 pubmed publisher
    Human Cox17 is the mitochondrial copper chaperone responsible for supplying copper ions, through the assistance of Sco1, Sco2, and Cox11, to cytochrome c oxidase, the terminal enzyme of the mitochondrial energy-transducing respiratory ..
  27. Banci L, Bertini I, Ciofi Baffoni S, Hadjiloi T, Martinelli M, Palumaa P. Mitochondrial copper(I) transfer from Cox17 to Sco1 is coupled to electron transfer. Proc Natl Acad Sci U S A. 2008;105:6803-8 pubmed publisher
    The human protein Cox17 contains three pairs of cysteines. In the mitochondrial intermembrane space (IMS) it exists in a partially oxidized form with two S-S bonds and two reduced cysteines (HCox17(2S-S))...
  28. Banci L, Bertini I, Ciofi Baffoni S, Janicka A, Martinelli M, Kozlowski H, et al. A structural-dynamical characterization of human Cox17. J Biol Chem. 2008;283:7912-20 pubmed
    Human Cox17 is a key mitochondrial copper chaperone responsible for supplying copper ions, through the assistance of Sco1, Sco2, and Cox11, to cytochrome c oxidase, the terminal enzyme of the mitochondrial energy transducing respiratory ..
  29. Matson Dzebo M, Ariöz C, Wittung Stafshede P. Extended functional repertoire for human copper chaperones. Biomol Concepts. 2016;7:29-39 pubmed publisher
    ..e. CCS) and cytochrome c oxidase in mitochondria (i.e. Cox17). Many mechanistic aspects have been resolved with respect to how Cu ions are moved between these proteins...
  30. Voronova A, Kazantseva J, Tuuling M, Sokolova N, Sillard R, Palumaa P. Cox17, a copper chaperone for cytochrome c oxidase: expression, purification, and formation of mixed disulphide adducts with thiol reagents. Protein Expr Purif. 2007;53:138-44 pubmed
    Copper chaperone for cytochrome c oxidase (Cox17) is a 7 kDa copper-binding protein, which facilitates incorporation of copper ions into Cu(A) site of cytochrome c oxidase...