Gene Symbol: COX3
Description: cytochrome c oxidase subunit 3
Alias: OXI2, cytochrome c oxidase subunit 3
Species: Saccharomyces cerevisiae S288c

Top Publications

  1. Strogolova V, Furness A, Robb McGrath M, Garlich J, Stuart R. Rcf1 and Rcf2, members of the hypoxia-induced gene 1 protein family, are critical components of the mitochondrial cytochrome bc1-cytochrome c oxidase supercomplex. Mol Cell Biol. 2012;32:1363-73 pubmed publisher
    ..Rcf1 interacts with the Cox3 subunit and can do so prior to their assembly into the COX complex...
  2. Costanzo M, Seaver E, Fox T. At least two nuclear gene products are specifically required for translation of a single yeast mitochondrial mRNA. EMBO J. 1986;5:3637-41 pubmed
    Mitochondrial translation of the oxi2 mRNA, encoding yeast cytochrome c oxidase subunit III (coxIII), has previously been shown to specifically require the mitochondrially located protein product of the nuclear gene PET494...
  3. Foury F, Roganti T, Lecrenier N, Purnelle B. The complete sequence of the mitochondrial genome of Saccharomyces cerevisiae. FEBS Lett. 1998;440:325-31 pubmed
    ..Our data suggest that shuffling of mobile elements plays an important role in the remodelling of the yeast mitochondrial genome. ..
  4. Wiesenberger G, Costanzo M, Fox T. Analysis of the Saccharomyces cerevisiae mitochondrial COX3 mRNA 5' untranslated leader: translational activation and mRNA processing. Mol Cell Biol. 1995;15:3291-300 pubmed
    We used transformation of yeast mitochondria and homologous gene replacement to study features of the 613-base COX3 mRNA 5' untranslated leader (5'-UTL) required for translational activation by the protein products of the nuclear genes ..
  5. Rak M, Gokova S, Tzagoloff A. Modular assembly of yeast mitochondrial ATP synthase. EMBO J. 2011;30:920-30 pubmed publisher
    ..These studies show that assembly of the ATP synthase is not a single linear process, as previously thought, but rather involves two separate but coordinately regulated pathways that converge at the end stage. ..
  6. Chen Y, Taylor E, Dephoure N, Heo J, Tonhato A, Papandreou I, et al. Identification of a protein mediating respiratory supercomplex stability. Cell Metab. 2012;15:348-60 pubmed publisher
    ..We suggest that Rcf1 is a member of an evolutionarily conserved protein family that acts to promote respiratory supercomplex assembly and activity. ..
  7. Costanzo M, Fox T. Suppression of a defect in the 5' untranslated leader of mitochondrial COX3 mRNA by a mutation affecting an mRNA-specific translational activator protein. Mol Cell Biol. 1993;13:4806-13 pubmed
    Translation of the Saccharomyces cerevisiae mitochondrial COX3 mRNA, encoding subunit III of cytochrome c oxidase, specifically requires the action of the nuclear gene products PET54, PET122, and PET494 at a site encoded in the 612-base 5'..
  8. McStay G, Su C, Thomas S, Xu J, Tzagoloff A. Characterization of assembly intermediates containing subunit 1 of yeast cytochrome oxidase. J Biol Chem. 2013;288:26546-56 pubmed publisher
  9. Fontanesi F, SOTO I, Horn D, Barrientos A. Mss51 and Ssc1 facilitate translational regulation of cytochrome c oxidase biogenesis. Mol Cell Biol. 2010;30:245-59 pubmed publisher
    ..We conclude that Ssc1, by interacting with Mss51 and Mss51-containing complexes, plays a critical role in Cox1 biogenesis, COX assembly, and the translational regulation of these processes. ..

More Information


  1. McStay G, Su C, Tzagoloff A. Stabilization of Cox1p intermediates by the Cox14p-Coa3p complex. FEBS Lett. 2013;587:943-9 pubmed publisher
  2. Perez Martinez X, Butler C, Shingu Vazquez M, Fox T. Dual functions of Mss51 couple synthesis of Cox1 to assembly of cytochrome c oxidase in Saccharomyces cerevisiae mitochondria. Mol Biol Cell. 2009;20:4371-80 pubmed publisher
    ..Regulation of COX1 mRNA translation by Mss51 seems to be an example of a homeostatic mechanism in which a positive effector of gene expression interacts with the product it regulates in a posttranslational assembly process. ..
  3. Mick D, Wagner K, van der Laan M, Frazier A, Perschil I, Pawlas M, et al. Shy1 couples Cox1 translational regulation to cytochrome c oxidase assembly. EMBO J. 2007;26:4347-58 pubmed
    ..We suggest that Shy1 links Cox1 translational regulation to complex IV assembly and supercomplex formation. ..
  4. Horan S, Bourges I, Taanman J, Meunier B. Analysis of COX2 mutants reveals cytochrome oxidase subassemblies in yeast. Biochem J. 2005;390:703-8 pubmed
    ..The identification of these novel cytochrome oxidase subcomplexes should encourage the reexamination of other yeast mutants. ..
  5. de Zamaroczy M, Bernardi G. The primary structure of the mitochondrial genome of Saccharomyces cerevisiae--a review. Gene. 1986;47:155-77 pubmed
  6. Meunier B. Site-directed mutations in the mitochondrially encoded subunits I and III of yeast cytochrome oxidase. Biochem J. 2001;354:407-12 pubmed
    ..However, mutations Met-273-->Thr and Ile-280-->Thr were mildly deleterious, decreased cytochrome oxidase activity and slightly perturbed the properties of the binuclear centre. ..
  7. Hell K, Neupert W, Stuart R. Oxa1p acts as a general membrane insertion machinery for proteins encoded by mitochondrial DNA. EMBO J. 2001;20:1281-8 pubmed
    ..Taken together, we conclude that the Oxa1 complex represents a general membrane protein insertion machinery in the inner membrane of mitochondria. ..
  8. Williams E, Bsat N, Bonnefoy N, Butler C, Fox T. Alteration of a novel dispensable mitochondrial ribosomal small-subunit protein, Rsm28p, allows translation of defective COX2 mRNAs. Eukaryot Cell. 2005;4:337-45 pubmed
    ..The rsm28 null mutation also reduced translation of an ARG8m reporter sequence inserted at the COX1, COX2, and COX3 mitochondrial loci...
  9. Thalenfeld B, Tzagoloff A. Assembly of the mitochondrial membrane system. Sequence of the oxi 2 gene of yeast mitochondrial DNA. J Biol Chem. 1980;255:6173-80 pubmed
    ..The orientation of the DS40 mtDNA segment relative to wild type mtDNA indicates that the oxi 2 gene is transcribed from the same DNA strand as the oxi 1 and several other mitochondrial genes. ..
  10. Garlich J, Strecker V, Wittig I, Stuart R. Mutational Analysis of the QRRQ Motif in the Yeast Hig1 Type 2 Protein Rcf1 Reveals a Regulatory Role for the Cytochrome c Oxidase Complex. J Biol Chem. 2017;292:5216-5226 pubmed publisher
    ..We speculate that the Rcf1/Hig1 proteins play a role in the incorporation and/or remodeling of lipids, in particular cardiolipin, into complex IV and. possibly, other mitochondrial proteins such as ADP/ATP carrier proteins. ..
  11. Shen H, Walters D, Mueller D. Introduction of the chloroplast redox regulatory region in the yeast ATP synthase impairs cytochrome c oxidase. J Biol Chem. 2008;283:32937-43 pubmed publisher
    ..These results indicate that the chimeric ATP synthase containing cysteine, but not serine, decreases the expression or assembly of cytochrome c oxidase with little effect on the activity of the ATP synthase. ..
  12. Horn D, Fontanesi F, Barrientos A. Exploring protein-protein interactions involving newly synthesized mitochondrial DNA-encoded proteins. Methods Mol Biol. 2008;457:125-39 pubmed
  13. Mayorga J, Camacho Villasana Y, Shingú Vázquez M, García Villegas R, Zamudio Ochoa A, García Guerrero A, et al. A Novel Function of Pet54 in Regulation of Cox1 Synthesis in Saccharomyces cerevisiae Mitochondria. J Biol Chem. 2016;291:9343-55 pubmed publisher
    Cytochrome c oxidase assembly requires the synthesis of the mitochondria-encoded core subunits, Cox1, Cox2, and Cox3. In yeast, Pet54 protein is required to activate translation of the COX3 mRNA and to process the aI5β intron on the ..
  14. Su C, McStay G, Tzagoloff A. Assembly of the rotor component of yeast mitochondrial ATP synthase is enhanced when Atp9p is supplied by Atp9p-Cox6p complexes. J Biol Chem. 2014;289:31605-16 pubmed publisher
  15. Keil M, Bareth B, Woellhaf M, Peleh V, Prestele M, Rehling P, et al. Oxa1-ribosome complexes coordinate the assembly of cytochrome C oxidase in mitochondria. J Biol Chem. 2012;287:34484-93 pubmed publisher
    ..This points to a role for Oxa1 in the spatial coordination of the ribosome with assembly factors that are critical for enzyme biogenesis. ..
  16. Fiumera H, Broadley S, Fox T. Translocation of mitochondrially synthesized Cox2 domains from the matrix to the intermembrane space. Mol Cell Biol. 2007;27:4664-73 pubmed
    ..Our data suggest that the C-tail domain is recognized posttranslationally by a specialized translocation apparatus after the N-tail has been translocated by Oxa1. ..
  17. Taanman J, Capaldi R. Purification of yeast cytochrome c oxidase with a subunit composition resembling the mammalian enzyme. J Biol Chem. 1992;267:22481-5 pubmed
    ..The enzyme isolated in lauryl maltoside contains these same nine different polypeptides and three others, including homologues of subunits VIa and VIb of the mammalian enzyme. ..
  18. Linder P, Slonimski P. An essential yeast protein, encoded by duplicated genes TIF1 and TIF2 and homologous to the mammalian translation initiation factor eIF-4A, can suppress a mitochondrial missense mutation. Proc Natl Acad Sci U S A. 1989;86:2286-90 pubmed
    ..Elevated gene dosage of TIF1 or TIF2 results in the suppression of a missense mutation in the mitochondrial oxi2 gene, which codes for subunit III of cytochrome-c oxidase, although the sequence of the Tif protein indicates its ..
  19. Shingu Vazquez M, Camacho Villasana Y, Sandoval Romero L, Butler C, Fox T, Perez Martinez X. The carboxyl-terminal end of Cox1 is required for feedback assembly regulation of Cox1 synthesis in Saccharomyces cerevisiae mitochondria. J Biol Chem. 2010;285:34382-9 pubmed publisher
    ..Our data suggest a novel activity of Pet54 required for normal synthesis of Cox1 that is independent of the Cox1 C-terminal end. ..
  20. Green Willms N, Fox T, Costanzo M. Functional interactions between yeast mitochondrial ribosomes and mRNA 5' untranslated leaders. Mol Cell Biol. 1998;18:1826-34 pubmed nuclear genes that suppress translation defects due to certain alterations in the 5'-UTLs of both the COX2 and COX3 mRNAs, indicating a general function in translational activation...
  21. Hell K, Herrmann J, Pratje E, Neupert W, Stuart R. Oxa1p, an essential component of the N-tail protein export machinery in mitochondria. Proc Natl Acad Sci U S A. 1998;95:2250-5 pubmed
    ..Thus, Oxa1p represents a component of a general export machinery of the mitochondrial inner membrane. ..
  22. Geier B, Schagger H, Ortwein C, Link T, Hagen W, Brandt U, et al. Kinetic properties and ligand binding of the eleven-subunit cytochrome-c oxidase from Saccharomyces cerevisiae isolated with a novel large-scale purification method. Eur J Biochem. 1995;227:296-302 pubmed
    ..This high content of fast enzyme was also reflected in the characteristics of optical and EPR spectra for cytochrome-c oxidase purified with our method. ..
  23. Su C, McStay G, Tzagoloff A. The Cox3p assembly module of yeast cytochrome oxidase. Mol Biol Cell. 2014;25:965-76 pubmed publisher
    ..These studies support our proposal that Cox3p and Cox1p are separate assembly modules with unique compositions of ancillary factors and subunits derived from the nuclear genome. ..
  24. Costanzo M, Fox T. Product of Saccharomyces cerevisiae nuclear gene PET494 activates translation of a specific mitochondrial mRNA. Mol Cell Biol. 1986;6:3694-703 pubmed
    ..These mutations were rearrangements of the mitochondrial gene oxi2 that encodes coxIII...
  25. Folley L, Fox T. Site-directed mutagenesis of a Saccharomyces cerevisiae mitochondrial translation initiation codon. Genetics. 1991;129:659-68 pubmed
    ..applicable strategy for gene replacement in yeast mitochondria to mutate the translation initiation codon of the COX3 gene from AUG to AUA...
  26. Kaspar B, Bifano A, Caprara M. A shared RNA-binding site in the Pet54 protein is required for translational activation and group I intron splicing in yeast mitochondria. Nucleic Acids Res. 2008;36:2958-68 pubmed publisher
    ..example of a dual functioning ('moonlighting') protein: it is required for translational activation of the COX3 mRNA and splicing of the aI5beta group I intron in the COX1 pre-mRNA in Saccharomyces cerevisiae mitochondria (mt)...