Gene Symbol: COX1
Description: cytochrome c oxidase subunit 1
Alias: OXI3, cytochrome c oxidase subunit 1
Species: Saccharomyces cerevisiae S288c

Top Publications

  1. Khalimonchuk O, Bestwick M, Meunier B, Watts T, Winge D. Formation of the redox cofactor centers during Cox1 maturation in yeast cytochrome oxidase. Mol Cell Biol. 2010;30:1004-17 pubmed publisher
    The biogenesis of cytochrome c oxidase initiates with synthesis and maturation of the mitochondrion-encoded Cox1 subunit prior to the addition of other subunits...
  2. 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. ..
  3. Perez Martinez X, Broadley S, Fox T. Mss51p promotes mitochondrial Cox1p synthesis and interacts with newly synthesized Cox1p. EMBO J. 2003;22:5951-61 pubmed
    ..found that MSS51 is required for expression of the mitochondrial reporter gene ARG8(m) when it is inserted at the COX1 locus, but not when it is at COX2 or COX3...
  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. 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
    Functional interactions of the translational activator Mss51 with both the mitochondrially encoded COX1 mRNA 5'-untranslated region and with newly synthesized unassembled Cox1 protein suggest that it has a key role in coupling Cox1 ..
  6. Pierrel F, Bestwick M, Cobine P, Khalimonchuk O, Cricco J, Winge D. Coa1 links the Mss51 post-translational function to Cox1 cofactor insertion in cytochrome c oxidase assembly. EMBO J. 2007;26:4335-46 pubmed
    ..The respiratory defect in coa1Delta cells is suppressed by high-copy MSS51, MDJ1 and COX10. Mss51 functions in Cox1 translation and elongation, whereas Cox10 participates in the biosynthesis of heme a, a key cofactor of CcO...
  7. 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
    ..association with different protein modules; Shy1 interacts with Mss51 and Cox14, translational regulators of Cox1. Additionally, Shy1 associates with the subcomplexes of complex IV that are potential assembly intermediates...
  8. Barrientos A, Zambrano A, Tzagoloff A. Mss51p and Cox14p jointly regulate mitochondrial Cox1p expression in Saccharomyces cerevisiae. EMBO J. 2004;23:3472-82 pubmed
    ..studies of the yeast model of this disease showed that mutant forms of Mss51p, a translational activator of COX1 mRNA, partially rescue the COX deficiency of shy1 mutants by restoring normal synthesis of the mitochondrially ..
  9. Zambrano A, Fontanesi F, Solans A, de Oliveira R, Fox T, Tzagoloff A, et al. Aberrant translation of cytochrome c oxidase subunit 1 mRNA species in the absence of Mss51p in the yeast Saccharomyces cerevisiae. Mol Biol Cell. 2007;18:523-35 pubmed
    ..Mss51p is a translational factor for cytochrome c oxidase subunit 1 (COX1) mRNA and a key player in down-regulating Cox1p expression when subunits with which it normally ..

More Information


  1. Gebert M, Schrempp S, Mehnert C, Heißwolf A, Oeljeklaus S, Ieva R, et al. Mgr2 promotes coupling of the mitochondrial presequence translocase to partner complexes. J Cell Biol. 2012;197:595-604 pubmed publisher
    ..We conclude that Mgr2 is a coupling factor of the presequence translocase crucial for cell growth at elevated temperature and for efficient protein import. ..
  2. Bonitz S, Coruzzi G, Thalenfeld B, Tzagoloff A, Macino G. Assembly of the mitochondrial membrane system. Structure and nucleotide sequence of the gene coding for subunit 1 of yeast cytochrme oxidase. J Biol Chem. 1980;255:11927-41 pubmed
    the oxi3 locus of yeast mitochondrial DNA has been sequenced in Saccharomyces cerevisiae D273-10B...
  3. Lemaire C, Robineau S, Netter P. Molecular and biochemical analysis of Saccharomyces cerevisiae cox1 mutants. Curr Genet. 1998;34:138-45 pubmed
    ..analysis of a set of 13 respiratory deficient mutants of Saccharomyces cerevisiae which are specifically altered in COX1, the gene encoding the subunit Cox1p of cytochrome c oxidase...
  4. 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. ..
  5. Bestwick M, Khalimonchuk O, Pierrel F, Winge D. The role of Coa2 in hemylation of yeast Cox1 revealed by its genetic interaction with Cox10. Mol Cell Biol. 2010;30:172-85 pubmed publisher
    Saccharomyces cerevisiae cells lacking the cytochrome c oxidase (CcO) assembly factor Coa2 are impaired in Cox1 maturation and exhibit a rapid degradation of newly synthesized Cox1...
  6. Watts T, Khalimonchuk O, Wolf R, Turk E, Mohr G, Winge D. Mne1 is a novel component of the mitochondrial splicing apparatus responsible for processing of a COX1 group I intron in yeast. J Biol Chem. 2011;286:10137-46 pubmed publisher
    Saccharomyces cerevisiae cells lacking Mne1 are deficient in intron splicing in the gene encoding the Cox1 subunit of cytochrome oxidase but contain wild-type levels of the bc(1) complex...
  7. 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
    ..Based on our findings, we propose a model where the Hig1 proteins regulate the COX enzyme activity through Cox3 and associated Cox12 protein, in a manner that may be influenced by the neighboring AAC proteins. ..
  8. Leadsham J, Sanders G, Giannaki S, Bastow E, Hutton R, Naeimi W, et al. Loss of cytochrome c oxidase promotes RAS-dependent ROS production from the ER resident NADPH oxidase, Yno1p, in yeast. Cell Metab. 2013;18:279-86 pubmed publisher
    ..Our data suggest that dysfunctional mitochondria can serve as a signaling platform to promote the loss of redox homeostasis, ROS accumulation, and accelerate aging in yeast...
  9. Soto I, Fontanesi F, Valledor M, Horn D, Singh R, Barrientos A. Synthesis of cytochrome c oxidase subunit 1 is translationally downregulated in the absence of functional F1F0-ATP synthase. Biochim Biophys Acta. 2009;1793:1776-86 pubmed publisher
    ..Our findings imply that fully assembled ATPase and its proton pumping function are both required for COX biogenesis in yeast and mammalian cells through a mechanism independent of Cox1p synthesis...
  10. Bareth B, Nikolov M, Lorenzi I, Hildenbeutel M, Mick D, Helbig C, et al. Oms1 associates with cytochrome c oxidase assembly intermediates to stabilize newly synthesized Cox1. Mol Biol Cell. 2016;27:1570-80 pubmed publisher
    ..The assembly process of the enzyme is initiated by membrane insertion of the mitochondria-encoded Cox1 subunit...
  11. Tavares Carreón F, Camacho Villasana Y, Zamudio Ochoa A, Shingu Vazquez M, Torres Larios A, Perez Martinez X. The pentatricopeptide repeats present in Pet309 are necessary for translation but not for stability of the mitochondrial COX1 mRNA in yeast. J Biol Chem. 2008;283:1472-9 pubmed
    Pet309 is a protein essential for respiratory growth. It is involved in translation of the yeast mitochondrial COX1 gene, which encodes subunit I of the cytochrome c oxidase. Pet309 is also involved in stabilization of the COX1 mRNA...
  12. Schilder J, Lohr F, Schwalbe H, Ubbink M. The cytochrome c peroxidase and cytochrome c encounter complex: the other side of the story. FEBS Lett. 2014;588:1873-8 pubmed publisher
    ..This work demonstrates the need of obtaining as many independent data sets as possible to achieve the most accurate description of an encounter complex. ..
  13. Seraphin B, Simon M, Jacq C, Faye G. Sequence of the yeast mitochondrial OX13/OL12 promoter region. Nucleic Acids Res. 1989;17:4886 pubmed
  14. 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
  15. Zamudio Ochoa A, Camacho Villasana Y, García Guerrero A, Pérez Martínez X. The Pet309 pentatricopeptide repeat motifs mediate efficient binding to the mitochondrial COX1 transcript in yeast. RNA Biol. 2014;11:953-67 pubmed publisher
    Mitochondrial synthesis of Cox1, the largest subunit of the cytochrome c oxidase complex, is controlled by Mss51 and Pet309, two mRNA-specific translational activators that act via the COX1 mRNA 5'-UTR through an unknown mechanism...
  16. Bareth B, Dennerlein S, Mick D, Nikolov M, Urlaub H, Rehling P. The heme a synthase Cox15 associates with cytochrome c oxidase assembly intermediates during Cox1 maturation. Mol Cell Biol. 2013;33:4128-37 pubmed publisher
    b>Cox1, the core subunit of the cytochrome c oxidase, receives two heme a cofactors during assembly of the 13-subunit enzyme complex. However, at which step of the assembly process and how heme is inserted into Cox1 have remained an enigma...
  17. Fontanesi F, Clemente P, Barrientos A. Cox25 teams up with Mss51, Ssc1, and Cox14 to regulate mitochondrial cytochrome c oxidase subunit 1 expression and assembly in Saccharomyces cerevisiae. J Biol Chem. 2011;286:555-66 pubmed publisher
    ..Mss51, a specific COX1 mRNA translational activator and Cox1 chaperone, drives the regulatory mechanism...
  18. 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
  19. 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...
  20. García Villegas R, Camacho Villasana Y, Shingú Vázquez M, Cabrera Orefice A, Uribe Carvajal S, Fox T, et al. The Cox1 C-terminal domain is a central regulator of cytochrome c oxidase biogenesis in yeast mitochondria. J Biol Chem. 2017;292:10912-10925 pubmed publisher
    ..The CcO core is formed by mitochondrial DNA-encoded Cox1, Cox2, and Cox3 subunits...
  21. 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. ..
  22. Moreno J, Buie K, Price R, Piva M. Ccm1p/Ygr150cp, a pentatricopeptide repeat protein, is essential to remove the fourth intron of both COB and COX1 pre-mRNAs in Saccharomyces cerevisiae. Curr Genet. 2009;55:475-84 pubmed publisher
    ..RNA hybridization studies showed that Deltaygr150c meiotic segregants produced COB and COX1 transcripts but failed to process them into the mature forms...
  23. Pierrel F, Khalimonchuk O, Cobine P, Bestwick M, Winge D. Coa2 is an assembly factor for yeast cytochrome c oxidase biogenesis that facilitates the maturation of Cox1. Mol Cell Biol. 2008;28:4927-39 pubmed publisher
    ..Coa1 and Shy1 function at an early step in maturation of the Cox1 subunit of CcO...
  24. Mick D, Vukotic M, Piechura H, Meyer H, Warscheid B, Deckers M, et al. Coa3 and Cox14 are essential for negative feedback regulation of COX1 translation in mitochondria. J Cell Biol. 2010;191:141-54 pubmed publisher
    Regulation of eukaryotic cytochrome oxidase assembly occurs at the level of Cox1 translation, its central mitochondria-encoded subunit...
  25. 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. ..
  26. 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. ..
  27. 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. ..
  28. Levchenko M, Wuttke J, Römpler K, Schmidt B, Neifer K, Juris L, et al. Cox26 is a novel stoichiometric subunit of the yeast cytochrome c oxidase. Biochim Biophys Acta. 2016;1863:1624-32 pubmed publisher
    ..The enzyme complex assembles in a stepwise process from the three mitochondria-encoded core subunits Cox1, Cox2 and Cox3, which associate with nuclear-encoded subunits and cofactors...
  29. Cui T, Conte A, Fox J, Zara V, Winge D. Modulation of the respiratory supercomplexes in yeast: enhanced formation of cytochrome oxidase increases the stability and abundance of respiratory supercomplexes. J Biol Chem. 2014;289:6133-41 pubmed publisher
    ..This study presents the first evidence showing that supercomplexes can be stabilized by the stimulated formation of CcO. ..
  30. 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. ..
  31. Pel H, Maat C, Rep M, Grivell L. The yeast nuclear gene MRF1 encodes a mitochondrial peptide chain release factor and cures several mitochondrial RNA splicing defects. Nucleic Acids Res. 1992;20:6339-46 pubmed
    ..of a respiratory deficient mutant disturbed in the expression of the mitochondrial genes encoding cytochrome c oxidase subunit 1 and 2, COX1 and COX2...
  32. Fiori A, Perez Martinez X, Fox T. Overexpression of the COX2 translational activator, Pet111p, prevents translation of COX1 mRNA and cytochrome c oxidase assembly in mitochondria of Saccharomyces cerevisiae. Mol Microbiol. 2005;56:1689-704 pubmed
    ..The respiratory phenotype appears to be caused primarily by inhibition of the COX1 mitochondrial mRNA translation, a finding confirmed by lack of cox1Delta::ARG8(m) reporter mRNA translation...
  33. Barros M, Myers A, Van Driesche S, Tzagoloff A. COX24 codes for a mitochondrial protein required for processing of the COX1 transcript. J Biol Chem. 2006;281:3743-51 pubmed
    In most strains of Saccharomyces cerevisiae the mitochondrial gene COX1, for subunit 1 of cytochrome oxidase, contains multiple exons and introns...
  34. Römpler K, Müller T, Juris L, Wissel M, Vukotic M, Hofmann K, et al. Overlapping Role of Respiratory Supercomplex Factor Rcf2 and Its N-terminal Homolog Rcf3 in Saccharomyces cerevisiae. J Biol Chem. 2016;291:23769-23778 pubmed
    ..Furthermore, our data suggest an association of all three Rcf proteins with the bc1 complex in the absence of a functional cytochrome c oxidase and identify a supercomplex independent interaction network of the Rcf proteins. ..
  35. Khalimonchuk O, Jeong M, Watts T, Ferris E, Winge D. Selective Oma1 protease-mediated proteolysis of Cox1 subunit of cytochrome oxidase in assembly mutants. J Biol Chem. 2012;287:7289-300 pubmed publisher
    ..The conserved Oma1 metalloproteinase mediates facile Cox1 degradation in cells lacking the Coa2 assembly factor, but not in a series of other mutants stalled in CcO ..
  36. Reinhold R, Bareth B, Balleininger M, Wissel M, Rehling P, Mick D. Mimicking a SURF1 allele reveals uncoupling of cytochrome c oxidase assembly from translational regulation in yeast. Hum Mol Genet. 2011;20:2379-93 pubmed publisher
    ..enigmatic, loss of function leads to cytochrome c oxidase deficiency and reduced expression of the central subunit Cox1 in yeast...
  37. 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
    Synthesis of the largest cytochrome c oxidase (CcO) subunit, Cox1, on yeast mitochondrial ribosomes is coupled to assembly of CcO...
  38. 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 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)...
  39. Schmidt U, Lehmann K, Stahl U. A novel mitochondrial DEAD box protein (Mrh4) required for maintenance of mtDNA in Saccharomyces cerevisiae. FEMS Yeast Res. 2002;2:267-76 pubmed
    ..The gene encodes the fourth putative RNA helicase of Saccharomyces cerevisiae implicated in a mitochondrial function and was therefore termed MRH4 (for mitochondrial RNA helicase). ..
  40. Asher E, Groudinsky O, Dujardin G, Altamura N, Kermorgant M, Slonimski P. Novel class of nuclear genes involved in both mRNA splicing and protein synthesis in Saccharomyces cerevisiae mitochondria. Mol Gen Genet. 1989;215:517-28 pubmed
    ..The possible mechanisms by which the NAM1 gene product may function are discussed. ..
  41. McStay G, Su C, Tzagoloff A. Stabilization of Cox1p intermediates by the Cox14p-Coa3p complex. FEBS Lett. 2013;587:943-9 pubmed publisher
    Cox14p and Coa3p have been shown to regulate translation of the mitochondrial COX1 mRNA and to be required for assembly of cytochrome oxidase...
  42. 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
    ..Synthesis of subunit 1 (Cox1) is contingent on the availability of its assembly partners, thereby acting as a negative feedback loop that ..
  43. Page T, Hoffman B. Control of cyclic photoinitiated electron transfer between cytochrome c peroxidase (W191F) and cytochrome c by formation of dynamic binary and ternary complexes. Biochemistry. 2015;54:1188-97 pubmed publisher
  44. 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
  45. Horn D, Al Ali H, Barrientos A. Cmc1p is a conserved mitochondrial twin CX9C protein involved in cytochrome c oxidase biogenesis. Mol Cell Biol. 2008;28:4354-64 pubmed publisher
    ..Our results suggest that Cmc1p could play a direct or indirect role in copper trafficking and distribution to COX and Sod1p. ..