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Gene Review

COX10  -  Cox10p

Saccharomyces cerevisiae S288c

Synonyms: Heme O synthase, Protoheme IX farnesyltransferase, mitochondrial, YPL172C
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Disease relevance of COX10

  • COX10 codes for a protein homologous to the ORF1 product of Paracoccus denitrificans and is required for the synthesis of yeast cytochrome oxidase [1].
  • B. subtilis CtaB, which is homologous to Saccharomyces cerevisiae COX10 and E. coli CyoE, also has a role in haem A synthesis and seems to be required for both cytochrome a and cytochrome o synthesis [2].

High impact information on COX10

  • In this and in other respects cox11 and cox10 mutants have very similar phenotypes [3].
  • Mutation analysis followed by a complementation study in yeast permitted us to ascribe the COX deficiency to a homozygous missense mutation in the COX10 gene [4].
  • Based on the nucleotide sequence of COX10, the primary translation product has an Mr of 52,000 [1].
  • The wild type COX10 gene has been cloned by transformation of a mutant from complementation group G19 with a yeast genomic library [1].
  • Genes homologous to COX10 are present in at least four different bacterial cytochrome oxidase operons [5].

Anatomical context of COX10


Other interactions of COX10


Analytical, diagnostic and therapeutic context of COX10

  • The two enzymes responsible for the conversion of heme B to heme A, heme O synthase (HOS) and heme A synthase (HAS), were separately genomically epitope-tagged in Saccharomyces cerevisiae, and their expression under various copper conditions was quantified by Western blot analysis [6].


  1. COX10 codes for a protein homologous to the ORF1 product of Paracoccus denitrificans and is required for the synthesis of yeast cytochrome oxidase. Nobrega, M.P., Nobrega, F.G., Tzagoloff, A. J. Biol. Chem. (1990) [Pubmed]
  2. Bacillus subtilis CtaA and CtaB function in haem A biosynthesis. Svensson, B., Lübben, M., Hederstedt, L. Mol. Microbiol. (1993) [Pubmed]
  3. Cytochrome oxidase assembly in yeast requires the product of COX11, a homolog of the P. denitrificans protein encoded by ORF3. Tzagoloff, A., Capitanio, N., Nobrega, M.P., Gatti, D. EMBO J. (1990) [Pubmed]
  4. A mutation in the human heme A:farnesyltransferase gene (COX10 ) causes cytochrome c oxidase deficiency. Valnot, I., von Kleist-Retzow, J.C., Barrientos, A., Gorbatyuk, M., Taanman, J.W., Mehaye, B., Rustin, P., Tzagoloff, A., Munnich, A., Rötig, A. Hum. Mol. Genet. (2000) [Pubmed]
  5. On the functions of the yeast COX10 and COX11 gene products. Tzagoloff, A., Nobrega, M., Gorman, N., Sinclair, P. Biochem. Mol. Biol. Int. (1993) [Pubmed]
  6. The biosynthesis of heme O and heme A is not regulated by copper. Morrison, M.S., Cricco, J.A., Hegg, E.L. Biochemistry (2005) [Pubmed]
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