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Gene Review

COX17  -  COX17 cytochrome c oxidase copper chaperone

Homo sapiens

Synonyms: Cytochrome c oxidase copper chaperone
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Disease relevance of COX17


High impact information on COX17

  • We browsed bacterial genomes to search proteins functionally equivalent to Cox17, and we identified a class of proteins of unknown function displaying a conserved gene neighborhood to bacterial Sco1 genes, all sharing a potential metal binding motif H(M)X10MX21HXM [2].
  • PCR analysis of RNA from synaptosomal polysomes establishes that COX17 and CoQ7 mRNAs are being actively translated [3].
  • Using a panel of human:mouse-monochromosomal hybrids, the expression of COX17 was specifically restricted to chromosome 3, indicating that the previously reported sequence on chromosome 13 represents a pseudogene [1].
  • Porcine Cox17p/dopuin possesses high sequence homology to the product of human COX17 gene (h-Cox17p) [4].


  1. Characterization of human SCO1 and COX17 genes in mitochondrial cytochrome-c-oxidase deficiency. Horvath, R., Lochmüller, H., Stucka, R., Yao, J., Shoubridge, E.A., Kim, S.H., Gerbitz, K.D., Jaksch, M. Biochem. Biophys. Res. Commun. (2000) [Pubmed]
  2. A copper(I) protein possibly involved in the assembly of CuA center of bacterial cytochrome c oxidase. Banci, L., Bertini, I., Ciofi-Baffoni, S., Katsari, E., Katsaros, N., Kubicek, K., Mangani, S. Proc. Natl. Acad. Sci. U.S.A. (2005) [Pubmed]
  3. Local synthesis of nuclear-encoded mitochondrial proteins in the presynaptic nerve terminal. Gioio, A.E., Eyman, M., Zhang, H., Lavina, Z.S., Giuditta, A., Kaplan, B.B. J. Neurosci. Res. (2001) [Pubmed]
  4. Isolation and characterization of Cox17p from porcine heart by determining its survival-promoting activity in NIH3T3 cells. Takenouchi, T., Fujimoto, M., Shimamoto, A., Munekata, E. Biochim. Biophys. Acta (1999) [Pubmed]
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