Isolation of a cDNA encoding the human homolog of COX17, a yeast gene essential for mitochondrial copper recruitment

Hum Genet. 1997 Mar;99(3):329-33. doi: 10.1007/s004390050367.


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. To determine if Cox17p is present in mammalian cells, a yeast strain carrying a null mutation in COX17 was transformed with a human cDNA expression library. All the respiratory competent clones obtained from the transformations carried a common cDNA sequence with a reading frame predicting a product homologous to yeast Cox17p. The cloning of a mammalian COX17 homolog suggests that the encoded product is likely to function in copper recruitment in eucaryotic cells in general. Its presence in humans provides a possible target for genetically inherited deficiencies in cytochrome oxidase.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Amino Acid Sequence
  • Base Sequence
  • Carrier Proteins
  • Cation Transport Proteins*
  • Copper / metabolism*
  • Copper Transport Proteins
  • DNA, Complementary / isolation & purification
  • Electron Transport Complex IV / metabolism
  • Escherichia coli
  • Genetic Complementation Test
  • HeLa Cells
  • Humans
  • Mitochondria / metabolism*
  • Molecular Chaperones
  • Molecular Sequence Data
  • Mutation
  • Plasmids
  • Proteins / genetics*
  • Restriction Mapping
  • Saccharomyces cerevisiae / genetics*
  • Saccharomyces cerevisiae / growth & development
  • Saccharomyces cerevisiae / metabolism
  • Saccharomyces cerevisiae Proteins*
  • Sequence Homology, Amino Acid
  • Transformation, Genetic


  • COX17 protein, S cerevisiae
  • COX17 protein, human
  • Carrier Proteins
  • Cation Transport Proteins
  • Copper Transport Proteins
  • DNA, Complementary
  • Molecular Chaperones
  • Proteins
  • Saccharomyces cerevisiae Proteins
  • Copper
  • Electron Transport Complex IV

Associated data

  • GENBANK/L77701