hCOX18 and hCOX19: two human genes involved in cytochrome c oxidase assembly.
We identified the human homologues of yCOX18 and yCOX19, two Saccharomyces cerevisiae genes involved in the biogenesis of mitochondrial respiratory chain complexes. In yeast, these two genes are required for the expression of cytochrome c oxidase: Cox18p catalyses the insertion of Cox2p COOH-tail into the mitochondrial inner membrane, and Cox19p is probably involved in metal transport to the intermembrane space. Both hCox18p and hCox19p present significant amino acid identity with the corresponding yeast polypeptides and reveal highly conserved functional domains. In addition, their subcellular localization is analogous to that of the yeast proteins. These data strongly suggest that the human gene products share similar functions with their yeast homologues. These two COX-assembly genes represent new candidates for mutational analysis in patients with isolated COX deficiency of unknown etiology.
Sacconi
Sabrina
S
INSERM U638, Faculté de Médicine, Université de Nice, France.
Trevisson
Eva
E
Pistollato
Francesca
F
Baldoin
Maria Cristina
MC
Rezzonico
Roger
R
Bourget
Isabelle
I
Desnuelle
Claude
C
Tenconi
Romano
R
Basso
Giuseppe
G
DiMauro
Salvatore
S
Salviati
Leonardo
L
D000595:Amino Acid Sequence; D002460:Cell Line; D003576:Electron Transport Complex IV / Q000737:chemistry / Q000378:metabolism*; D006801:Humans; D007668:Kidney / Q000378:metabolism*; D008565:Membrane Proteins / Q000737:chemistry* / Q000235:genetics / Q000378:metabolism*; D008928:Mitochondria / Q000737:chemistry / Q000378:metabolism*; D024101:Mitochondrial Proteins / Q000737:chemistry* / Q000235:genetics / Q000378:metabolism*; D008958:Models, Molecular; D008969:Molecular Sequence Data; D009928:Organ Specificity; D017386:Sequence Homology, Amino Acid; D014018:Tissue Distribution
C506210:COX18 protein, human; C506211:COX19 protein, human; D008565:Membrane Proteins; D024101:Mitochondrial Proteins; D003576:Electron Transport Complex IV