hCOX18 and hCOX19: Two human genes involved in cytochrome c oxidase assembly

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• 作者：Sabrina Sacconi ; Eva Trevisson ; Francesca Pistollato ; Maria Cristina Baldoin ; Roger Rezzonico ; Isabelle Bourget ; Claude Desnuelle ; Romano Tenconi ; Giuseppe Basso ; Salvatore DiMauro and Leonardo Salvia
• 关键词：Cytochrome c oxidase ; Respiratory chain ; Metal chaperones ; Mitochondria ; COX-assembly genes ; COX deficiency ; Oxa1
• 刊名：Biochemical and Biophysical Research Communications
• 出版年：2005
• 出版时间：2005
• 年：2005
• 卷：337
• 期：3
• 页码：832-839
• 全文大小：241 K

文摘

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.