title: Biogenesis and assembly of eukaryotic cytochrome c oxidase catalytic core.
abstract: Eukaryotic cytochrome c oxidase (COX) is the terminal enzyme of the mitochondrial respiratory chain. COX is a multimeric enzyme formed by subunits of dual genetic origin which assembly is intricate and highly regulated. The COX catalytic core is formed by three mitochondrial DNA encoded subunits, Cox1, Cox2 and Cox3, conserved in the bacterial enzyme. Their biogenesis requires the action of messenger-specific and subunit-specific factors which facilitate the synthesis, membrane insertion, maturation or assembly of the core subunits. The study of yeast strains and human cell lines from patients carrying mutations in structural subunits and COX assembly factors has been invaluable to identify these ancillary factors. Here we review the current state of knowledge of the biogenesis and assembly of the eukaryotic COX catalytic core and discuss the degree of conservation of the players and mechanisms operating from yeast to human. This article is part of a Special Issue entitled: Biogenesis/Assembly of Respiratory Enzyme Complexes.
authors.affiliation: Department of Biochemistry & Molecular Biology, University of Miami Miller School of Medicine, Miami, FL 33136, USA.
authors.forename: Ileana C
authors.initials: IC
authors.lastname: Soto
authors.forename: Flavia
authors.initials: F
authors.lastname: Fontanesi
authors.forename: Jingjing
authors.initials: J
authors.lastname: Liu
authors.forename: Antoni
authors.initials: A
authors.lastname: Barrientos
mesh_terms: D000818:Animals; D003067:Coenzymes / Q000096:biosynthesis / Q000378:metabolism; D003576:Electron Transport Complex IV / Q000096:biosynthesis* / Q000235:genetics / Q000378:metabolism; D004795:Enzyme Stability; D005786:Gene Expression Regulation; D050259:Genes, Mitochondrial; D006801:Humans; D024101:Mitochondrial Proteins / Q000096:biosynthesis / Q000235:genetics / Q000378:metabolism; D055503:Protein Multimerization; D021122:Protein Subunits / Q000096:biosynthesis / Q000235:genetics / Q000378:metabolism; D012441:Saccharomyces cerevisiae / Q000201:enzymology; D029701:Saccharomyces cerevisiae Proteins / Q000096:biosynthesis / Q000235:genetics / Q000378:metabolism
chemical_list: D003067:Coenzymes; D024101:Mitochondrial Proteins; D021122:Protein Subunits; D029701:Saccharomyces cerevisiae Proteins; D003576:Electron Transport Complex IV