title: Copper chaperones, intracellular copper trafficking proteins. Function, structure, and mechanism of action.
abstract: This review summarizes findings on a new family of small cytoplasmic proteins called copper chaperones. The copper chaperones bind and deliver copper ions to intracellular compartments and insert the copper into the active sites of specific partners, copper-dependent enzymes. Three types of copper chaperones have been found in eukaryotes. Their three-dimensional structures have been determined, intracellular target proteins identified, and mechanisms of action have been revealed. The Atx1 copper chaperone binds Cu(I) and interacts directly with the copper-binding domains of a P-type ATPase copper transporter, its physiological partner. The copper chaperone CCS delivers Cu(I) to Cu,Zn-superoxide dismutase 1. Cox17 and Cox11 proteins serve as copper chaperones for cytochrome c oxidase, a copper-dependent enzyme.
authors.affiliation: Bach Institute of Biochemistry, Russian Academy of Sciences, Moscow 119071, Russia. markossian@inbi.ras.ru
authors.forename: K A
authors.initials: KA
authors.lastname: Markossian
authors.forename: B I
authors.initials: BI
authors.lastname: Kurganov
mesh_terms: D000818:Animals; D002352:Carrier Proteins / Q000378:metabolism; D027682:Cation Transport Proteins / Q000378:metabolism; D003300:Copper / Q000378:metabolism*; D000079922:Copper Transport Proteins; D045222:Electron Transport Chain Complex Proteins; D003576:Electron Transport Complex IV / Q000378:metabolism; D006801:Humans; D008565:Membrane Proteins / Q000378:metabolism; D024101:Mitochondrial Proteins; D008958:Models, Molecular; D018832:Molecular Chaperones / Q000378:metabolism*; D021381:Protein Transport / Q000502:physiology; D029701:Saccharomyces cerevisiae Proteins / Q000378:metabolism; D013482:Superoxide Dismutase / Q000378:metabolism
chemical_list: C093341:ATX1 protein, S cerevisiae; C114678:CCS protein, human; C491960:CCS1 protein, S cerevisiae; C086106:COX11 protein, S cerevisiae; C484188:COX11 protein, human; C491006:COX17 protein, human; D002352:Carrier Proteins; D027682:Cation Transport Proteins; D000079922:Copper Transport Proteins; D045222:Electron Transport Chain Complex Proteins; D008565:Membrane Proteins; D024101:Mitochondrial Proteins; D018832:Molecular Chaperones; D029701:Saccharomyces cerevisiae Proteins; D003300:Copper; D013482:Superoxide Dismutase; D003576:Electron Transport Complex IV