title: Ceruloplasmin metabolism and function.
abstract: Ceruloplasmin is a serum ferroxidase that contains greater than 95% of the copper found in plasma. This protein is a member of the multicopper oxidase family, an evolutionarily conserved group of proteins that utilize copper to couple substrate oxidation with the four-electron reduction of oxygen to water. Despite the need for copper in ceruloplasmin function, this protein plays no essential role in the transport or metabolism of this metal. Aceruloplasminemia is a neurodegenerative disease resulting from inherited loss-of-function mutations in the ceruloplasmin gene. Characterization of this disorder revealed a critical physiological role for ceruloplasmin in determining the rate of iron efflux from cells with mobilizable iron stores and has provided new insights into human iron metabolism and nutrition.
authors.affiliation: Edward Mallinckrodt Department of Pediatrics, Washington University School of Medicine, St. Louis, Missouri 63110, USA. hellman_n@kids.wustl.edu
authors.forename: Nathan E
authors.initials: NE
authors.lastname: Hellman
authors.forename: Jonathan D
authors.initials: JD
authors.lastname: Gitlin
mesh_terms: D002570:Ceruloplasmin / Q000172:deficiency / Q000235:genetics / Q000378:metabolism* / Q000502:physiology*; D003300:Copper / Q000378:metabolism* / Q000502:physiology; D006801:Humans; D007501:Iron / Q000378:metabolism; D019636:Neurodegenerative Diseases / Q000175:diagnosis / Q000235:genetics* / Q000378:metabolism; D010084:Oxidation-Reduction
chemical_list: D003300:Copper; D007501:Iron; D002570:Ceruloplasmin