Ceruloplasmin metabolism and function.
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.
Hellman
Nathan E
NE
Edward Mallinckrodt Department of Pediatrics, Washington University School of Medicine, St. Louis, Missouri 63110, USA. hellman_n@kids.wustl.edu
Gitlin
Jonathan D
JD
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
D003300:Copper; D007501:Iron; D002570:Ceruloplasmin