title: The molecular basis of iron overload disorders and iron-linked anemias.
abstract: Iron homeostasis in vertebrates requires coordination between cells that export iron into plasma and those that utilize or store plasma iron. The coordination of iron acquisition and utilization is mediated by the interaction of the peptide hormone hepcidin and the iron exporter ferroportin. Hepcidin levels are increased during iron sufficiency and inflammation and are decreased in hypoxia or erythropoiesis. Hepcidin is a negative regulator of iron export. Hepcidin binds to cell surface ferroportin inducing ferroportin degradation and decreasing cellular iron export. Genetic disorders of iron overload of iron-linked anemia can be explained by changes in the level of hepcidin or ferroportin and of the ability of ferroportin to be internalized by hepcidin.
authors.affiliation: Department of Pathology, School of Medicine, University of Utah, Salt Lake City, UT 84312, USA. jerry.kaplan@path.utah.edu
authors.forename: Jerry
authors.initials: J
authors.lastname: Kaplan
authors.forename: Diane M
authors.initials: DM
authors.lastname: Ward
authors.forename: Ivana
authors.initials: I
authors.lastname: De Domenico
mesh_terms: D018798:Anemia, Iron-Deficiency / Q000235:genetics / Q000378:metabolism*; D000818:Animals; D023181:Antimicrobial Cationic Peptides / Q000235:genetics / Q000378:metabolism*; D001692:Biological Transport / Q000235:genetics; D027682:Cation Transport Proteins / Q000235:genetics / Q000378:metabolism*; D004920:Erythropoiesis; D064451:Hepcidins; D006801:Humans; D007501:Iron / Q000378:metabolism*; D019190:Iron Overload / Q000235:genetics / Q000378:metabolism*; D000098742:Ferroportin
chemical_list: D023181:Antimicrobial Cationic Peptides; D027682:Cation Transport Proteins; C578702:HAMP protein, human; D064451:Hepcidins; D000098742:Ferroportin; D007501:Iron