Hepcidin in beta-thalassemia.
Iron overload is the principal cause of morbidity and mortality in beta-thalassemia with or without transfusion dependence. Iron homeostasis is regulated by the hepatic peptide hormone hepcidin. Hepcidin controls dietary iron absorption, plasma iron concentrations, and tissue iron distribution. A deficiency in this hormone is the main or contributing factor of iron overload in iron-loading anemias such as beta-thalassemia. Hepcidin deficiency results from a strong suppressive effect of the high erythropoietic activity on hepcidin expression. Although in thalassemia major patients iron absorption contributes less to the total iron load than transfusions, in non-transfused thalassemia, low hepcidin, and the consequent hyperabsorption of dietary iron is the major cause of systemic iron overload. Hepcidin diagnostics and future therapeutic agonists may help in management of patients with beta-thalassemia.
Nemeth
Elizabeta
E
Department of Medicine, University of California at Los Angeles, Los Angeles, CA 90095, USA. enemeth@mednet.ucla.edu
D023181:Antimicrobial Cationic Peptides / Q000378:metabolism*; D064451:Hepcidins; D006706:Homeostasis; D006801:Humans; D019190:Iron Overload / Q000378:metabolism*; D019266:Iron, Dietary / Q000378:metabolism; D017086:beta-Thalassemia / Q000503:physiopathology* / Q000628:therapy
D023181:Antimicrobial Cationic Peptides; C578702:HAMP protein, human; D064451:Hepcidins; D019266:Iron, Dietary