title: The effects of erythropoetic activity and iron burden on hepcidin expression in patients with thalassemia major.
abstract: Hepcidin production is homeostatically regulated by iron stores, anemia and hypoxia. We evaluated the effect of iron overload and of ineffective erythropoeisis on hepcidin expression in patients with thalassemia major. Liver hepcidin mRNA levels correlated with hemoglobin concentration and inversely correlated with serum transferrin receptor, erythropoietin and non-transferrin-bound iron. They did not correlate with indices of iron load. Urinary hepcidin levels were disproportionably suppressed in regards to iron burden. We conclude that hepcidin expression is regulated mainly by increased erythropoietic activity rather than by iron load and that hepcidin plays a central regulatory role in iron circulation and iron toxicity in patients with thalassemia.
authors.affiliation: First Department of Pediatrics, National and Kapodistrian University of Athens Medical School, Athens 11527, Greece. ankatt@med.uoa.gr
authors.forename: Antonis
authors.initials: A
authors.lastname: Kattamis
authors.forename: Ioannis
authors.initials: I
authors.lastname: Papassotiriou
authors.forename: Danai
authors.initials: D
authors.lastname: Palaiologou
authors.forename: Filia
authors.initials: F
authors.lastname: Apostolakou
authors.forename: Angeliki
authors.initials: A
authors.lastname: Galani
authors.forename: Vassilis
authors.initials: V
authors.lastname: Ladis
authors.forename: Nikos
authors.initials: N
authors.lastname: Sakellaropoulos
authors.forename: George
authors.initials: G
authors.lastname: Papanikolaou
mesh_terms: D023181:Antimicrobial Cationic Peptides / Q000235:genetics*; D001803:Blood Transfusion; D017931:DNA Primers; D017707:Erythrocyte Transfusion; D004920:Erythropoiesis; D005786:Gene Expression Regulation; D064451:Hepcidins; D006801:Humans; D007223:Infant; D007501:Iron / Q000378:metabolism*; D008099:Liver / Q000503:physiopathology; D008297:Male; D016133:Polymerase Chain Reaction; D012313:RNA / Q000235:genetics / Q000302:isolation & purification; D017086:beta-Thalassemia / Q000097:blood / Q000235:genetics* / Q000628:therapy
chemical_list: D023181:Antimicrobial Cationic Peptides; D017931:DNA Primers; C578702:HAMP protein, human; D064451:Hepcidins; D012313:RNA; D007501:Iron