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Blood, 15 May 2005, Vol. 105, No. 10, pp. 4103-4105. Prepublished online as a Blood First Edition Paper on January 25, 2005; DOI 10.1182/blood-2004-12-4844. This Article Full Text Full Text (PDF) All Versions of this Article: 2004-12-4844v1 105/10/4103    most recent Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Papanikolaou, G. Articles by Nemeth, E. Search for Related Content PubMed PubMed Citation Articles by Papanikolaou, G. Articles by Nemeth, E. Related Collections Red Cells Brief Reports Clinical Trials and Observations Related Article in Blood Online Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  RED CELLS Brief report Hepcidin in iron overload disorders George Papanikolaou, Michalis Tzilianos, John I. Christakis, Dionisios Bogdanos, Konstantina Tsimirika, Julie MacFarlane, Y. Paul Goldberg, Nikos Sakellaropoulos, Tomas Ganz, and Elizabeta Nemeth From the First Department of Medicine, National and Kapodistrian University of Athens; Blood Bank Unit, Corfu General Hospital; Department of Hematology, Theagenion Cancer Center, Thessaloniki, Greece; Xenon Pharmaceuticals, Burnaby, British Columbia, Canada; and Department of Medicine, David Geffen School of Medicine, University of California, Los Angeles. Hepcidin is the principal regulator of iron absorption in humans. The peptide inhibits cellular iron efflux by binding to the iron export channel ferroportin and inducing its internalization and degradation. Either hepcidin deficiency or alterations in its target, ferroportin, would be expected to result in dysregulated iron absorption, tissue maldistribution of iron, and iron overload. Indeed, hepcidin deficiency has been reported in hereditary hemochromatosis and attributed to mutations in HFE, transferrin receptor 2, hemojuvelin, and the hepcidin gene itself. We measured urinary hepcidin in patients with other genetic causes of iron overload. Hepcidin was found to be suppressed in patients with thalassemia syndromes and congenital dyserythropoietic anemia type 1 and was undetectable in patients with juvenile hemochromatosis with HAMP mutations. Of interest, urine hepcidin levels were significantly elevated in 2 patients with hemochromatosis type 4. These findings extend the spectrum of iron disorders with hepcidin deficiency and underscore the critical importance of the hepcidin–ferroportin interaction in iron homeostasis. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? Related Article in Blood Online: Hepcidin: we always knew you were there Stanley L. Schrier Blood 2005 105: 3760. [Full Text] [PDF] This article has been cited by other articles: B. Young and J. Zaritsky Hepcidin for Clinicians Clin. J. Am. Soc. Nephrol., August 1, 2009; 4(8): 1384 - 1387. [Abstract] [Full Text] [PDF] S. A. Browne and D. Reddan Potential role of bone morphogenetic protein (BMP) signalling as a potential therapeutic target for modification of iron balance Nephrol. Dial. Transplant., January 1, 2009; 24(1): 28 - 30. [Full Text] [PDF] H. Tamary, H. Shalev, G. Perez-Avraham, M. Zoldan, I. Levi, D. W. Swinkels, T. Tanno, and J. L. 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