• Sara Gardenghi, Pedro Ramos, Antonia Follenzi, Niva Rao, Eliezer A Rachmilewitz, Patricia J Giardina, Robert W Grady, and Stefano Rivella.
• Division of Hematology-Oncology, Department of Pediatrics, Children's Cancer and Blood Foundation Laboratories, Weill Cornell Medical College, New York, New York, USA.
• Ann. N. Y. Acad. Sci. 2010 Aug 1;1202:221-5.

AbstractHepcidin (HAMP) negatively regulates iron absorption, degrading the iron exporter ferroportin at the level of enterocytes and macrophages. We showed that mice with beta-thalassemia intermedia (th3/+) have increased anemia and iron overload. However, their hepcidin expression is relatively low compared to their iron burden. We also showed that the iron metabolism gene Hfe is down-regulated in concert with hepcidin in th3/+ mice. These observations suggest that low hepcidin levels are responsible for abnormal iron absorption in thalassemic mice and that down-regulation of Hfe might be involved in the pathway that controls hepcidin synthesis in beta-thalassemia. Therefore, these studies suggest that increasing hepcidin and/or Hfe expression could be a strategy to reduces iron overload in these animals. The goal of this paper is to review recent findings that correlate hepcidin, Hfe, and iron metabolism in beta-thalassemia and to discuss potential novel therapeutic approaches based on these recent discoveries.

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