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Ann N Y Acad Sci. 2010 Aug;1202:221-5. doi: 10.1111/j.1749-6632.2010.05595.x.

Hepcidin and Hfe in iron overload in beta-thalassemia.

Author information

1
Division of Hematology-Oncology, Department of Pediatrics, Children's Cancer and Blood Foundation Laboratories, Weill Cornell Medical College, New York, New York, USA.

Abstract

Hepcidin (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.

PMID:
20712796
PMCID:
PMC3652388
DOI:
10.1111/j.1749-6632.2010.05595.x
[Indexed for MEDLINE]
Free PMC Article

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