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Hepcidin and Hfe in iron overload in β‐thalassemia
Author(s) -
Gardenghi Sara,
Ramos Pedro,
Follenzi Antonia,
Rao Niva,
Rachmilewitz Eliezer A.,
Giardina Patricia J.,
Grady Robert W.,
Rivella Stefano
Publication year - 2010
Publication title -
annals of the new york academy of sciences
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.712
H-Index - 248
eISSN - 1749-6632
pISSN - 0077-8923
DOI - 10.1111/j.1749-6632.2010.05595.x
Subject(s) - hepcidin , hamp , ferroportin , beta thalassemia , thalassemia , iron homeostasis , dietary iron , medicine , endocrinology , hemochromatosis , anemia , chemistry , metabolism , iron deficiency
Hepcidin (HAMP) negatively regulates iron absorption, degrading the iron exporter ferroportin at the level of enterocytes and macrophages. We showed that mice with β‐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 β‐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 β‐thalassemia and to discuss potential novel therapeutic approaches based on these recent discoveries.