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Volume 11 (2011), Pages 1357-1366
Mini-Review Article

Molecular Mechanisms Regulating Hepcidin Revealed by Hepcidin Disorders

Vita-Salute University and Division of Genetics and Cell Biology, San Raffaele Scientific Institute, Milan, Italy

Received 30 March 2011; Revised 27 June 2011; Accepted 28 June 2011

Academic Editor: Martin Goette

Copyright © 2011 Clara Camaschella and Laura Silvestri.


Iron is essential for human life, but toxic if present in excess. To avoid iron overload and maintain iron homeostasis, all cells are able to regulate their iron content through the post-transcriptional control of iron genes operated by the cytosolic iron regulatory proteins that interact with iron responsive elements on iron gene mRNA. At the systemic level, iron homeostasis is regulated by the liver peptide hepcidin. Disruption of these regulatory loops leads to genetic diseases characterized by iron deficiency (iron-refractory iron-deficiency anemia) or iron overload (hemochromatosis). Alterations of the same systems are also found in acquired disorders, such as iron-loading anemias characterized by ineffective erythropoiesis and anemia of chronic diseases (ACD) associated with common inflammatory conditions. In ACD, iron is present in the body, but maldistributed, being deficient for erythropoiesis, but sequestered in macrophages. Studies of the hepcidin regulation by iron and inflammatory cytokines are revealing new pathways that might become targets of new therapeutic intervention in iron disorders.