Hepcidin and Hfe in iron overload in beta-thalassemia. Review uri icon

Overview

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.

publication date

  • August 1, 2010

Research

keywords

  • Antimicrobial Cationic Peptides
  • Histocompatibility Antigens Class I
  • Iron Overload
  • Membrane Proteins
  • beta-Thalassemia

Identity

PubMed Central ID

  • PMC3652388

Scopus Document Identifier

  • 77955906936

Digital Object Identifier (DOI)

  • 10.1111/j.1749-6632.2010.05595.x

PubMed ID

  • 20712796

Additional Document Info

volume

  • 1202