Hepcidin and Hfe in iron overload in β-thalassemia Review uri icon

Overview

MeSH Major

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

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 2010

Research

keywords

  • Review

Identity

Language

  • eng

PubMed Central ID

  • PMC3652388

Digital Object Identifier (DOI)

  • 10.1111/j.1749-6632.2010.05595.x

PubMed ID

  • 20712796

Additional Document Info

start page

  • 221

end page

  • 5

volume

  • 1202