Hepcidin and Hfe in iron overload in β‐thalassemia
Open accessAbstract
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.
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Iron overload in β thalassaemia occurs because dysfunctional erythropoiesis increases iron demand.
“Iron overload occurs in β thalassaemia due to the increased iron demand caused by dysfunctional erythropoiesis [16].”
From A battle for iron: host sequestration and Staphylococcus aureus acquisition (2012) · cites this paper as [16]
β thalassaemia is an inherited hemoglobin synthesis disorder caused by β globin mutations, leading to defective β chain production, ineffective erythropoiesis, abnormal α/β chain ratios, iron overload, and anemia.
“β Thalassaemia is an inherited disorder affecting hemoglobin synthesis. Mutations within the β globin gene cause diverse degrees of defective β chain production, ineffective erythropoiesis, improper α/βchain ratios, iron overload, and a range of anemia [16].”
From A battle for iron: host sequestration and Staphylococcus aureus acquisition (2012) · cites this paper as [16]
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A battle for iron: host sequestration and Staphylococcus aureus acquisition
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- Authors
- Gardenghi, S., Ramos, P., Follenzi, A., Rao, N., Rachmilewitz, E. A., Giardina, P. J., Grady, R. W., & Rivella, S.
- Affiliations
- Children's Cancer and Blood Foundation (US)
- Albert Einstein College of Medicine (US)
- Wolfson Medical Center (IL)
- Corresponding author
- Rivella, Stefano
- Venue
- Annals of the New York Academy of Sciences
- Publisher
- Wiley
- Published
- 2010-08
- Volume
- 1202
- Issue
- 1
- Pages
- 221-225
- ISSN
- 0077-8923 (ISSN-L)
- 1749-6632
- PMC
- PMC3652388
- OpenAlex
- W1883925236
- Cited by
- 27
- References
- 30
- Citations by year
- 2026: 1 · 2025: 1 · 2024: 5 · 2023: 1 · 2022: 1 · 2021: 3 · 2020: 2 · 2018: 2 · 2016: 3 · 2014: 1 · 2012: 1
- FWCI
- 1.19
- Type
- journal article
- Language
- en
- Open access
- green
- europepmc.org
- Locations
- Annals of the New York Academy of Sciences (publishedVersion)
- PubMed (publishedVersion)
- Europe PMC (PubMed Central) (acceptedVersion)
- PubMed Central (acceptedVersion)
- Funders
- National Institute of Diabetes and Digestive and Kidney Diseases (R01DK055463, R21DK065169, R01DK090554)
- Topics
- Health Sciences › Medicine › Hematology › Iron Metabolism and Disorders
- Health Sciences › Medicine › Genetics › Hemoglobinopathies and Related Disorders
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Cell Biology › Hemoglobin structure and function
- Keywords
- hepcidin, HFE gene, iron overload, beta-thalassemia, beta-thalassemia intermedia, iron metabolism, iron absorption, ferroportin, anemia, treatment options
- MeSH terms
- Hemochromatosis Protein, Anemia, Animals, Anti-Bacterial Agents, Genetic Vectors, Humans, Membrane Proteins, Genetic Therapy, Histocompatibility Antigens Class I, beta-Thalassemia, Iron Overload, Antimicrobial Cationic Peptides, Hepcidins
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