Journal
BLOOD
Volume 126, Issue 17, Pages 2031-2037Publisher
AMER SOC HEMATOLOGY
DOI: 10.1182/blood-2015-07-658419
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Funding
- National Institutes of Health, National Cancer Institute [P30 CA016042]
- National Institutes of Health, National Institute of Allergy and Infectious Diseases [5P30 AI028697]
- National Institutes of Health, National Institute of Diabetes and Digestive and Kidney Diseases [R01 DK 065029]
- American Society of Hematology
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Inherited anemias with ineffective erythropoiesis, such as beta-thalassemia, manifest inappropriately low hepcidin production and consequent excessive absorption of dietary iron, leading to iron overload. Erythroferrone (ERFE) is an erythroid regulator of hepcidin synthesis and iron homeostasis. Erfe expression was highly increased in the marrow and spleen of Hbb Th3/1 mice (Th3/1), a mouse model of thalassemia intermedia. Ablation of Erfe in Th3/1 mice restored normal levels of circulating hepcidin at 6 weeks of age, suggesting ERFE could be a factor suppressing hepcidin production in beta-thalassemia. We examined the expression of Erfe and the consequences of its ablation in thalassemic mice from 3 to 12 weeks of age. The loss of ERFE in thalassemic mice led to full restoration of hepcidin mRNA expression at 3 and 6 weeks of age, and significant reduction in liver and spleen iron content at 6 and 12 weeks of age. Ablation of Erfe slightly ameliorated ineffective erythropoiesis, as indicated by reduced spleen index, red cell distribution width, and mean corpuscular volume, but did not improve the anemia. Thus, ERFE mediates hepcidin suppression and contributes to iron overload in a mouse model of beta-thalassemia.
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