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Haematologica. 2012 Feb;97(2):189-92. doi: 10.3324/haematol.2011.054031. Epub 2011 Oct 11.

Hemojuvelin is essential for transferrin-dependent and transferrin-independent hepcidin expression in mice.

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  • 1Department of Pathology, Children's Hospital, Boston, MA 02115, USA. tbartnikas@enders.tch.harvard.edu

Abstract

Here we investigate the regulation of hepcidin, a hormone that inhibits dietary iron absorption and macrophage iron recycling, by the serum iron-binding protein transferrin. Mice deficient in transferrin (Tf(hpx/hpx)) and hemojuvelin (Hjv(-/-)), a gene mutated in juvenile hemochromatosis, a disease of hepcidin deficiency and iron overload, were generated. While Tf(hpx/hpx) Hjv(+/+) and Tf(hpx/hpx) Hjv(-/-) phenotypes did not differ markedly, transferrin treatment and RBC transfusions robustly increased hepcidin levels in Tf(hpx/hpx) Hjv(+/+) but not Tf(hpx/hpx) Hjv(-/-)mice. These results suggest that, while hemojuvelin is not essential for the establishment or maintenance of hepcidin deficiency in transferrin-deficient mice, hemojuvelin is essential for transferrin-dependent and transferrin-independent hepcidin expression in conditions of iron overload.

PMID:
21993681
[PubMed - indexed for MEDLINE]
PMCID:
PMC3269476
Free PMC Article
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