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

First Author  Bartnikas TB Year  2012
Journal  Haematologica Volume  97
Issue  2 Pages  189-92
PubMed ID  21993681 Mgi Jnum  J:219070
Mgi Id  MGI:5619453 Doi  10.3324/haematol.2011.054031
Citation  Bartnikas TB, et al. (2012) Hemojuvelin is essential for transferrin-dependent and transferrin-independent hepcidin expression in mice. Haematologica 97(2):189-92
abstractText  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.
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