Hemojuvelin is essential for transferrin-dependent and transferrin-independent hepcidin expression in mice
Copyright policy )Hemojuvelin is essential for transferrin-dependent and transferrin-independent hepcidin expression in mice
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.
- Boston Children's Hospital United States
- CHILDRENS HOSPITAL CORPORATION
Mice, Knockout, Mice, Inbred BALB C, Iron Overload, Transferrin, Membrane Proteins, GPI-Linked Proteins, Mice, Inbred C57BL, Mice, Gene Expression Regulation, Hepcidins, Animals, Diseases of the blood and blood-forming organs, RC633-647.5, Hemochromatosis Protein, Antimicrobial Cationic Peptides
Mice, Knockout, Mice, Inbred BALB C, Iron Overload, Transferrin, Membrane Proteins, GPI-Linked Proteins, Mice, Inbred C57BL, Mice, Gene Expression Regulation, Hepcidins, Animals, Diseases of the blood and blood-forming organs, RC633-647.5, Hemochromatosis Protein, Antimicrobial Cationic Peptides
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