[Role of hepcidin in the pathogenesis of hemochromatosis].
In the last few years, biochemical and molecular study of the various types of hemochromatosis have established that the hepcidin peptide is the central regulator of iron absorption. This peptide, which is synthesized in the liver, acts through ferroportin degradation. Ferroportin is an iron exporter situated in the intestinal epithelium and in the macrophage membrane whose function is to transport iron from the intestinal cell to plasma and from the macrophage to the erythron. In hemochromatosis, there is a physical or functional hepcidin deficit that increases ferroportin, thus producing excessive iron absorption. The opposite occurs in situations of inflammation: hepcidin synthesis is stimulated while iron entry into the organism and hemoglobin synthesis are blocked.
Altes Hernández
Albert
A
Servei d'Hematologia i Hemoteràpia, Fundació Althaia, Manresa, Barcelona, España. aaltesh@althaia.cat
Aranalde Fortó
Joan
J
D023181:Antimicrobial Cationic Peptides / Q000502:physiology*; D006432:Hemochromatosis / Q000209:etiology*; D064451:Hepcidins; D006801:Humans
D023181:Antimicrobial Cationic Peptides; C578702:HAMP protein, human; D064451:Hepcidins