作者: Nicole Wilkinson , Kostas Pantopoulos , Giada Sebastiani
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摘要: The iron regulatory hormone hepcidin limits fluxes to the bloodstream by promoting degradation of exporter ferroportin in target cells. Hepcidin insufficiency causes hyperabsorption dietary iron, hyperferremia and tissue overload, which are hallmarks hereditary hemochromatosis. Similar responses also observed iron-loading anemias due ineffective erythropoiesis (such as thalassemias, dyserythropoietic myelodysplastic syndromes) chronic liver diseases. On other hand, excessive expression inhibits absorption leads hypoferremia retention within macrophages. This reduces availability for erythroblasts contributes development with iron-restricted anemia disease iron-refractory iron-deficiency anemia). Pharmacological targeting hepcidin/ferroportin axis may offer considerable therapeutic benefits correcting traffic. review summarizes principles underlying hepcidin-based therapies treatment iron-related disorders, discusses emerging strategies manipulating pathways.