Hemoglobin synthesis pathway and iron homeostasis

The hemoglobin synthesis pathway and iron homeostasis describe the series of molecular and cellular processes required for delivering iron to developing erythrocytes, its incorporation into heme, and subsequent assembly into the hemoglobin molecule. Iron is transported in blood bound to transferrin, taken up by erythroid precursors via the transferrin receptor, and inserted into protoporphyrin IX by ferrochelatase to generate heme, with tight regulation at several enzymatic and transport steps. Cellular and systemic iron storage involves proteins such as ferritin and processes mediated by hepcidin, ferroportin, and various import/export transporters; dysregulation leads to a spectrum of clinical disorders including anemia, sideroblastic anemia, and iron overload syndromes. Therapeutic strategies often target iron availability, iron transport/storage proteins, or the regulators in this pathway.

Other names

Iron metabolism pathwayIron stores regulationErythropoiesis iron pathwayHeme synthesis pathway

Iron chelation (removing/binding excess iron); Modulation of iron absorption and release (e.g. via hepcidin, ferroportin); Promotion of erythropoiesis; Altering expression/activity of enzymes/transporter proteins in the pathway