The protective role of Hepcidin against thalassemia

Thalassemia: It is a defect resulting from Thalassemia is a hereditary condition where there is an abnormal balance of the globin chains found in hemoglobin. In healthy individuals, each alpha and beta chain is produced at approximately the same rate. In thalassemia, there is reduced production of one of the globin chains compared to the other; as a result, the two chains do not match and the overall percentage of hemoglobin in the bloos is decreased. The primary cause of thalassemia is the formation of ineffective erythrocytes that have a high rate of hemolysis and that cannot carry adequate amounts of oxygen to the tissues throughout the body. In summary, thalassemia is a diverse range of hereditary (genetic) diseases caused by reduced production rates of either of the two types of hemoglobin chains (alpha and beta). Hemoglobin transports oxygen to red blood cells; when there is an imbalance in the ratio between alpha and beta chains of hemoglobin, chronic hemolytic anaemia will result.
Iron homeostasis in the body is regulated by hepcidin (Hp) which promotes iron transport from intestinal absorption of iron-containing foods to blood circulation. Specifically, Hp stimulates iron absorption from intestinal epithelial cells (Enterocytes). Conversely, Hp has been shown to inhibit the function of iron-export protein ferroportin (Fpn), thereby preventing iron from being transported out of storage sites such as liver and bone marrow cells. The relationship between thalassemic individuals and hepcidin occurs primarily as a result of the relationship between blood transfusions, anemia, erythropoiesis (production of red blood cells) and inflammation. Furthermore, in thalassemics, blood transfusions are associated with increased hepcidin levels due to the increased production of erythrocytes as a result of increased haemoglobin concentrations within the blood. The role of hepcidin is now understood to provide a key and protective factor in regulating iron metabolism in those suffering from Mediterranean anaemia. (thalassemia).