File:Pharmaceuticals-11-00131-g001-550.jpg

English: Figure 1. Schematic representation of the pathophysiological mechanisms leading to the development of iron overload during HFE-related hemochromatosis. (A): Normal situation with adequate HFE signaling allowing control of transferrin saturation level (<45%). (B): Genetic hemochromatosis with low activity of HFE-related signaling that limits hepcidin expression and in turn favors iron release in plasma, despite the presence of a sufficient amount of iron. Thus, the transferrin saturation increases and non-transferrin bound iron appears and targets organs such as liver, leading to abnormal iron accumulation.