Huebers H A, Finch C A
Blood. 1984 Oct;64(4):763-7.
The transferrin iron transport system, along with its procurement sites and delivery receptors, provides a highly effective means of satisfying internal iron requirements. Iron uptake by individual tissues is determined by their receptor number, by the relative amounts of monoferric and diferric transferrin in circulation, and by the amount of available iron in donor tissues. Although the modus operandi of this system under basal conditions has been characterized, its exquisite regulation remains an enigma. In some manner, the procurement of iron is determined by iron requirements. What seems to be an inappropriate behavior of the absorptive mechanism in thalassemia and certain other erythroid overload states may actually be life-saving in the absence of transfusion, since it results in higher levels of plasma iron and thereby higher levels of erythropoiesis. The definition of the regulatory mechanism in such conditions may well lead to an understanding of the molecular defect in idiopathic hemochromatosis.
转铁蛋白铁转运系统及其获取位点和递送受体，提供了一种满足体内铁需求的高效方式。各个组织对铁的摄取取决于它们的受体数量、循环中单核铁转铁蛋白和双核铁转铁蛋白的相对量，以及供体组织中可利用铁的量。尽管该系统在基础条件下的运作方式已得到描述，但其精确调控仍是一个谜。铁的获取在某种程度上由铁需求决定。在没有输血的情况下，地中海贫血和某些其他红系过载状态下吸收机制看似不适当的行为实际上可能是救命的，因为它会导致血浆铁水平升高，从而使红细胞生成水平升高。在这些情况下对调节机制的定义很可能有助于理解特发性血色素沉着症的分子缺陷。
