Molecular control of iron transport
Molecular control of iron transport
复制标题
DOI：
10.1681/asn.2006070802
复制
发表时间：
2007-02-01
影响因子：
13.6
通讯作者：
Ganz, Tomas
中科院分区：
文献类型：
--
作者：
Ganz, Tomas
铁调节激素铁调素是在肝细胞中合成的25个氨基酸的肽。铁调素与细胞铁输出通道膜铁转运蛋白结合并引起其内化和降解，从而减少铁从铁输出组织流出到血浆中。通过这种机制，铁调素抑制膳食铁吸收，脾和肝巨噬细胞回收铁的流出，以及肝细胞储存铁的释放。铁调素合成受血浆铁和铁储存的刺激，并受红细胞生成活性的抑制，确保细胞外血浆铁浓度和铁储存保持稳定，满足红细胞生成对铁的需求。在炎症期间，铁调素浓度增加导致巨噬细胞中的铁螯合，导致低铁血症并最终导致炎症性贫血。铁调素缺乏症在大多数铁超载疾病中起着核心作用。铁调素异常在与肾脏疾病相关的贫血和对红细胞生成疗法的抵抗中的作用仍有待阐明。
The iron-regulatory hormone hepcidin is a 25-amino acid peptide that is synthesized in hepatocytes. Hepcidin binds to the cellular iron export channel ferroportin and causes its internalization and degradation and thereby decreases iron efflux from iron exporting tissues into plasma. By this mechanism, hepcidin inhibits dietary iron absorption, the efflux of recycled iron from splenic and hepatic macrophages, and the release of iron from storage in hepatocytes. Hepcidin synthesis is stimulated by plasma iron and iron stores and is inhibited by erythropoietic activity, ensuring that extracellular plasma iron concentrations and iron stores remain stable and the erythropoietic demand for iron is met. During inflammation, increased hepcidin concentrations cause iron sequestration in macrophages, resulting in hypoferremia and eventually anemia of inflammation. Hepcidin deficiency plays a central role in most iron overload disorders. The role of hepcidin abnormalities in anemias that are associated with renal disease and in resistance to erythropoietic therapies remains to be elucidated.