Cardarelli G, Anatra G M
Dipartimento di Scienze Cliniche, Policlinico Umberto I, Roma, Italy.
Minerva Med. 2003 Jun;94(3):135-9.
Hepcidin (hepatic bactericidal protein) is a protein synthesised by the hepatocyte belonging to the family of endogenous peptide antimicrobes. It is produced in large quantities by the liver, heart and spinal cord and then is excreted in the urine. This protein, sequenced on human chromosoma 19, can be found in 2 main forms: Hepc 20 and Hepc 25 aminoacids respectively with 8 cystein residues connected by disulphine bridges. Evidence of lipopolysaccharide hepatocyte and the high concentrations of iron tied to fransferrin are elements which stimulate the production and release of Hepcidin. The latter, interacting with beta-2microglobulin-HFE-TfR1 complex determines an iron retention within the macrophages of the entherocyte in the duodenal pit. Hepcidin is therefore an important molecule which is able to regulate iron homeostasis and play a most significant role in the etiopathogenesis of the hemochromatosis system and, as recently shown, of anemia in chronic inflammatory diseases.
铁调素(肝脏杀菌蛋白)是一种由肝细胞合成的蛋白质,属于内源性肽类抗菌物质家族。它在肝脏、心脏和脊髓中大量产生,然后通过尿液排出。这种在人类19号染色体上测序的蛋白质有两种主要形式:分别为含有8个通过二硫键连接的半胱氨酸残基的20个氨基酸的铁调素和25个氨基酸的铁调素。脂多糖肝细胞以及与转铁蛋白结合的高浓度铁是刺激铁调素产生和释放的因素。后者与β-2微球蛋白-HFE-TfR1复合物相互作用,决定十二指肠隐窝肠上皮细胞巨噬细胞内的铁潴留。因此,铁调素是一种重要分子,能够调节铁稳态,在血色素沉着症系统的发病机制中发挥重要作用,并且如最近所示,在慢性炎症性疾病的贫血发病机制中也发挥重要作用。