Servizio di Immunoematologia e Medicina Trasfusionale, Dipartimento di Patologia e Medicina di Laboratorio, Azienda Ospedaliero-Universitaria di Parma, Italy.
Clin Chim Acta. 2010 Nov 11;411(21-22):1565-9. doi: 10.1016/j.cca.2010.07.003. Epub 2010 Jul 8.
Hepcidin is a liver-synthesized hormone that inhibits the cellular efflux of iron by binding to ferroportin and its subsequent degradation. The main role of hepcidin is regulation of ferroportin expression and cell membrane function. Recent studies implicate hepcidin in a variety of iron disorders in addition to its primary role in the regulation of systemic iron homeostasis. Hepcidin excess has a key pathologic role in anemia of inflammation, chronic kidney disease, and iron-refractory iron deficiency anemia, while hepcidin deficiency is responsible for most cases of familial hemochromatosis and iron-loading anemia. The most important advances on the role of hepcidin in normal and abnormal iron metabolism and the main clinical and diagnostic implications are summarized in this review.
亚铁调素是一种肝脏合成的激素,通过与铁蛋白和随后的降解结合来抑制铁的细胞外排。亚铁调素的主要作用是调节铁蛋白的表达和细胞膜功能。最近的研究表明,亚铁调素除了在调节系统铁平衡中起主要作用外,还与多种铁代谢紊乱有关。亚铁调素过多在炎症性贫血、慢性肾脏病和铁难治性缺铁性贫血中起着关键的病理作用,而亚铁调素缺乏则是导致大多数家族性血色病和铁负荷性贫血的原因。本文综述了亚铁调素在正常和异常铁代谢中的作用及其主要的临床和诊断意义的最重要进展。