Iron dysregulation in beta-thalassemia
Iron dysregulation in beta-thalassemia作者机构：Department of Clinical MicroscopyFaculty of Allied Health SciencesChulalongkorn University Department of BiologyFaculty of ScienceChiang Mai University Molecular Pathology Laboratory.Institute of Molecular BiosciencesMahidol University
出 版 物：《Asian Pacific Journal of Tropical Medicine》 (亚太热带医药杂志（英文版）)
年 卷 期：2016年第9卷第11期
页 面：1013-1021页
核心收录：
基 金：supported by the Thailand Research Fund(BRG5780004, IRG5780009 and IRN58W0002) by a Research Chair Grant from the National Science and Technology Development Agency(NSTDA) supported by a Thai Royal Golden Jubilee Ph.D.Research Scholarship(PHD/0101/2553)
主 题：Beta-thalassemia Hepcidin Iron overload Ferroportin
摘 要：Iron deficiency anemia and iron overload conditions affect more than one billion people worldwide.Iron homeostasis involves the regulation of cells that export iron into the plasma and cells that utilize or store iron.The cellular iron balance in humans is primarily mediated by the hepcidin-ferroportin axis.Ferroportin is the sole cellular iron export protein,and its expression is regulated transcriptionally,post-transcriptionally and posttranslationally.Hepcidin,a hormone produced by liver cells,post-translationally regulates ferroportin expression on iron exporting cells by binding with ferroportin and promoting its internalization by endocytosis and subsequent degradation by lysosomes.Dysregulation of iron homeostasis leading to iron deposition in vital organs is the main cause of death in betathalassemia patients.Beta-thalassemia patients show marked hepcidin suppression,ineffective eiythropoiesis,anemia and iron overload.Beta-thalassemia is common in the Mediterranean region,Southeast Asia and the Indian subcontinent,and the focus of this review is to provide an update on the factors mediating hepcidin related iron dysregulation in beta-thalassemia disease.Understanding this process may pave the way for new treatments to ameliorate iron overloading and improve the long term prognosis of these patients.
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