血红素是红细胞中必要辅基及关键调控者  

Heme is an essential prosthetic group and a key regulator during erythropoiesis

作　　者：刘金花 高洁 佟静媛 李亚朴 石莉红 LIU JinHua;GAO Jie;TONG JingYuan;LI YaPu;SHI LiHong(State Key Laboratory of Experimental Hematology, Institute of Hematology, Blood Disease Hospital, Chinese Academy of Medical Sciences ＆ Peking Union Medical College, Tianjin 300020, Chin)

机构地区：[1]中国医学科学院&北京协和医学院血液病医院(血液学研究所),实验血液学国家重点实验室,天津300020

出　　处：《中国科学：生命科学》2017年第12期1253-1263,共11页Scientia Sinica(Vitae)

基　　金：天津市应用基础面上项目(批准号:15JCYBJC54500);中国医学科学院医学与健康科技创新工程项目(批准号:2016-12M-1-018,2016-I2M-3-002);国家自然科学基金(批准号:31471291,81700105)资助

摘　　要：血红素不但能作为血红素蛋白的重要辅基参与细胞电子传递、送氧、酶作用等重要生理活动,而且是红细胞稳态及分化的关键调控者.本综述主要从3个方面小结了红细胞中血红素的研究进展.首先,在血红素生物合成方面,介绍了血红素生物合成过程、其起始及中间产物的跨膜运输及调控机制的研究现状.其次,重点阐述了血红素作为红细胞稳态及分化的调控者对红系特异基因的转录调控、mRNA稳定性及翻译、蛋白稳定性、miRNA加工合成等过程的调控机制.最后,简单总结了血红素的合成异常导致的疾病及可能的治疗策略.总之,红细胞稳态的维持需要协调平衡血红素合成,蛋白(尤其是珠蛋白)产生以及铁代谢等生物过程,对它们调控关系的阐明有助于深入了解红细胞分化的调控机制,同时也对红细胞相关疾病的研究及治疗有指导意义.Heme, as a structural component of hemoprotein, is an essential prosthetic group which is involved in various biological processes such as oxygen transfer and storage and electron transfer. During erythroid differentiation, heme biosynthesis is robustly induced to meet the mass production of hemoglobin for body needs. Accumulating evidence indicates that heme biosynthesis in erythroid cells is elaborately orchestrated with globin formation and iron hemostasis which ensures the efficient erythropoiesis. Defects of heme biosynthesis in developing erythroblasts can have profound medical implications, resulting in sideroblastic anemias or erythropoietic porphyrias. In this review, we focus on not only the regulatory processes of heme biosynthesis pathway in erythroid cells but also the regulatory function of heme during erythropoiesis. We also discuss regulatory mechanism and potential therapeutic strategy in erythroid disorders caused by heme deficiency.

关 键 词：血红素 红细胞 分化调控 卟啉症 铁幼粒细胞性贫血 

分 类 号：R55[医药卫生—血液循环系统疾病]