Known and potential roles of transferrin in iron biology
Known and potential roles of transferrin in iron biology
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DOI：
10.1007/s10534-012-9520-3
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发表时间：
2012-08-01
期刊：
影响因子：
3.5
通讯作者：
Bartnikas, Thomas Benedict
中科院分区：
文献类型：
--
作者：
Bartnikas, Thomas Benedict
转铁蛋白是一种丰富的血清金属结合蛋白，因其在铁转运中的作用而闻名。人类疾病、先天性转铁蛋白缺乏症和这种疾病的动物模型突出了转铁蛋白在红血球生成和铁代谢中的重要作用。缺乏转铁蛋白的患者和小鼠表现为贫血和自相矛盾的铁超载，归因于缺乏海普西丁，海普西丁是一种主要由肝脏合成的多肽激素，它抑制饮食中铁的吸收和巨噬细胞铁的外流。对遗传性人类疾病和模式生物的研究表明，转铁蛋白是海普西丁表达的重要调节因子。在这篇文章中，我们回顾了目前关于转铁蛋白缺乏症的文献，并介绍了我们的最新发现，包括转铁蛋白、铁和锰在调节海普西丁表达方面的潜在重叠。
Transferrin is an abundant serum metal-binding protein best known for its role in iron delivery. The human disease congenital atransferrinemia and animal models of this disease highlight the essential role of transferrin in erythropoiesis and iron metabolism. Patients and mice deficient in transferrin exhibit anemia and a paradoxical iron overload attributed to deficiency in hepcidin, a peptide hormone synthesized largely by the liver that inhibits dietary iron absorption and macrophage iron efflux. Studies of inherited human disease and model organisms indicate that transferrin is an essential regulator of hepcidin expression. In this paper, we review current literature on transferrin deficiency and present our recent findings, including potential overlaps between transferrin, iron and manganese in the regulation of hepcidin expression.