Division of Hematology, Brigham and Women's Hospital, Boston, Massachusetts, USA.
Curr Opin Hematol. 2012 May;19(3):156-62. doi: 10.1097/MOH.0b013e328351c48b.
Heme biosynthesis requires a series of enzymatic reactions that take place in the cytosol and the mitochondria as well as the proper intercellular and intracellular trafficking of iron. Heme can also be acquired by intestinal absorption and intercellular transport. The purpose of this review is to highlight recent work on heme and iron transport with an emphasis on their relevance in erythropoiesis.
Whereas the enzymes responsible for heme biosynthesis have been identified, transport mechanisms for iron, heme, or heme synthesis intermediates are only emerging. Recent studies have shed light on how these molecules are transported among various cellular compartments, as well as tissues. Much of this progress can be attributed to the use of model organisms such as S. cerevisiae, C. elegans, D. rerio, and M. musculus. Genetic studies in these models have led to the identification of several new genes involved in heme metabolism. Although our understanding has greatly improved, it is highly likely that other regulators exist and additional work is required to characterize the pathways by which heme and iron are transported within the erythron.
The identification of heme and iron transport mechanisms will improve our understanding of blood development and provide new insight into human blood disorders.
血红素生物合成需要一系列在细胞质和线粒体中发生的酶促反应，以及铁的适当细胞内和细胞间转运。血红素也可以通过肠吸收和细胞间转运获得。本文综述的目的是强调血红素和铁转运在红细胞生成中的相关性，突出近年来在血红素和铁转运方面的工作。
虽然已经确定了负责血红素生物合成的酶，但铁、血红素或血红素合成中间产物的转运机制尚在出现中。最近的研究揭示了这些分子如何在各种细胞区室以及组织之间运输。这一进展在很大程度上归因于使用模型生物，如酿酒酵母、秀丽隐杆线虫、斑马鱼和小鼠。这些模型中的遗传研究导致了几个参与血红素代谢的新基因的鉴定。尽管我们的理解已经大大提高，但很可能还有其他调节剂存在，需要进一步的工作来描述血红素和铁在红细胞内运输的途径。
血红素和铁转运机制的鉴定将提高我们对血液发育的理解，并为人类血液疾病提供新的见解。
