Heme metabolism and erythropoiesis.
Heme metabolism and erythropoiesis.
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DOI：
10.1097/moh.0b013e328351c48b
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发表时间：
2012-05
影响因子：
3.2
通讯作者：
Paw BH
中科院分区：
文献类型：
--
作者：
Chung J;Chen C;Paw BH
血红素的生物合成需要一系列的酶促反应，这些酶促反应发生在细胞质和线粒体中，以及铁在细胞间和细胞内的适当运输。血红素也可以通过肠道吸收和细胞间运输获得。这篇综述的目的是强调血红素和铁运输的最新工作，重点是它们在红细胞生成中的相关性。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体的运输机制还在研究中。最近的研究揭示了这些分子是如何在不同的细胞区室和组织之间运输的。这一进展在很大程度上可归功于模式生物的使用，如酿酒葡萄球菌、秀丽隐杆线虫、葡萄球菌和肌肉分枝杆菌。在这些模型中进行的遗传研究已经鉴定出几个参与血红素代谢的新基因。虽然我们的理解已经有了很大的提高，但很有可能存在其他调节因子，并且需要额外的工作来表征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确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。
Heme biosynthesis requires a series of enzymatic reactions that take place in the cytosol and the mitochondria as well as the proper inter- 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. While 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 into 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.