Regulation of hemoglobin synthesis during the development of the red cell (third of three parts).
Regulation of hemoglobin synthesis during the development of the red cell (third of three parts).
复制标题
红细胞发育过程中血红蛋白合成的调节（三部分中的第三部分）。
DOI：
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发表时间：
1977
影响因子：
158.5
通讯作者：
Edward J. Benz
中科院分区：
文献类型：
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作者：
A. Nienhuis;Edward J. Benz
在本文中，我们调查了有关红细胞中珠蛋白 mRNA 和血红蛋白积累的知识现状。我们试图研究实现这种高度分化状态所必需的众多过程的相互作用。最后，我们努力制定一些机制，使单个红细胞可能含有不同比例的特定血红蛋白。过去几年的特点是有关珠蛋白结构基因以及红系细胞中珠蛋白 mRNA 的结构、转录、加工和功能的知识的真正爆炸。现在似乎可以通过体外培养和检查红细胞集落来分析红细胞生成的早期阶段。导致特定球蛋白产生的初级分化事件，尤其是人类血红蛋白 F 的产生，现在可以通过实验获得。我们有充分的理由希望这些进展很快就能实现长期期望的治疗目标，即增强患有严重β链血红蛋白病的患者的血红蛋白F合成。我们的目的是回顾可能为改善遗传异常珠蛋白基因的患者的临床表型提供合理依据的科学信息。
In this article we have surveyed the current state of knowledge regarding the accumulation of globin mRNA and hemoglobin in red cells. We have attempted to examine the interplay of numerous processes that seem to be necessary to achieve this highly differentiated state. Finally, we have made an effort to formulate some of the mechanisms whereby individual red cells may come to contain varying proportions of specific hemoglobins. The past several years have been characterized by a veritable explosion of knowledge concerning the globin structure genes, and the structure, transcription, processing and function of globin mRNA in erythroid cells. It now seems possible to analyze the earlier stages of erythropoiesis by cultivation and examination of erythroid colonies in vitro. The primary differentiation events leading to the production of specific globins, especially for hemoglobin F production in man, are now experimentally accessible. There is good reason to hope that these advances will soon permit achievement of the long desired therapeutic goal of enhancing hemoglobin F synthesis in patients with severe beta-chain hemoglobinopathies. Our aim has been to review the scientific information that might provide the rationable for amelioration of the clinical phenotypes in patients inheriting abnormal globin genes.