V. Sankaran
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摘要

红细胞生成是一个高度调控的多步骤过程,在这个过程中,干细胞经过一系列的扩增分裂,产生多电位祖细胞,然后是寡系红细胞祖细胞,最后是单系红细胞祖细胞,然后是形态学上可识别的红细胞前体和成熟红细胞。红细胞生成的个体发生涉及胚胎期和胎儿早期一系列协调良好的事件。在胎儿中,红细胞生成的主要部位是肝脏,最初主要产生胎儿血红蛋白(HbF, α - 2γ - 2)和一小部分(10-15%)成人血红蛋白(HbA, α - 2β - 2),出生时HbA的比例上升到50%左右。出生后,红细胞生成终生维持的部位是骨髓,在出生后几个月达到最终的成体红细胞模式(成体Hb与胎儿Hb <1%)。红细胞生成的调节——主要的调节因子是促红细胞生成素,这是一种由肾间质细胞在组织缺氧时产生的咽糖蛋白,通过与特定受体结合在红细胞爆发形成单位、红细胞集落形成单位和原红细胞上发挥作用。促红细胞生成素产生异常-贫血可由后天或先天性促红细胞生成素产生不足引起,最常见于慢性肾脏疾病。组织氧输送受损是促红细胞生成素驱动的继发性红细胞增多症的常见原因。一些肾癌增加促红细胞生成素的产生,从而引起继发性红细胞增多。其他导致红细胞生成异常的原因包括:(1)后天和先天的促红细胞生成素信号缺陷;(2)获得性和先天性转录因子GATA1或EKLF缺陷;(3)后天或先天核糖体合成或剪接因子异常;(4)导致红细胞过早破坏的因素。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Erythropoiesis
Erythropoiesis is a highly regulated, multistep process in which stem cells, after a series of amplification divisions, generate multipotential progenitor cells, then oligo- and finally unilineage erythroid progenitors, and then morphologically recognizable erythroid precursors and mature red cells. The ontogeny of erythropoiesis involves a series of well-coordinated events during embryonic and early fetal life. In the fetus, the main site of erythropoiesis is the liver, which initially produces mainly fetal haemoglobin (HbF, α‎2γ‎2) and a small component (10–15%) of adult haemoglobin (HbA, α‎2β‎2), with the fraction of HbA rising to about 50% at birth. After birth, the site of erythroid cell production maintained throughout life is the bone marrow, with the final adult erythroid pattern (adult Hb with <1% fetal Hb) being reached a few months after birth. Regulation of erythropoiesis—the main regulator is erythropoietin, a sialoglycoprotein that is produced by interstitial cells in the kidney in response to tissue hypoxia and exerts its effect by binding to a specific receptor on erythroid burst-forming units, erythroid colony-forming units, and proerythroblasts. Abnormal erythropoietin production—anaemia can be caused by acquired or congenital deficiency in erythropoietin production, most commonly in chronic kidney disease. Impaired tissue oxygen delivery is a common cause of erythropoietin-driven secondary erythrocytosis. Some kidney cancers increase erythropoietin production and hence cause secondary erythrocytosis. Other causes of abnormal erythroid production include (1) acquired and congenital defects in erythropoietin signalling; (2) acquired and congenital defects in the transcription factors GATA1 or EKLF; (3) acquired or congenital abnormalities in ribosome synthesis or splicing factors; and (4) factors that lead to premature red cell destruction.
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