Thalassemia Research Center, Institute of Molecular Biosciences, Mahidol University, Salaya, Nakornpathom, Thailand.
Ann Hematol. 2010 Oct;89(10):953-8. doi: 10.1007/s00277-010-0980-7. Epub 2010 May 12.
MicroRNAs (miRNAs) are negative regulators of gene expression that play an important role in hematopoiesis. Thalassemia, a defective globin synthesis leading to precipitate of excess unbound globins in red blood cell precursors, results in defective erythroid precursors and ineffective erythropoiesis. Expression pattern of miR-451, an erythroid-specific miRNA, was analyzed during differentiation of erythroid progenitors derived from normal and thalassemic peripheral blood CD34-positive cells, after 14 days of culture. A biphasic expression with transient up-regulation of miRNA-451 on day 3 of cultures was observed during thalassemic erythroid differentiation. In contrast, the expression pattern of the miR-451 in erythroid cells obtained from the other extravascular hemolytic anemia, i.e., hereditary spherocytosis patients showed no transient up-regulation of miR-451 on day 3 of cultures. Our results suggest that early erythroid progenitors in beta-thalassemia have a dysregulated miRNA-451 expression program, and analysis of microRNA is a relevant approach to determine abnormalities of erythropoiesis.
微小 RNA（miRNA）是基因表达的负调控因子，在造血过程中发挥重要作用。地中海贫血是一种球蛋白合成缺陷导致过多未结合的球蛋白在红细胞前体中沉淀的疾病，导致红细胞前体缺陷和无效的红细胞生成。分析了正常和地中海贫血外周血 CD34+细胞来源的红细胞祖细胞在培养 14 天后分化过程中，红细胞特异性 miRNA miR-451 的表达模式。在地中海贫血性红细胞分化过程中，观察到 miRNA-451 的表达呈双峰型，培养第 3 天短暂上调。相比之下，从其他血管外溶血性贫血（即遗传性球形红细胞增多症患者）获得的红细胞中 miR-451 的表达模式在培养第 3 天没有出现 miR-451 的短暂上调。我们的结果表明，β-地中海贫血的早期红细胞前体细胞存在 miRNA-451 表达程序失调，分析 microRNA 是确定红细胞生成异常的一种相关方法。
