Suppr超能文献

雷帕霉素对β地中海贫血患者红系前体细胞中α、β和γ珠蛋白mRNA积累的影响。

Effects of rapamycin on accumulation of alpha-, beta- and gamma-globin mRNAs in erythroid precursor cells from beta-thalassaemia patients.

作者信息

Fibach Eitan, Bianchi Nicoletta, Borgatti Monica, Zuccato Cristina, Finotti Alessia, Lampronti Ilaria, Prus Eugenia, Mischiati Carlo, Gambari Roberto

机构信息

Department of Haematology, Hadassah - Hebrew University Medical Centre, Jerusalem, Israel.

出版信息

Eur J Haematol. 2006 Nov;77(5):437-41. doi: 10.1111/j.1600-0609.2006.00731.x. Epub 2006 Aug 24.

Abstract

We studied the effects of rapamycin on cultures of erythroid progenitors derived from the peripheral blood of 10 beta-thalassaemia patients differing widely with respect to their potential to produce foetal haemoglobin (HbF). For this, we employed the two-phase liquid culture procedure for growing erythroid progenitors, high performance liquid chromatography for analysis of HbF production and reverse transcription polymerase chain reaction for quantification of the accumulation of globin mRNAs. The results demonstrated that rapamycin induced an increase of HbF in cultures from all the beta-thalassaemia patients studied and an increase of their overall Hb content/cell. The inducing effect of rapamycin was restricted to gamma-globin mRNA accumulation, being only minor for beta-globin and none for alpha-globin mRNAs. The ability of rapamycin to preferentially increase gamma-globin mRNA content and production of HbF in erythroid precursor cells from beta-thalassaemia patients is of great importance as this agent (also known as sirolimus or rapamune) is already in clinical use as an anti-rejection agent following kidney transplantation. These data suggest that rapamycin warrants further evaluation as a potential therapeutic drug in beta-thalassaemia and sickle cell anaemia.

摘要

我们研究了雷帕霉素对来自10名β地中海贫血患者外周血的红系祖细胞培养物的影响,这些患者在产生胎儿血红蛋白(HbF)的潜力方面差异很大。为此,我们采用两阶段液体培养程序来培养红系祖细胞,用高效液相色谱法分析HbF的产生情况,并用逆转录聚合酶链反应来定量珠蛋白mRNA的积累。结果表明,雷帕霉素使所有研究的β地中海贫血患者培养物中的HbF增加,且细胞的总体血红蛋白含量也增加。雷帕霉素的诱导作用仅限于γ珠蛋白mRNA的积累,对β珠蛋白mRNA的影响较小,对α珠蛋白mRNA则无影响。雷帕霉素优先增加β地中海贫血患者红系前体细胞中γ珠蛋白mRNA含量和HbF产生的能力非常重要,因为这种药物(也称为西罗莫司或雷帕鸣)已在临床上用作肾移植后的抗排斥药物。这些数据表明,雷帕霉素作为β地中海贫血和镰状细胞贫血的潜在治疗药物值得进一步评估。

文献AI研究员

20分钟写一篇综述,助力文献阅读效率提升50倍。

立即体验

用中文搜PubMed

大模型驱动的PubMed中文搜索引擎

马上搜索

文档翻译

学术文献翻译模型,支持多种主流文档格式。

立即体验