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羟基脲治疗β地中海贫血——羟基脲对珠蛋白基因表达的影响

Treatment of beta-thalassemia with hydroxyurea (HU)--effects of HU on globin gene expression.

作者信息

Huang S Z, Ren Z R, Chen M J, Xu H P, Zeng Y T, Rodgers G P, Zeng F Y, Schechter A N

机构信息

Shanghai Institute of Medical Genetics, Shanghai Children's Hospital, PRC.

出版信息

Sci China B. 1994 Nov;37(11):1350-9.

PMID:7865126
Abstract

A newly developed method of RT-PCR/competitive PCR for measuring the relative and absolute content of globin mRNAs as well as micro-globin chain biosynthetic assay have been used to study the alterations of globin gene expressions in the patients with beta-thalassemia pre- and post-hydroxyurea (HU) treatment. It was found for the first time that HU had the effect of enhancing beta-globin gene expression in some patients. Two cases with beta-thalassemia who were subjected to HU treatment for over two years showed a marked increase in beta-globin mRNA level and beta-globin chain synthesis, resulting in more effective erythropoiesis and the alleviation of clinical symptoms.

摘要

一种新开发的用于测量珠蛋白mRNA相对和绝对含量的逆转录聚合酶链反应/竞争性聚合酶链反应方法以及微球蛋白链生物合成测定法,已被用于研究β地中海贫血患者在羟基脲(HU)治疗前后珠蛋白基因表达的变化。首次发现HU对某些患者有增强β珠蛋白基因表达的作用。两名接受HU治疗超过两年的β地中海贫血患者,其β珠蛋白mRNA水平和β珠蛋白链合成显著增加,从而使红细胞生成更有效,并缓解了临床症状。

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1
Treatment of beta-thalassemia with hydroxyurea (HU)--effects of HU on globin gene expression.羟基脲治疗β地中海贫血——羟基脲对珠蛋白基因表达的影响
Sci China B. 1994 Nov;37(11):1350-9.
2
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Response to hydroxyurea therapy in beta-thalassemia.β地中海贫血对羟基脲治疗的反应
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Nan Fang Yi Ke Da Xue Xue Bao. 2009 Oct;29(10):2073-6, 2081.

引用本文的文献

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Regulating the Regulators: The Role of Histone Deacetylase 1 (HDAC1) in Erythropoiesis.调控调控因子:组蛋白去乙酰化酶 1(HDAC1)在红细胞生成中的作用。
Int J Mol Sci. 2020 Nov 11;21(22):8460. doi: 10.3390/ijms21228460.
2
Genotype-phenotype relationship of patients with β-thalassemia taking hydroxyurea: a 13-year experience in Iran.β-地中海贫血患者服用羟基脲的基因型-表型关系:伊朗 13 年的经验。
Int J Hematol. 2012 Jan;95(1):51-6. doi: 10.1007/s12185-011-0985-6. Epub 2011 Dec 20.
3
The hydroxyurea-induced small GTP-binding protein SAR modulates gamma-globin gene expression in human erythroid cells.
羟基脲诱导的小GTP结合蛋白SAR调节人类红系细胞中的γ-珠蛋白基因表达。
Blood. 2005 Nov 1;106(9):3256-63. doi: 10.1182/blood-2003-10-3458. Epub 2005 Jun 28.