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[健康人和血红蛋白病患者外周血红细胞前体体外培养中胎儿血红蛋白的优先合成]

[Preferential synthesis of fetal hemoglobin in in vitro cultures of erythroid precursors from peripheral blood of healthy persons and those with hemoglobinopathies].

作者信息

Efremov G D, Huisman T H

出版信息

Bilt Hematol Transfuz. 1979;7(1 Suppl):39-45.

PMID:95430
Abstract

The synthesis of fetal hemoglobin was investigated in in vitro cultures of erythroid precursors isolated from peripheral blood of normal individuals, newborns and of subjects with different hemoglobinopathies. Synthesis of hemoglobin was assessed by 35s-methionine labeling of cultures and measurement of the radioactivity incorporated into the hemoglobins A2, A, F and S isolated by column chromatography on DE 52 cellulose. The erythroid precursors from most of the studied individuals cultured in in vitro system responded with synthesized an average of 15% of Hb F while cultures from newborns produced an average of 60% of Hb F in comparison of 73% of Hb F in peripheral blood of the same newborns. Erythroid precusors from subjects heterozygotes for beta-thalassemia, heterozygotes for HPFH, and homozygotes for Hb S produced an average of 20%, 43% and 30% of Hb F, respectively, in comparison of 7%, 14% and 9% of Hb F, respectively, present in the RBC of the same individuals. These data support the previously published results (6-11) that erythroid bursts in culture reactivate the structural genes for the gamma chain synthesis.

摘要

在从正常个体、新生儿以及患有不同血红蛋白病的受试者外周血中分离出的红系前体细胞的体外培养中,对胎儿血红蛋白的合成进行了研究。通过用³⁵S-甲硫氨酸标记培养物并测量通过DE 52纤维素柱色谱法分离出的血红蛋白A2、A、F和S中掺入的放射性来评估血红蛋白的合成。在体外系统中培养的大多数研究个体的红系前体细胞合成的Hb F平均为15%,而新生儿的培养物产生的Hb F平均为60%,相比之下,同一新生儿外周血中的Hb F为73%。β地中海贫血杂合子、遗传性胎儿血红蛋白持续存在(HPFH)杂合子和Hb S纯合子受试者的红系前体细胞分别产生平均20%、43%和30%的Hb F,相比之下,同一受试者红细胞中分别存在7%、14%和9%的Hb F。这些数据支持了先前发表的结果(6 - 11),即培养中的红系集落重新激活了γ链合成的结构基因。

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