Effect of human beta (s)-globin chains on cellular properties of red cells from beta-thalassemic mice.
Effect of human beta (s)-globin chains on cellular properties of red cells from beta-thalassemic mice.
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人β-珠蛋白链对β-地中海贫血小鼠红细胞细胞特性的影响。
DOI：
10.1172/jci113670
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发表时间：
1988
期刊：
影响因子：
--
通讯作者：
Mohandas,N
中科院分区：
文献类型：
--
作者：
Rubin,EM;Kan,YW;Mohandas,N
转基因小鼠系统提供了一个体内环境，用于检查已基因导入动物基因组中的血红蛋白基因对小鼠红细胞的影响。在本报告中，我们分析了纯合 β 地中海贫血小鼠 (Hbbth-1/Hbbth-1)、含有人类 β-镰状 (beta(s)) 基因的纯合 β 地中海贫血转基因小鼠 (Hbb(th-1)/Hbb(th-1) + beta(s)) 和正常动物的红细胞的细胞特性。 Hbbth-1/Hbb(th-1) + beta(s) 转基因动物红细胞中人 β-珠蛋白链的存在对细胞变形性和密度分布以及这些动物的贫血程度具有显着影响。我们从这些研究中得出结论，红细胞变形性和密度分布是在细胞水平上评估通过基因引入整个生物体的珠蛋白基因的影响的敏感手段。此外，这些研究表明，过量 α 珠蛋白链数量的小幅减少可以显着改善 β 地中海贫血小鼠贫血的严重程度。
The transgenic mouse system provides an in vivo setting in which to examine the effects on mouse red cells of hemoglobin genes that have been genetically introduced into the animals' genome. In this report, we have analyzed the cellular properties of red cells from homozygous beta-thalassemic mice (Hbbth-1/Hbbth-1), homozygous beta-thalassemic transgenic mice containing a human beta-sickle (beta(s)) gene (Hbb(th-1)/Hbb(th-1) + beta(s)), and normal animals. The presence of human beta(s)-globin chains in red cells from the Hbbth-1/Hbb(th-1) + beta(s) transgenic animals was noted to have a significant effect on cellular deformability and density distribution, as well as on the degree of anemia in these animals. We conclude from these studies that red cell deformability and density distribution is a sensitive means for assessing at the cellular level the effects of globin genes genetically introduced into whole organisms. In addition, these studies suggest that small decreases in the amount of excess alpha-globin chains can significantly ameliorate the severity of anemia in the beta-thalassemic mouse.Images