Honig George R, Lu Shi-Jiang, Feng Qiang, Vida Loyda N, Lee Bao-Shiang, Lanza Robert
Department of Pediatrics, University of Illinois at Chicago, Chicago, Illinois, USA.
Hemoglobin. 2010 Jan;34(2):145-50. doi: 10.3109/03630261003676850.
Under culture conditions that promote hematopoietic differentiation, human embryonic stem cells (huESC) give rise to primitive erythroid cells that closely resemble the nucleated erythrocytes of early-stage human embryos. The globin chain distribution of these cells is similar to that seen during the embryonic and fetal stages of development. Here we show that huESC-derived erythroid cells produce substantial quantities of homotetrameric hemoglobin (Hb) composed exclusively of gamma-globin-containing subunits. The globin synthesis of these erythroid cells was also significantly unbalanced, with a substantial decrease of alpha-like globin chain synthesis in relation to that of their beta-like globins, a pattern characteristically associated with alpha-thalassemia (alpha-thal). This pattern of unbalanced globin synthesis appears to be an inherent feature of human erythroid cells that synthesize predominantly embryonic-stage globins.
在促进造血分化的培养条件下，人类胚胎干细胞（huESC）可产生原始红细胞，这些细胞与早期人类胚胎的有核红细胞极为相似。这些细胞的珠蛋白链分布与发育的胚胎期和胎儿期所见相似。在此我们表明，huESC来源的红细胞产生大量仅由含γ珠蛋白亚基组成的同型四聚体血红蛋白（Hb）。这些红细胞的珠蛋白合成也明显失衡，α样珠蛋白链合成相对于β样珠蛋白合成大幅减少，这种模式典型地与α地中海贫血（α-thal）相关。这种珠蛋白合成失衡模式似乎是主要合成胚胎期珠蛋白的人类红细胞的固有特征。
