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  • Title: alpha-Thalassemia-like globin gene expression by primitive erythrocytes derived from human embryonic stem cells.
    Author: Honig GR, Lu SJ, Feng Q, Vida LN, Lee BS, Lanza R.
    Journal: Hemoglobin; 2010 Jan; 34(2):145-50. PubMed ID: 20353349.
    Abstract:
    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.
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