title: alpha-Thalassemia-like globin gene expression by primitive erythrocytes derived from human embryonic stem cells.
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.
authors.affiliation: Department of Pediatrics, University of Illinois at Chicago, Chicago, Illinois, USA. ghonig@uic.edu
authors.forename: George R
authors.initials: GR
authors.lastname: Honig
authors.forename: Shi-Jiang
authors.initials: SJ
authors.lastname: Lu
authors.forename: Qiang
authors.initials: Q
authors.lastname: Feng
authors.forename: Loyda N
authors.initials: LN
authors.lastname: Vida
authors.forename: Bao-Shiang
authors.initials: BS
authors.lastname: Lee
authors.forename: Robert
authors.initials: R
authors.lastname: Lanza
mesh_terms: D002478:Cells, Cultured / Q000166:cytology / Q000378:metabolism; D053595:Embryonic Stem Cells / Q000166:cytology*; D004900:Erythroblasts / Q000378:metabolism*; D004920:Erythropoiesis / Q000235:genetics*; D018507:Gene Expression Regulation, Developmental; D006455:Hemoglobins, Abnormal / Q000096:biosynthesis / Q000235:genetics; D006801:Humans; D055542:alpha-Globins / Q000096:biosynthesis* / Q000235:genetics; D017085:alpha-Thalassemia / Q000235:genetics*; D055544:beta-Globins / Q000096:biosynthesis* / Q000235:genetics; D055545:gamma-Globins / Q000096:biosynthesis* / Q000235:genetics; D055543:zeta-Globins / Q000096:biosynthesis / Q000235:genetics
chemical_list: D006455:Hemoglobins, Abnormal; D055542:alpha-Globins; D055544:beta-Globins; D055545:gamma-Globins; D055543:zeta-Globins; C030447:hemoglobin Portland; C030446:hemoglobin Gower; C030448:hemoglobin Bart's