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  • Title: [Analysis of the relative rates of synthesis of G gamma and A gamma globin chains in the erythropoietic bursts in patients with trisomy 13 syndrome].
    Author: Sasaki H, Matsuda M, Terasawa T, Ito T, Fukushima Y, Kuroki Y, Matsuyama S.
    Journal: Nihon Ketsueki Gakkai Zasshi; 1989 May; 52(3):583-7. PubMed ID: 2482646.
    Abstract:
    To clarify the pathogenesis of fetal-like erythropoiesis including high levels of Hb F observed in trisomy 13 syndrome, we examined the biosynthesis rates of G gamma and A gamma globin chains in the erythropoietic bursts cultured from the peripheral blood mononuclear cells of 3 patients aged 1-month-, 2-month and 7-month-old. Globin chains were labeled with 14C-amino acids, separated by isoelectric focusing and quantitated by autoradiography. The synthesis rates of gamma-chains in the erythropoietic bursts were between 83% and 88%. The Gr:A gamma ratios were similar to the synthesis ratio (0.71) of fetal liver BFU-E (12-wk gestation). Furthermore, the G gamma values in the bursts of the patients were in agreement with the G gamma:A gamma ratios in their circulating red blood cells (0.70, 0.71 and 0.67). These results suggest that the erythropoiesis associated with high levels of Hb F in trisomy 13 syndrome is controlled by erythropoietic precursor cells, in which normal switchings of G gamma:A gamma ratio and from Hb F to Hb A have not occurred.
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