Please use this identifier to cite or link to this item: https://www.um.edu.mt/library/oar/handle/123456789/28895
Title: Adult and fetal hemoglobin production in erythroid colonies from subjects with β-thalassemia or with hereditary persistence of fetal hemoglobin (HPFH)
Authors: Huisman, Titus Hendrik Jan
Reese, A. L.
Gravely, Marsha E.
Wilson, Jerry B.
Webber, Brooke B.
Felice, Alex E.
Keywords: Genetic disorders
Fetal hemoglobin
Erythroid cells
Issue Date: 1980
Publisher: Taylor & Francis
Citation: Huisman, T. H. J., Reese, A. L., Gravely, M. E., Wilson, J. B., Webber, B., & Felice, A. E. (1980). Adult and fetal hemoglobin production in erythroid colonies from subjects with β-thalassemia or with hereditary persistence of fetal hemoglobin (HPFH). Hemoglobin, 4(3-4), 449-467.
Abstract: The synthesis of α and non-α chains (β, δ, Gγ and Aγ) was studied in cultures of peripheral blood mononuclear cells from eleven β-thalassemia heterozygotes, two HPFH heterozygotes, and one HPFH homozygote. The synthesis of Hb F in the thalassemia colonies (average value: 12.6%) was comparable to that in normal adult colonies (average value: 12.2%). The percent Gγ chain in the Hb F varied greatly but a relationship between the Gγ chain percentage in the Hb F from colonies and that from peripheral blood was established. The relative synthesis of Hb A2 in colonies of β-thalassemia heterozygotes (average value: 5.8%) was 1.6 times as much as that in colonies of normal adults (average value: 3.6%). Hb A2 and Hb A were absent in the colonies of the HPFH homozygote. The α/non-α (i.e., β, γ, and δ) ratio of the hemoglobins in the cultured cells of the β-thalassemia heterozygotes and the α/β and α/δ ratios of isolated Hb A and Hb A2 were about one (range 0.74 to 1.38). The α/γ ratio of the Hb F synthesized in BFUe-derived colonies of the HPFH homozygote, however, was 1.5. The results suggest a deficiency in the in vitro culture system resulting in decreased levels of α-mRNA or in a partial inhibition of initiation of protein synthesis which is known to reduce the synthesis of α chains more than that of the β chains.
URI: https://www.um.edu.mt/library/oar//handle/123456789/28895
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