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DC Field | Value | Language |
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dc.contributor.author | Huisman, Titus Hendrik Jan | - |
dc.contributor.author | Reese, A. L. | - |
dc.contributor.author | Gravely, Marsha E. | - |
dc.contributor.author | Wilson, Jerry B. | - |
dc.contributor.author | Webber, Brooke B. | - |
dc.contributor.author | Felice, Alex | - |
dc.date.accessioned | 2018-04-06T12:19:58Z | - |
dc.date.available | 2018-04-06T12:19:58Z | - |
dc.date.issued | 1980 | - |
dc.identifier.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. | en_GB |
dc.identifier.uri | https://www.um.edu.mt/library/oar//handle/123456789/28895 | - |
dc.description.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. | en_GB |
dc.language.iso | en | en_GB |
dc.publisher | Taylor & Francis | en_GB |
dc.rights | info:eu-repo/semantics/restrictedAccess | en_GB |
dc.subject | Genetic disorders | en_GB |
dc.subject | Fetal hemoglobin | en_GB |
dc.subject | Erythroid cells | en_GB |
dc.title | Adult and fetal hemoglobin production in erythroid colonies from subjects with β-thalassemia or with hereditary persistence of fetal hemoglobin (HPFH) | en_GB |
dc.type | article | en_GB |
dc.rights.holder | The copyright of this work belongs to the author(s)/publisher. The rights of this work are as defined by the appropriate Copyright Legislation or as modified by any successive legislation. Users may access this work and can make use of the information contained in accordance with the Copyright Legislation provided that the author must be properly acknowledged. Further distribution or reproduction in any format is prohibited without the prior permission of the copyright holder. | en_GB |
dc.description.reviewed | peer-reviewed | en_GB |
dc.identifier.doi | 10.3109/03630268008996226 | - |
dc.publication.title | Hemoglobin | en_GB |
Appears in Collections: | Scholarly Works - FacM&SPB |
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