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Please use this identifier to cite or link to this item: https://www.um.edu.mt/library/oar/handle/123456789/82207
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dc.contributor.authorHuisman, Titus Hendrik Jan-
dc.contributor.authorGravely, Marsha E.-
dc.contributor.authorHenson, J.-
dc.contributor.authorFelice, Alex-
dc.contributor.authorWilson, J. B.-
dc.contributor.authorAbraham, Edathara C.-
dc.contributor.authorVella, Frank-
dc.contributor.authorLittle, M. W.-
dc.date.accessioned2021-10-14T09:09:29Z-
dc.date.available2021-10-14T09:09:29Z-
dc.date.issued1978-
dc.identifier.citationHuisman, T. H. J., Gravely, M. E., Henson, J., Felice, A., Wilson, J. B., Abraham, E. C., Vella, F., & Little, M. W. (1978). Variability in the interaction of β-thalassemia with the α-chain variants Hb G-Philadelphia and Hb Rampa. Translational Research, 92(2), 311-320.en_GB
dc.identifier.urihttps://www.um.edu.mt/library/oar/handle/123456789/82207-
dc.description.abstractTwo unrelated families are reported in which a β-thalassemia trait occurred with a heterozygosity for Hb G-Philadelphia (α2 68(E17)Asn → Lys β2) in one family and with Hb Rampa (α2 95(G2)Pro → Serβ2) in the other. The percentage of Hb G-Philadelphia was not influenced by the simultaneous presence of a β-thalassemia determinant, but that of Hb Rampa was decreased from 20% in the simple heterozygote to about 6% in persons with the Hb Rampa-β-thalassemia combination. Data from in vitro recombination experiments with isolated αX, αA, and βA chains, with heme attached, indicated a preferential formation of Hb A over Hb Rampa but not over Hb G-Philadelphia in conditions of relative β-chain deficiency. This suggests that the rate of assembly of monomers to form dimers or tetramers can be an important mechanism of controlling the quantity of certain hemoglobin variants with critical substitutions in heterozygotes.en_GB
dc.language.isoenen_GB
dc.publisherElsevier Inc.en_GB
dc.rightsinfo:eu-repo/semantics/openAccessen_GB
dc.subjectEthylenediaminetetraacetic aciden_GB
dc.subjectDiethylanilineen_GB
dc.subjectThalassemen_GB
dc.subjectHemoglobinopathyen_GB
dc.titleVariability in the interaction of β-thalassemia with the α-chain variants Hb G-Philadelphia and Hb Rampaen_GB
dc.typearticleen_GB
dc.rights.holderThe 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.reviewedpeer-revieweden_GB
dc.identifier.doi10.5555/uri:pii:0022214378900604-
dc.publication.titleTranslational Researchen_GB
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