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Hemoglobin Hasharon [α2 47 (CD5) Asp→ His β2]Linked to α-Thalassemia in Northern Italian Carriers

Hemoglobin Hasharon [α2 47 (CD5) Asp→ His β2]Linked to α-Thalassemia in Northern Italian Carriers This report is concerned with the evaluation of hematological parameters and of both relative (%) and absolute (mean pg/cell) quantities of the abnormal Hemoglobin (Hb) Hasharon in 53 heterozygous carriers and 7 double heterozygotes for Hb Hasharon and β-Thalassemia from 43 apparently unrelated families living in the province of Rovigo (northern Italy). Biosynthetic studies are also reported. The data strongly suggest the presence of an <sub>α</sub>-thalassemia-2 determinant closely linked to the α<sup>Hasharon</sup>-chain locus. Selective advantage of heterozygotes carrying such α-haplotype would explain the relatively high frequency of Hb Hasharon (0.23%) in northeastern Italy, a past-endemic malaria region. The interaction between Hb Hasharon and β-Thalassemia results in preferential decrease of the abnormal Hb level. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Acta Haematologica Karger

Hemoglobin Hasharon [α2 47 (CD5) Asp→ His β2]Linked to α-Thalassemia in Northern Italian Carriers

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Publisher
Karger
Copyright
© 1980 S. Karger AG, Basel
ISSN
0001-5792
eISSN
1421-9662
DOI
10.1159/000207426
Publisher site
See Article on Publisher Site

Abstract

This report is concerned with the evaluation of hematological parameters and of both relative (%) and absolute (mean pg/cell) quantities of the abnormal Hemoglobin (Hb) Hasharon in 53 heterozygous carriers and 7 double heterozygotes for Hb Hasharon and β-Thalassemia from 43 apparently unrelated families living in the province of Rovigo (northern Italy). Biosynthetic studies are also reported. The data strongly suggest the presence of an <sub>α</sub>-thalassemia-2 determinant closely linked to the α<sup>Hasharon</sup>-chain locus. Selective advantage of heterozygotes carrying such α-haplotype would explain the relatively high frequency of Hb Hasharon (0.23%) in northeastern Italy, a past-endemic malaria region. The interaction between Hb Hasharon and β-Thalassemia results in preferential decrease of the abnormal Hb level.

Journal

Acta HaematologicaKarger

Published: Jan 1, 1980

Keywords: α -Chain genes; α -Thalassemia; β-Thalassemia; Hb Hasharon

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