The population genetics and dynamics of the thalassemias.

The inherited disorders of hemoglobin, including the thalassemias, are by far the commonest monogenic diseases. Although several factors are responsible for their very high frequency, the major mechanism seems to be natural selection mediated by heterozygote protection against severe forms of malari...

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Main Authors: Weatherall, D, Williams, T, Allen, S, O'Donnell, A
Format: Journal article
Language:English
Published: 2010
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author Weatherall, D
Williams, T
Allen, S
O'Donnell, A
author_facet Weatherall, D
Williams, T
Allen, S
O'Donnell, A
author_sort Weatherall, D
collection OXFORD
description The inherited disorders of hemoglobin, including the thalassemias, are by far the commonest monogenic diseases. Although several factors are responsible for their very high frequency, the major mechanism seems to be natural selection mediated by heterozygote protection against severe forms of malaria. Recent work has highlighted the complexity of the interplay among the different hemoglobin variants themselves and among different levels of malaria resistance, and is helping to explain the extraordinary heterogeneity in the distribution of the hemoglobin disorders even within short geographical distances. Some progress has also been made toward understanding the cellular and immune mechanisms that may underlie heterozygote protection against malaria in these conditions. In addition to providing valuable information about human evolutionary biology, work in this field has an increasingly important influence on the development of programs for the better management of the hemoglobin disorders, particularly in the poorer countries of the tropical world.
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spelling oxford-uuid:619b7639-e897-4692-a7a1-608690940cac2022-03-26T18:01:11ZThe population genetics and dynamics of the thalassemias.Journal articlehttp://purl.org/coar/resource_type/c_dcae04bcuuid:619b7639-e897-4692-a7a1-608690940cacEnglishSymplectic Elements at Oxford2010Weatherall, DWilliams, TAllen, SO'Donnell, AThe inherited disorders of hemoglobin, including the thalassemias, are by far the commonest monogenic diseases. Although several factors are responsible for their very high frequency, the major mechanism seems to be natural selection mediated by heterozygote protection against severe forms of malaria. Recent work has highlighted the complexity of the interplay among the different hemoglobin variants themselves and among different levels of malaria resistance, and is helping to explain the extraordinary heterogeneity in the distribution of the hemoglobin disorders even within short geographical distances. Some progress has also been made toward understanding the cellular and immune mechanisms that may underlie heterozygote protection against malaria in these conditions. In addition to providing valuable information about human evolutionary biology, work in this field has an increasingly important influence on the development of programs for the better management of the hemoglobin disorders, particularly in the poorer countries of the tropical world.
spellingShingle Weatherall, D
Williams, T
Allen, S
O'Donnell, A
The population genetics and dynamics of the thalassemias.
title The population genetics and dynamics of the thalassemias.
title_full The population genetics and dynamics of the thalassemias.
title_fullStr The population genetics and dynamics of the thalassemias.
title_full_unstemmed The population genetics and dynamics of the thalassemias.
title_short The population genetics and dynamics of the thalassemias.
title_sort population genetics and dynamics of the thalassemias
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