| Adaptation to anemia in hemoglobin E-ß thalassemia. | |
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MedLine Citation:
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PMID: 20833979 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Hemoglobin E β thalassemia is the commonest form of severe thalassemia in many Asian countries. Its remarkably variable clinical phenotype presents a major challenge to determining its most appropriate management. In particular, it is not clear why some patients with this condition can develop and function well at very low hemoglobin levels. Here, we demonstrate that patients with hemoglobin Eβ thalassemia have a significant decrease in the oxygen affinity of their hemoglobin, that is an increased P(50) value, in response to anemia. This may in part reflect the lower level of hemoglobin F in this condition compared with other forms of β thalassemia intermedia. The ability to right-shift the oxygen dissociation curve was retained across the spectrum of mild and severe phenotypes, despite the significantly higher levels of hemoglobin F in the former, suggesting that efforts directed at producing a modest increase in the level of hemoglobin F in symptomatic patients with this disease should be of therapeutic value. |
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Authors:
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Angela Allen; Christopher Fisher; Anuja Premawardhena; Timothy Peto; Stephen Allen; Mahinda Arambepola; Vivekanandan Thayalsutha; Nancy Olivieri; David Weatherall |
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Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't Date: 2010-09-10 |
Journal Detail:
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Title: Blood Volume: 116 ISSN: 1528-0020 ISO Abbreviation: Blood Publication Date: 2010 Dec |
Date Detail:
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Created Date: 2010-12-14 Completed Date: 2011-01-06 Revised Date: 2012-02-01 |
Medline Journal Info:
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Nlm Unique ID: 7603509 Medline TA: Blood Country: United States |
Other Details:
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Languages: eng Pagination: 5368-70 Citation Subset: AIM; IM |
Affiliation:
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Weatherall Institute of Molecular Medicine, University of Oxford, John Radcliffe Hospital, Oxford, UK. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Adaptation, Physiological Anemia / blood* Biological Transport Fetal Hemoglobin / analysis Hemoglobin E / analysis, metabolism* Humans Oxygen / metabolism* Protein Binding beta-Thalassemia / blood*, complications* |
| Grant Support | |
ID/Acronym/Agency:
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//Wellcome Trust |
| Chemical | |
Reg. No./Substance:
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7782-44-7/Oxygen; 9034-61-1/Hemoglobin E; 9034-63-3/Fetal Hemoglobin |
| Comments/Corrections | |
Erratum In:
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Blood. 2011 Dec 22;118(26):6994-5 |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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