Publication: Implication of globin gene expression, hemoglobin F and hemoglobin E levels on β-thalassemia/Hb E disease severity
Issued Date
2014-01-01
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ISSN
15508080
00917370
00917370
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2-s2.0-84910008421
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Mahidol University
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SCOPUS
Bibliographic Citation
Annals of Clinical and Laboratory Science. Vol.44, No.4 (2014), 437-442
Suggested Citation
Suwimol Siriworadechkul, Sumalee Jindadamrongwech, Suporn Chuncharunee, Saranya Aupparakkitanon Implication of globin gene expression, hemoglobin F and hemoglobin E levels on β-thalassemia/Hb E disease severity. Annals of Clinical and Laboratory Science. Vol.44, No.4 (2014), 437-442. Retrieved from: https://repository.li.mahidol.ac.th/handle/20.500.14594/33385
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Title
Implication of globin gene expression, hemoglobin F and hemoglobin E levels on β-thalassemia/Hb E disease severity
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Abstract
© 2014 by the Association of Clinical Scientists, Inc. One of the factors affecting the degree of severity in β-thalassemia disease is the presence of unmatched a-hemoglobin chains. Thus, the expression levels of globin genes in reticulocytes of β-thalassemia subjects were measured using quantitative RT-PCR, demonstrating that a/β globin mRNA ratio, as well as levels of ?-globin mRNA and Hb F, increased with progressing degree of β globin synthesis defect. The levels of γ-globin mRNA and Hb F could not be directly correlated with severity of β-thalassemia/Hb E disease due to a low statistical power of this analysis. Higher levels of Hb E were present, however, in clinically mild patients, as compared to moderately severe β-thalassemia/Hb E subjects. This suggests that in β-thalassemia/Hb E disease, elevation of Hb E level through enhancing correctly spliced βE-globin mRNA offers another approach in ameliorating disease severity. In addition, co-inheritance of a-thalassemia 2 trait in β-thalassemia/Hb E subjects was associated with milder outcome compared with those with the same β-thalassemia genotypes, confirming the notion of the beneficial effect of a more balanced a:β-globin chain ratio.