Correlation of BACH1 and hemoglobin E/Beta-thalassemia globin expression
Objective: The diverse clinical phenotype of hemoglobin E (HbE)/β-thalassemia has not only confounded clinicians in matters of patient management but has also led scientists to investigate the complex mechanisms involved in maintaining the delicate red cell environment where, even with apparent simi...
| Main Authors: | , , , , , , |
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| Format: | Article |
| Language: | English |
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Turk Hematoloji Dernegi
2016
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| Online Access: | http://psasir.upm.edu.my/id/eprint/55392/ http://psasir.upm.edu.my/id/eprint/55392/1/Correlation%20of%20BACH1%20and%20hemoglobin.pdf |
| _version_ | 1848852790045048832 |
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| author | Tze, Yan Lee Muniandy, Logeswaran Lai, Kuan Teh Abdullah, Maha George, Elizabeth Sathar, Jameela Mei, I. Lai |
| author_facet | Tze, Yan Lee Muniandy, Logeswaran Lai, Kuan Teh Abdullah, Maha George, Elizabeth Sathar, Jameela Mei, I. Lai |
| author_sort | Tze, Yan Lee |
| building | UPM Institutional Repository |
| collection | Online Access |
| description | Objective: The diverse clinical phenotype of hemoglobin E (HbE)/β-thalassemia has not only confounded clinicians in matters of patient management but has also led scientists to investigate the complex mechanisms involved in maintaining the delicate red cell environment where, even with apparent similarities of α- and β-globin genotypes, the phenotype tells a different story. The BTB and CNC homology 1 (BACH1) protein is known to regulate α- and β-globin gene transcriptions during the terminal differentiation of erythroid cells. With the mutations involved in HbE/β-thalassemia disorder, we studied the role of BACH1 in compensating for the globin chain imbalance, albeit for fine-tuning purposes.
Materials and Methods: A total of 47 HbE/β-thalassemia samples were analyzed using real-time quantitative polymerase chain reaction and correlated with age, sex, red blood cell parameters, globin gene expressions, and some clinical data.
Results: The BACH1 expression among the β-thalassemia intermedia patients varied by up to 2-log differences and was positively correlated to age; α-, β-, and γ-globin gene expression level; and heme oxygenase 1 protein. BACH1 was also negatively correlated to reticulocyte level and had a significant correlation with splenectomy.
Conclusion: This study indicates that the expression of BACH1 could be elevated as a compensatory mechanism to decrease the globin chain imbalance as well as to reduce the oxidative stress found in HbE/β-thalassemia. |
| first_indexed | 2025-11-15T10:43:40Z |
| format | Article |
| id | upm-55392 |
| institution | Universiti Putra Malaysia |
| institution_category | Local University |
| language | English |
| last_indexed | 2025-11-15T10:43:40Z |
| publishDate | 2016 |
| publisher | Turk Hematoloji Dernegi |
| recordtype | eprints |
| repository_type | Digital Repository |
| spelling | upm-553922017-10-05T05:08:14Z http://psasir.upm.edu.my/id/eprint/55392/ Correlation of BACH1 and hemoglobin E/Beta-thalassemia globin expression Tze, Yan Lee Muniandy, Logeswaran Lai, Kuan Teh Abdullah, Maha George, Elizabeth Sathar, Jameela Mei, I. Lai Objective: The diverse clinical phenotype of hemoglobin E (HbE)/β-thalassemia has not only confounded clinicians in matters of patient management but has also led scientists to investigate the complex mechanisms involved in maintaining the delicate red cell environment where, even with apparent similarities of α- and β-globin genotypes, the phenotype tells a different story. The BTB and CNC homology 1 (BACH1) protein is known to regulate α- and β-globin gene transcriptions during the terminal differentiation of erythroid cells. With the mutations involved in HbE/β-thalassemia disorder, we studied the role of BACH1 in compensating for the globin chain imbalance, albeit for fine-tuning purposes. Materials and Methods: A total of 47 HbE/β-thalassemia samples were analyzed using real-time quantitative polymerase chain reaction and correlated with age, sex, red blood cell parameters, globin gene expressions, and some clinical data. Results: The BACH1 expression among the β-thalassemia intermedia patients varied by up to 2-log differences and was positively correlated to age; α-, β-, and γ-globin gene expression level; and heme oxygenase 1 protein. BACH1 was also negatively correlated to reticulocyte level and had a significant correlation with splenectomy. Conclusion: This study indicates that the expression of BACH1 could be elevated as a compensatory mechanism to decrease the globin chain imbalance as well as to reduce the oxidative stress found in HbE/β-thalassemia. Turk Hematoloji Dernegi 2016 Article PeerReviewed application/pdf en http://psasir.upm.edu.my/id/eprint/55392/1/Correlation%20of%20BACH1%20and%20hemoglobin.pdf Tze, Yan Lee and Muniandy, Logeswaran and Lai, Kuan Teh and Abdullah, Maha and George, Elizabeth and Sathar, Jameela and Mei, I. Lai (2016) Correlation of BACH1 and hemoglobin E/Beta-thalassemia globin expression. Turkish Journal of Hematology, 33 (1). pp. 15-20. ISSN 1300-7777; ESSN: 1308-5263 http://www.tjh.com.tr/ 10.4274/tjh.2014.0197 |
| spellingShingle | Tze, Yan Lee Muniandy, Logeswaran Lai, Kuan Teh Abdullah, Maha George, Elizabeth Sathar, Jameela Mei, I. Lai Correlation of BACH1 and hemoglobin E/Beta-thalassemia globin expression |
| title | Correlation of BACH1 and hemoglobin E/Beta-thalassemia globin expression |
| title_full | Correlation of BACH1 and hemoglobin E/Beta-thalassemia globin expression |
| title_fullStr | Correlation of BACH1 and hemoglobin E/Beta-thalassemia globin expression |
| title_full_unstemmed | Correlation of BACH1 and hemoglobin E/Beta-thalassemia globin expression |
| title_short | Correlation of BACH1 and hemoglobin E/Beta-thalassemia globin expression |
| title_sort | correlation of bach1 and hemoglobin e/beta-thalassemia globin expression |
| url | http://psasir.upm.edu.my/id/eprint/55392/ http://psasir.upm.edu.my/id/eprint/55392/ http://psasir.upm.edu.my/id/eprint/55392/ http://psasir.upm.edu.my/id/eprint/55392/1/Correlation%20of%20BACH1%20and%20hemoglobin.pdf |