Down-regulation of the transcriptional repressor ZNF802 (JAZF1) reactivates fetal hemoglobin in β<sup>0</sup>-thalassemia/HbE
Reactivating of fetal hemoglobin (HbF; α2γ2) can ameliorate the severity of β-thalassemia disease by compensating for adult hemoglobin deficiency in patients. Previously, microarray analysis revealed that zinc finger protein (ZNF)802 (also known as Juxta-posed with another zinc finger gene-1 (JAZF1)...
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th-mahidol.864362023-06-19T01:04:44Z Down-regulation of the transcriptional repressor ZNF802 (JAZF1) reactivates fetal hemoglobin in β<sup>0</sup>-thalassemia/HbE Wongborisuth C. Mahidol University Multidisciplinary Reactivating of fetal hemoglobin (HbF; α2γ2) can ameliorate the severity of β-thalassemia disease by compensating for adult hemoglobin deficiency in patients. Previously, microarray analysis revealed that zinc finger protein (ZNF)802 (also known as Juxta-posed with another zinc finger gene-1 (JAZF1)) was upregulated in human erythroblasts derived from adult peripheral blood compared with fetal liver-derived cells, implying a potential role as a HbF repressor. However, deficiency in ZNF802 induced by lentiviral shRNA in β0-thalassemia/hemoglobinE erythroblasts had no effect on erythroblast proliferation and differentiation. Remarkably, the induction of HBG expression was observed at the transcriptional and translational levels resulting in an increase of HbF to 35.0 ± 3.5%. Interestingly, the embryonic globin transcripts were also upregulated but the translation of embryonic globin was not detected. These results suggest ZNF802 might be a transcriptional repressor of the γ-globin gene in adult erythroid cells. 2023-06-18T18:04:44Z 2023-06-18T18:04:44Z 2022-12-01 Article Scientific Reports Vol.12 No.1 (2022) 10.1038/s41598-022-08920-8 20452322 35322124 2-s2.0-85126892520 https://repository.li.mahidol.ac.th/handle/123456789/86436 SCOPUS |
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Multidisciplinary Wongborisuth C. Down-regulation of the transcriptional repressor ZNF802 (JAZF1) reactivates fetal hemoglobin in β<sup>0</sup>-thalassemia/HbE |
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Reactivating of fetal hemoglobin (HbF; α2γ2) can ameliorate the severity of β-thalassemia disease by compensating for adult hemoglobin deficiency in patients. Previously, microarray analysis revealed that zinc finger protein (ZNF)802 (also known as Juxta-posed with another zinc finger gene-1 (JAZF1)) was upregulated in human erythroblasts derived from adult peripheral blood compared with fetal liver-derived cells, implying a potential role as a HbF repressor. However, deficiency in ZNF802 induced by lentiviral shRNA in β0-thalassemia/hemoglobinE erythroblasts had no effect on erythroblast proliferation and differentiation. Remarkably, the induction of HBG expression was observed at the transcriptional and translational levels resulting in an increase of HbF to 35.0 ± 3.5%. Interestingly, the embryonic globin transcripts were also upregulated but the translation of embryonic globin was not detected. These results suggest ZNF802 might be a transcriptional repressor of the γ-globin gene in adult erythroid cells. |
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Down-regulation of the transcriptional repressor ZNF802 (JAZF1) reactivates fetal hemoglobin in β<sup>0</sup>-thalassemia/HbE |
title_short |
Down-regulation of the transcriptional repressor ZNF802 (JAZF1) reactivates fetal hemoglobin in β<sup>0</sup>-thalassemia/HbE |
title_full |
Down-regulation of the transcriptional repressor ZNF802 (JAZF1) reactivates fetal hemoglobin in β<sup>0</sup>-thalassemia/HbE |
title_fullStr |
Down-regulation of the transcriptional repressor ZNF802 (JAZF1) reactivates fetal hemoglobin in β<sup>0</sup>-thalassemia/HbE |
title_full_unstemmed |
Down-regulation of the transcriptional repressor ZNF802 (JAZF1) reactivates fetal hemoglobin in β<sup>0</sup>-thalassemia/HbE |
title_sort |
down-regulation of the transcriptional repressor znf802 (jazf1) reactivates fetal hemoglobin in β<sup>0</sup>-thalassemia/hbe |
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2023 |
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https://repository.li.mahidol.ac.th/handle/123456789/86436 |
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1781416600541331456 |