Down-regulation of the transcriptional repressor ZNF802 (JAZF1) reactivates fetal hemoglobin in β<sup>0</sup>-thalassemia/HbE
Issued Date
2022-12-01
Resource Type
eISSN
20452322
Scopus ID
2-s2.0-85126892520
Pubmed ID
35322124
Journal Title
Scientific Reports
Volume
12
Issue
1
Rights Holder(s)
SCOPUS
Bibliographic Citation
Scientific Reports Vol.12 No.1 (2022)
Suggested Citation
Wongborisuth C., Chumchuen S., Sripichai O., Anurathaphan U., Sathirapongsasuti N., Songdej D., Tangprasittipap A., Hongeng S. Down-regulation of the transcriptional repressor ZNF802 (JAZF1) reactivates fetal hemoglobin in β<sup>0</sup>-thalassemia/HbE. Scientific Reports Vol.12 No.1 (2022). doi:10.1038/s41598-022-08920-8 Retrieved from: https://repository.li.mahidol.ac.th/handle/20.500.14594/86436
Title
Down-regulation of the transcriptional repressor ZNF802 (JAZF1) reactivates fetal hemoglobin in β<sup>0</sup>-thalassemia/HbE
Other Contributor(s)
Abstract
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.
