Down-regulation of the transcriptional repressor ZNF802 (JAZF1) reactivates fetal hemoglobin in β⁰-thalassemia/HbE.

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%. Inter

SUBMITTER: Wongborisuth C 

PROVIDER: S-EPMC8943019 | biostudies-literature | 2022 Mar

REPOSITORIES: biostudies-literature