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SOX6 Downregulation Induces ?-Globin in Human ?-Thalassemia Major Erythroid Cells.

ABSTRACT: Fetal hemoglobin (HbF; ?2?2) is a potent genetic modifier of the severity of ?-thalassemia and sickle cell anemia. Differences in the levels of HbF that persist into adulthood affect the severity of sickle cell disease and the ?-thalassemia syndromes. Sry type HMG box (SOX6) is a potent silencer of HbF. Here, we reactivated ?-globin expression by downregulating SOX6 to alleviate anemia in the ?-thalassemia patients.SOX6 was downregulated by lentiviral RNAi (RNA interference) in K562 cell line and an in vitro culture model of human erythropoiesis in which erythroblasts are derived from the normal donor mononuclear cells (MNC) or ?-thalassemia major MNC. The expression of ?-globin was analyzed by qPCR (quantitative real-time PCR) and WB (western blot).Our data showed that downregulation of SOX6 induces ?-globin production in K562 cell line and human erythrocytes from normal donors and ?-thalassemia major donors, without altering erythroid maturation.This is the first report on ?-globin induction by downregulation of SOX6 in human erythroblasts derived from ?-thalassemia major.

SUBMITTER: Li J 

PROVIDER: S-EPMC5733236 | biostudies-literature | 2017

REPOSITORIES: biostudies-literature

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SOX6 Downregulation Induces <i>γ</i>-Globin in Human <i>β</i>-Thalassemia Major Erythroid Cells.

Li Jing J   Lai Yongrong Y   Luo Jun J   Luo Lin L   Liu Rongrong R   Liu Zhenfang Z   Zhao Weihua W  

BioMed research international 20171128

<h4>Background</h4>Fetal hemoglobin (HbF; <i>α</i><sub>2</sub><i>γ</i><sub>2</sub>) is a potent genetic modifier of the severity of <i>β</i>-thalassemia and sickle cell anemia. Differences in the levels of HbF that persist into adulthood affect the severity of sickle cell disease and the <i>β</i>-thalassemia syndromes. Sry type HMG box (SOX6) is a potent silencer of HbF. Here, we reactivated <i>γ</i>-globin expression by downregulating SOX6 to alleviate anemia in the <i>β</i>-thalassemia patient  ...[more]

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