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Author Luo, Jun ♦ Liu, Rongrong ♦ Zhao, Weihua ♦ Liu, Zhenfang ♦ Li, Jing ♦ Lai, Yongrong ♦ Luo, Lin
Source Directory of Open Access Journals (DOAJ)
Content type Text
Publisher Hindawi Limited
File Format HTM / HTML
Date Created 2017-12-11
Copyright Year ©2017
Language English
Subject Domain (in LCC) R
Subject Keyword Medicine
Abstract Background. Fetal hemoglobin (HbF; α2γ2) is a potent genetic modifier of the severity of β-thalassemia and sickle cell anemia. Differences in the levels of HbF that persist into adulthood affect the severity of sickle cell disease and the β-thalassemia syndromes. Sry type HMG box (SOX6) is a potent silencer of HbF. Here, we reactivated γ-globin expression by downregulating SOX6 to alleviate anemia in the β-thalassemia patients. Methods. 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 β-thalassemia major MNC. The expression of γ-globin was analyzed by qPCR (quantitative real-time PCR) and WB (western blot). Results. Our data showed that downregulation of SOX6 induces γ-globin production in K562 cell line and human erythrocytes from normal donors and β-thalassemia major donors, without altering erythroid maturation. Conclusions. This is the first report on γ-globin induction by downregulation of SOX6 in human erythroblasts derived from β-thalassemia major.
ISSN 23146133
Age Range 18 to 22 years ♦ above 22 year
Educational Use Research
Education Level UG and PG ♦ Career/Technical Study
Learning Resource Type Article
Publisher Date 2017-01-01
e-ISSN 23146133
Journal BioMed Research International
Volume Number 2017


Source: Directory of Open Access Journals (DOAJ)