title: Thalidomide is more efficient than sodium butyrate in enhancing GATA-1 and EKLF gene expression in erythroid progenitors derived from HSCs with β-globin gene mutation.
abstract: BACKGROUND
  Efficient induction of fetal hemoglobin (HbF) is considered as an effective therapeutic approach in beta thalassemia. HbF inducer agents can induce the expression of γ-globin gene and produce high levels of HbF via different epigenetic and molecular mechanisms. Thalidomide and sodium butyrate are known as HbF inducer drugs.
  
  
  METHODS
  CD133(+) stem cells were isolated from umbilical cord blood of a newborn with minor β-thalassemia in order to evaluate the effects of these two drugs on the in vitro expression of GATA-1 and EKLF genes as erythroid transcription factors. CD133(+) stem cells were expanded and differentiated into erythroid lineage and then treated with thalidomide and sodium butyrate and finally analyzed by quantitative real-time PCR. Statistical analysis was performed using student's t-test by SPSS software.
  
  
  RESULTS
  Thalidomide and sodium butyrate increased GATA-1 and EKLF gene expression, compared to the non-treated control (P<0.05).
  
  
  CONCLUSIONS
  Thalidomide was more efficient than sodium butyrate in augmenting expression of GATA-1 and EKLF genes. It seems that GATA-1 and EKLF have crucial roles in the efficient induction of HbF by thalidomide.
authors.affiliation: Health Research Institute, Research Center of Thalassemia and Hemoglobinopathy, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran.
authors.forename: Mohammad Ali
authors.initials: MA
authors.lastname: Jalali Far
authors.affiliation: Sarem Cell Research Center-SCRC, Sarem Women's Hospital, Tehran, Iran.
authors.forename: Ali
authors.initials: A
authors.lastname: Dehghani Fard
authors.affiliation: Diagnostic Laboratory Sciences and Technology Research Center, School of Paramedical Sciences, Shiraz University of Medical Sciences, Shiraz, Iran.
authors.forename: Saiedeh
authors.initials: S
authors.lastname: Hajizamani
authors.affiliation: Department of Hematology, Faculty of Medical Sciences, Tarbiat Modares University, Tehran, Iran.
authors.forename: Majid
authors.initials: M
authors.lastname: Mossahebi-Mohammadi
authors.affiliation: Department of Medical Laboratory Sciences, School of Paramedicine, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran.
authors.forename: Hamid
authors.initials: H
authors.lastname: Yaghooti
authors.affiliation: Health Research Institute, Research Center of Thalassemia and Hemoglobinopathy, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran.
authors.forename: Najmaldin
authors.initials: N
authors.lastname: Saki
keywords: Fetal hemoglobin; Sodium butyrate; Thalidomide; β-thalassemia