Paper Information

Journal:   INTERNATIONAL JOURNAL OF HEMATOLOGY-ONCOLOGY AND STEM CELL RESEARCH (IJHOSCR)   JANUARY 2016 , Volume 10 , Number 1; Page(s) 37 To 41.
 
Paper: 

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

 
 
Author(s):  JALALI FAR MOHAMMAD ALI, DEHGHANI FARD ALI, HAJIZAMANI SAIEDEH, MOSSAHEBI MOHAMMADI MAJID, YAGHOOTI HAMID, SAKI NAJMALDIN*
 
* HEALTH RESEARCH INSTITUTE, RESEARCH CENTER OF THALASSEMIA AND HEMOGLOBINOPATHY, AHVAZ JUNDISHAPUR UNIVERSITY OF MEDICAL SCIENCES, AHVAZ, IRAN
 
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 ?-globin gene and produce high levels of HbF via different epigenetic and molecular mechanisms. Thalidomide and sodium butyrate are known as HbF inducer drugs. Material and methods: CD133+ stem cells were isolated from umbilical cord blood of a newborn with minor b-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). Conclusion: 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.

 
Keyword(s): FETAL HEMOGLOBIN, THALIDOMIDE, SODIUM BUTYRATE, β-THALASSEMIA
 
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