Abstract
The role of HbF inducers in ameliorating the pathophysiology and severity associated with hemoglobinopathies like sickle cell disease and thalassemia is well demonstrated. So many studies have focused on explain the pathways that involved in the re-activation of γ-globin gene expression. The HbF inducers are mainly includes chemotherapeutic agents (e.g. 5-Azacytidine and hydroxyurea), short-chain fatty acid derivatives (e.g. butyrates, valporate, sodium phenylbutyrate) and other HbF inducers like stem cell factor and synthetic gene-targeted transcription factors. These HbF inducers have significant effects on improve clinical manifestations of hemoglobinopathies and decreasing transfusion dependency. But regarding some limitations of these agents, more studies for the investigation of new agents with more safety and effectiveness is needed.
Highlights
The role of HbF inducers in ameliorating the pathophysiology and lassemia this proportion may increased
The embryonic Hb m switch occurs at approximately 6 to 8 weeks which switch from embryo onic Hbs to fetal Hb (Hb F (a2 γ2)) and the fetal switch which c occurs in the prenatal period to switch from fetal Hb to adult Hb (Hb - A(a2 b2)). [1,2] In the fetal life, HbF is the predominant Hb but it’s Non Correspondence: Mehran Karimi in order to decreased the clinical manifestations and severity of these hemoglobinopathies
HbF is composed of two types of γ chains that differ in their number 136 amino acid including: glycine or alanine (Gγ and Aγ) which are the products of separate globin gene loci
Summary
The HbF inducers are mainly includes chemothera- of γ-globin gene expression and increased the HbF level peutic agents (e.g. 5-Azacytidine and hydroxyurea), short-chain fatty acid derivatives (e.g. butyrates, valporate, sodium phenylbutyrate) and ly other HbF inducers like stem cell factor and synthetic gene-targeted transcription factors These HbF inducers have significant effects on n improve clinical manifestations of hemoglobinopathies and decreaso ing transfusion dependency. Finding the genetic variants associated with variations in HbF levels were done by genome-wide association (GWA) studies [7] These studies have founded polymorphisms in 3 loci (quantitative trait loci: QTLs) that account as a major heritable variation (~50%) in HbF expression in adults which includes: the b-globin locus on chromosome 11, HBS1L-MYB intergenic region on chromosome 6 and the gene encoding BCL11A on chromosome 2 [7,8,9,10,11]. Parts of this work were presented at the “3rd Pan-European Conference on Haemoglobinopathies and Rare Anaemias”, Limassol (Cyprus), 24-26 October 2012
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