Induction of HBF

• Main Authors: Mehran Karimi, Nader Cohan
• Format: Article
• Language: English
• Published: PAGEPress Publications 2013-03-01
• Series: Thalassemia Reports
• Subjects: HbF inducer, hemoglobinopathy, γ-globin gene, re-activation.
• Online Access: http://www.pagepressjournals.org/index.php/thal/article/view/1507

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 &gamma;-globin gene expression. The HbF inducers are mainly includes chemotherapeutic agents (<em>e.g.</em> 5-Azacytidine and hydroxyurea), short-chain fatty acid derivatives (<em>e.g. </em>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.