Author/Authors :
Farshdousti Hagh, Majid tabriz university of medical sciences - Hematology and Oncology Research Center, ايران , Dehghani Fard, Ali tarbiat modares university - Faculty of Medical Sciences - Department of Hematology and Blood Banking, تهران, ايران , Saki, Najmaldin ahvaz jundishapur university of medical sciences - Research Center of Thalassemia and Hemoglobinopathies, اهواز, ايران , Shahjahani, Mohammad tarbiat modares university - Faculty of Medical Sciences - Department of Hematology and Blood Banking, تهران, ايران , Kaviani, Saied tarbiat modares university - Faculty of Medical Sciences - Department of Hematology and Blood Banking, تهران, ايران
Abstract :
Hemoglobin F (HbF, α2γ2) is a major contributor to the clinical heterogeneity and ameliorating agent observed in patients with the ß-globin disorders including ß-thalassemia and sickle cell disease (SCD). During fetal life, HbF is the major hemoglobin but is largely substituted by adult hemoglobin (HbA, α2ß2) following a globin expression switch after birth. Increased γ-globin expression can reduce the clinical severity of ß-thalassemia and SCD. Therefore, increase in HbF production has served as a longstanding goal. The progression of target-based therapeutics has been confused by limited comprehension of molecular mechanisms of gamma-globin gene expression. However, recent discoveries of regulators of HbF level represent a major development and provide new opportunities in employing novel rational therapeutic strategies. In this review, molecular mechanisms of hemoglobin F induction will be discussed
Keywords :
Hemoglobin F , β-thalassemia , Expression , Induction
