Paola Izzo

By Michela Grosso, Raffaele Sessa, Stella Puzone, Maria Rosaria Storino and Paola Izzo

Part of the book: Anemia

By Francesca Duraturo, Raffaella Liccardo, Angela Cavallo, Marina De Rosa and Paola Izzo

Part of the book: Carcinogenesis

By Marianna De Martino, Raffaele Sessa, Maria Rosaria Storino, Mariarosaria Giuliano, Silvia Trombetti, Rosa Catapano, Alessandra Lo Bianco, Paola Izzo and Michela Grosso

During development the human β-globin gene cluster undergoes two switching processes at the embryo-fetal and fetal-adult stages, respectively, involving changes in chromatin remodeling and in transcriptional regulatory networks. In particular, during the perinatal period, the switch from fetal-to-adult globin gene expression leads to fetal globin genes silencing and progressive decline of fetal hemoglobin (HbF). Impaired hemoglobin switching is associated with hereditary persistence of HbF (HPFH), a condition in which the fetal globin genes fail to be completely silenced in adult red blood cells. This condition, when co-inherited with hemoglobinopathies, has great therapeutic potential because elevated HbF levels can ameliorate β-thalassemia and sickle cell anemia. Therefore, there is a growing interest about the complex network of factors that regulate fetal globin genes expression. Here we discuss the activity of transcriptional repressors of fetal globin genes and their potential role as novel therapeutic targets in β-thalassemia.

Part of the book: Beta Thalassemia