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Indexed/Abstracted in: EMBASE, Science Citation Index Expanded (SciSearch), Scopus
Impact Factor 0,246
Online ISSN 1827-160X
TRENDS IN MOLECULAR DIAGNOSIS AND THERAPY OF β-THALASSEMIA AND SICKLE CELL ANEMIA
Department of Hematology, Hadassah University Hospital, Jerusalem, Israel
High fetal hemoglobin (HbF) has been shown to ameliorate the clinical symptoms of patients with β-hemoglobinopathies, β-thalassemia (β-thal) and sickle cell anemia (SCA). Research is therefore focus on finding drugs capable of reactivating the γ-globin genes and stimulating the production of HbF. Several in vitro experimental models have been developed to serve this purpose. Two models are the subject of this review: in vitro established erythroid-like cell lines and primary cultures of erythroid cells derived from progenitors obtained from normal donors and patients with β-thal and SCA. These experimental models are useful for screening of compounds and for studying their mechanism of action at the cellular and molecular levels. These studies are essential for finding, testing and developing new, effective and safe drugs.