Abstract:β-thalassemia and sickle cell anemia (SCA) are hemoglobin diseases caused by abnormal β-globin, occurring in South-east Asia, South Asia, North Africa and the Mediterranean region with different degrees. Elevated fetal hemoglobin (HbF) levels can significantly improve their clinical symptoms. B-cell lymphoma/leukemia 11A (BCL11A), a zinc finger structure transcription factor, plays an important negative regulatory role in fetal-to-adult hemoglobin expression. Down-regulated BCL11A activates γ-globin, so as to increase fetal hemoglobin expression, alleviating clinical symptoms of β-thalassemia and SCA. This article focuses on the mechanism of BCL11A on γ-globin, and the treatment of β-thalassemia and SCA based on BCL11A, thereby providing a theoretical basis for the study of β-globin disease.
