Premium
Regulation of the fetal hemoglobin silencing factor BCL11A
Author(s) -
Basak Anindita,
Sankaran Vijay G.
Publication year - 2016
Publication title -
annals of the new york academy of sciences
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.712
H-Index - 248
eISSN - 1749-6632
pISSN - 0077-8923
DOI - 10.1111/nyas.13024
Subject(s) - fetal hemoglobin , gene silencing , transcription factor , hemoglobin , regulator , biology , fetus , globin , bioinformatics , genetics , gene , biochemistry , pregnancy
The clinical severity of sickle cell disease and β‐thalassemia, the major disorders of β‐globin, can be ameliorated by increased production of fetal hemoglobin (HbF). Here, we provide a brief overview of the fetal‐to‐adult hemoglobin switch that occurs in humans shortly after birth and review our current understanding of one of the most potent known regulators of this switching process, the multiple zinc finger–containing transcription factor BCL11A. Originally identified in genome‐wide association studies, multiple orthogonal lines of evidence have validated BCL11A as a key regulator of hemoglobin switching and as a promising therapeutic target for HbF induction. We discuss recent studies that have highlighted its importance in silencing the HbF‐encoding genes and discuss opportunities that exist to further understand the regulation of BCL11A and its mechanism of action, which could provide new insight into opportunities to induce HbF for therapeutic purposes.