KLF1 directly activates expression of the novel fetal globin repressor ZBTB7A/LRF in erythroid cells.

ABSTRACT: Genes encoding the human β-like hemoglobin proteins undergo a developmental switch from fetal γ-globin to adult β-globin expression around the time of birth. β-hemoglobinopathies, such as sickle-cell disease and β-thalassemia, result from mutations affecting the adult β-globin gene. The only treatment options currently available carry significant adverse effects. Analyses of heritable variations in fetal hemoglobin (HbF) levels have provided evidence that reactivation of the silenced fetal γ-globin genes in adult erythroid cells is a promising therapy. The γ-globin repressor BCL11A has become the major focus, with several studies investigating its regulation and function as a first step to inhibiting its expression or activity. However, a second repressio

SUBMITTER: Norton LJ 

PROVIDER: S-EPMC5727813 | biostudies-literature | 2017 Apr

REPOSITORIES: biostudies-literature