Understanding the factors that regulate ZBTB7A expression has a direct impact on planning various therapeutic strategies to treat Sickle-cell anemia and β-thalassemia.
Laura J. Norton, Alister P.W. Funnell (2017)
KLF1 directly activates expression of the novel fetal globin repressor ZBTB7A/LRF in erythroid cells
Blood Advances 1:685-692
KLF1 directly activates expression of the novel fetal globin repressor ZBTB7A/LRF in erythroid cells
Blood Advances 1:685-692
(Translated by Wassal Alhammad)
Significance
Sickle-cell anemia and β-thalassemia are widespread genetic diseases that are caused by the disruption of adult β-globin genes. As discovered from the Fetal Hemoglobin (HbF) analysis, reactivating this gene lead to significant amelioration of these diseases. It has been known that BCL11A represses the expression of fetal hemoglobin through direct activity of Kruppel-like transcription factor 1 (KLF1). A second layer of fetal hemoglobin repression is achieved by the activity of transcriptional factor repressor called ZBTB7A.
Contents
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Fig. 1: Structure of Fetal Hemoglobin (HbF) protien: Fetal Hemoglobin is a protien composed of four polypeptide chain (ribbon-shaped, rainbow-colored). two alpha chains (top) and two gamma chains (bottom). Each chain contains a heme group in the center (red and gray dots). The heme group is responsible for carrying oxygen from to the tissue. Image taken from Fetal hemoglobin (Wikipedia) by Angel Herráez [doi:10.2210/pdb4mqj/pdb]#3. |