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[...] Blood Rev [Internet] [cited 2023 Jan 9];8(4):213-24. Available from: https://pubmed.ncbi.nlm.nih.gov/7534152/ 6. Tang DC, Ebb Hardison RC, Rodgers GP. Restoration of the CCAAT box or insertion of the CACCC motif activates [corrected] delta-globin gene expression. Blood [Internet] [cited 2023 Jan 9];90(1):421-7. Available from: https://pubmed.ncbi.nlm.nih.gov/9207479/ 7. Zhu Chin Aerbajinai Trainor Gao Rodgers GP. Recombinant erythroid Kruppel-like factor fused to GATA1 up-regulates delta- and gamma-globin expression in erythroid cells. Blood [Internet] [cited 2023 Jan 9];117(11):3045-52. Available from: http://dx.doi.org/10.1182/blood-2010-07-294751 8. Zhu Li Aerbajinai Kumkhaek Pirooznia Saxena et al. [...]
[...] globin and KLF1-GATA1 fusion protein could increase ? globin expression (Zhu et al. 2011). In recent study, they demonstrate the effect of the KLF1-GATA1 fusion protein on HbA2 expression and correction of the SCD phenotype in vivo and in vitro (Zhu et al. 2022). They found a significant increase in ? globin expression and reduction of hypoxia-related sickling in SCD human and mouse erythrocytes due to forced expression of medium and long forms of the KLF1-GATA1 fusion protein (Zhu et al. 2022). [...]
[...] Furthermore, transduction by the KLF1- GATA1 fusion protein had no effect on human sickle CD34+ erythroid cells' differentiation and proliferation (Zhu et al., 2022). Study results suggest that KLF1-GATA1 fusion proteins could be useful as a genetic therapeutic tool for SCD and ?-thalassemia (Zhu et al., 2022). References Weatherall, D. J., & Clegg, J. B. (2001). Inherited haemoglobin disorders: an increasing global health problem. Bulletin of the World Health Organization, 704-712. https://pubmed.ncbi.nlm.nih.gov/11545326/ Rees, D. C., Williams, T. N., & Gladwin, M. T. [...]
[...] Deoxygenation of HbS leads to polymerization, which causes erythrocyte deformation and blood hemolysis, resulting in severe clinical complications (Platt, 2008). Hemoglobin A2 (HbA2, and HbF are both found in adult blood at low levels (Steinberg & Adams, 1991) (Rochette et al., 1994). Furthermore, the absence of a KLF1-binding site within -globin results in low HbA2 expression (Tang et al., 1997). Previous study had shown that Interaction between ? globin and KLF1-GATA1 fusion protein could increase ? globin expression (Zhu et al., 2011). [...]
[...] Furthermore, transduction by the KLF1-GATA1 fusion protein had no effect on human sickle CD34+ erythroid cells' differentiation and proliferation (Zhu, Li, et al.). Study results suggest that KLF1-GATA1 fusion proteins could be useful as a genetic therapeutic tool for SCD and thalassemia (Zhu, Li, et al.). References Weatherall, D. J., and J. B. Clegg. "Inherited Haemoglobin Disorders: An Increasing Global Health Problem." Bulletin of the World Health Organization, vol no pp. 704-712, https://pubmed.ncbi.nlm.nih.gov/11545326/. Rees, David C., et al. "Sickle-Cell Disease." Lancet, vol no pp. 2018-2031, doi:10.1016/S0140-6736(10)61029-X. [...]
