Department of General Biology, Medical School, University of Patras, 26504 Patras, Greece.
Centre of Basic Research, Biomedical Research Foundation of the Academy of Athens (BRFAA), 11527 Athens, Greece.
Genes (Basel). 2023 Sep 8;14(9):1774. doi: 10.3390/genes14091774.
β-Thalassemia is a subgroup of inherited blood disorders associated with mild to severe anemia with few and limited conventional therapy options. Lately, lentiviral vector-based gene therapy has been successfully applied for disease treatment. However, the current development of non-viral episomal vectors (EV), non-integrating and non-coding for viral proteins, may be helpful in generating valid alternatives to viral vectors. We constructed a non-viral, episomal vector pEPβ-globin for the physiological β-globin gene based on two human chromosomal elements: the scaffold or matrix attachment region (S/MAR), allowing for long nuclear retention and non-integration and the β-globin replication initiation region (IR), allowing for enhancement of replication and establishment. After nucleofections into K562 cells with a transfection efficiency of 24.62 ± 7.7%, the vector induces stable transfection and is detected in long-term cultures as a non-integrating, circular episome expressing the β-globin gene efficiently. Transfections into CD34+ cells demonstrate an average efficiency of 15.57 ± 11.64%. In the colony-forming cell assay, fluorescent colonies are 92.21%, which is comparable to those transfected with vector pEP-IR at 92.68%. Additionally, fluorescent colonies produce β-globin mRNA at a physiologically 3-fold higher level than the corresponding non-transfected cells. Vector pEPβ-globin provides the basis for the development of therapeutic EV for gene therapy of β-thalassemias.
β-地中海贫血是一组遗传性血液疾病,与轻度至重度贫血有关,传统治疗选择有限。最近,基于慢病毒载体的基因治疗已成功应用于疾病治疗。然而,目前非病毒的游离体载体(EV)的发展,非整合和非病毒蛋白编码,可能有助于产生替代病毒载体的有效方法。我们构建了一种非病毒的、基于两个人类染色体元件的游离体载体 pEPβ-globin,用于生理性β-珠蛋白基因:支架或基质附着区(S/MAR),允许长核保留和非整合,以及β-珠蛋白复制起始区(IR),允许增强复制和建立。在转染效率为 24.62±7.7%的 K562 细胞中进行核转染后,该载体诱导稳定转染,并在长期培养中作为非整合的、环状游离体检测到,有效地表达β-珠蛋白基因。在 CD34+细胞中的转染显示平均效率为 15.57±11.64%。在集落形成细胞测定中,荧光集落为 92.21%,与转染载体 pEP-IR 的 92.68%相当。此外,荧光集落产生的β-珠蛋白 mRNA 水平比相应的未转染细胞高 3 倍。pEPβ-globin 载体为治疗性 EV 的开发提供了基础,用于β-地中海贫血的基因治疗。