Watson Deborah J, Longhi Luca, Lee Edward B, Fulp Carl T, Fujimoto Scott, Royo Nicolas C, Passini Marco A, Trojanowski John Q, Lee Virginia M Y, McIntosh Tracy K, Wolfe John H
Department of Pathobiology, Center for Comparative Medical Genetics, School of Veterinary Medicine, University of Pennsylvania, Philadelphia, USA.
J Neuropathol Exp Neurol. 2003 Apr;62(4):368-80. doi: 10.1093/jnen/62.4.368.
Human Ntera-2 (NT2) cells can be differentiated in vitro into well-characterized populations of NT2N neurons that engraft and mature when transplanted into the adult CNS of rodents and humans. They have shown promise as treatments for neurologic disease, trauma, and ischemic stroke. Although these features suggest that NT2N neurons would be an excellent platform for ex vivo gene therapy in the CNS, stable gene expression has been surprisingly difficult to achieve in these cells. In this report we demonstrate stable, efficient, and nontoxic gene transfer into undifferentiated NT2 cells using a pseudotyped lentiviral vector encoding the human elongation factor 1-alpha promoter and the reporter gene eGFP. Expression of eGFP was maintained when the NT2 cells were differentiated into NT2N neurons after treatment with retinoic acid. When transplanted into the striatum of adult nude mice, transduced NT2N neurons survived, engrafted, and continued to express the reporter gene for long-term time points in vivo. Furthermore, transplantation of NT2N neurons genetically modified to express nerve growth factor significantly attenuated cognitive dysfunction following traumatic brain injury in mice. These results demonstrate that defined populations of genetically modified human NT2N neurons are a practical and effective platform for stable ex vivo gene delivery into the CNS.
人Ntera-2(NT2)细胞在体外可分化为特征明确的NT2N神经元群体,将其移植到啮齿动物和人类的成年中枢神经系统中时能够植入并成熟。它们已显示出有望用于治疗神经疾病、创伤和缺血性中风。尽管这些特性表明NT2N神经元将是中枢神经系统离体基因治疗的理想平台,但在这些细胞中实现稳定的基因表达却出人意料地困难。在本报告中,我们使用编码人延伸因子1-α启动子和报告基因eGFP的假型慢病毒载体,证明了可将稳定、高效且无毒的基因导入未分化的NT2细胞。在用视黄酸处理后,当NT2细胞分化为NT2N神经元时,eGFP的表达得以维持。当被移植到成年裸鼠的纹状体中时,转导的NT2N神经元存活、植入并在体内长期持续表达报告基因。此外,移植经基因改造以表达神经生长因子的NT2N神经元可显著减轻小鼠创伤性脑损伤后的认知功能障碍。这些结果表明,经过基因改造的特定人群的人NT2N神经元是将稳定的离体基因递送至中枢神经系统的实用且有效的平台。