Department of Regulatory Medicine for Thrombosis, Nara Medical University, 840 Shijo-cho, Kashihara Nara, 634-8521, Japan.
Int J Hematol. 2012 Feb;95(2):119-24. doi: 10.1007/s12185-012-1015-z.
As shown by the results of both pre-clinical and clinical studies reported in past decades, the goal of establishing an effective and successful gene therapy for hemophilia A remains feasible and realistic. However, at this time, no single approach has been shown to be clearly superior, and a number of recurring challenges remain to be overcome. Given the persistent problems presented by the host immune response to systemic in vivo gene delivery, and the additional obstacles of inadequate transgene delivery and expression, we propose a re-evaluation of an ex vivo gene transfer approach that utilizes a genetically modified stem cell population. In this strategy, autologous blood outgrowth endothelial progenitor cells are obtained from hemophilic animals, into which a normal copy of the factor VIII gene is introduced via an engineered virus. Cell numbers are expanded in culture prior to their re-implantation under the skin of the hemophilic animals in an artificially developed supporting environment. Follow-up assessment of the treatment involves the general evaluation of clotting activity, the specific measurement of factor VIII levels in the blood, and clinical observation.
过去几十年的临床前和临床研究结果表明,为血友病 A 建立一种有效且成功的基因治疗仍然是可行和现实的。然而,目前还没有一种方法被证明具有明显的优势,许多反复出现的挑战仍然需要克服。鉴于宿主对全身体内基因传递的免疫反应持续存在问题,以及转基因传递和表达不足的额外障碍,我们提出重新评估一种利用基因修饰干细胞群的体外基因转移方法。在这种策略中,从血友病动物中获得自体血液生长的内皮祖细胞,通过工程病毒将正常的因子 VIII 基因引入其中。在重新植入血友病动物的皮肤下之前,在人工开发的支持环境中对细胞数量进行培养扩增。治疗后的随访评估包括凝血活性的一般评估、血液中因子 VIII 水平的特异性测量以及临床观察。