University of South Florida, Tampa, FL 33606, United States of America.
Pediatric Pulmonology, Sidra Medicine, Doha, Qatar.
Gene. 2020 Dec;763S:100037. doi: 10.1016/j.gene.2020.100037. Epub 2020 Jul 31.
Despite the progress made in AAV-based gene therapy targeting different organ systems, lung-targeted gene therapy using AAV vectors has not been effective, mostly due to the poor transduction and un-sustained gene expression in airway epithelium. Furthermore, concerns over possible harmful insertional mutagenesis seen in other cell types, particularly hepatocytes, raised a question about AAV safety. In this study, we evaluate the long-term persistence of this vector in mouse lungs and any possible harmful integration of these vectors into the host genome. AAV6 vectors expressing reporter gene (firefly luciferase) were delivered to the lungs of C57BL/6 mice through intra-tracheal intubation. Despite the large variation among individual animals, most animals had high and sustained luciferase activity with a peak from 2 to 3 weeks post-transduction before a significant decline between 15 and 19 weeks post-transduction. More importantly, even after its decline, most animals maintained detectable luciferase expression for 150 days or more, which was confirmed by post-necropsy qPCR analysis of luciferase gene expression. At the termination point of experiments, an average of one copy of AAV expression cassette per mouse genome was detected. We also found that partial overlaps between the AAV6 expression cassette and the mouse genome were distributed broadly with no apparent systematic preference in any mouse chromosomal map location. In summary, our data suggest that AAV6 mediated long-term gene expression in the lungs with no evidence of genomic integration, and thus, any insertional mutagenesis.
尽管在针对不同器官系统的基于 AAV 的基因治疗方面取得了进展,但使用 AAV 载体进行靶向肺部的基因治疗效果并不理想,这主要是由于气道上皮细胞中的转导效率差和基因表达持续时间短。此外,人们对其他细胞类型(尤其是肝细胞)中可能出现的有害插入突变的担忧,引发了对 AAV 安全性的质疑。在这项研究中,我们评估了该载体在小鼠肺部中的长期持久性,以及这些载体是否有可能对宿主基因组进行有害整合。通过气管内插管将表达报告基因(萤火虫荧光素酶)的 AAV6 载体递送到 C57BL/6 小鼠的肺部。尽管个体动物之间存在很大差异,但大多数动物的荧光素酶活性较高且持续,在转导后 2 至 3 周达到峰值,然后在转导后 15 至 19 周显著下降。更重要的是,即使在其下降后,大多数动物仍能保持可检测的荧光素酶表达 150 天或更长时间,这通过对荧光素酶基因表达的死后 qPCR 分析得到了证实。在实验结束时,平均每个小鼠基因组检测到一个 AAV 表达盒拷贝。我们还发现,AAV6 表达盒与小鼠基因组之间的部分重叠广泛分布,在任何小鼠染色体图谱位置上都没有明显的系统偏好。总之,我们的数据表明,AAV6 介导了肺部的长期基因表达,没有基因组整合的证据,因此也没有插入突变。