Petit Lolita, Ma Shan, Cheng Shun-Yun, Gao Guangping, Punzo Claudio
1 Department of Ophthalmology and Gene Therapy Center, University of Massachusetts Medical School , Worcester, Massachusetts.
3 Department of Microbiology and Physiological Systems and Gene Therapy Center, University of Massachusetts Medical School , Worcester, Massachusetts.
Hum Gene Ther. 2017 Jun;28(6):464-481. doi: 10.1089/hum.2017.020.
Vectors based on the adeno-associated virus (AAV) are currently the preferred tools for delivering genes to photoreceptors (PR) in small and large animals. AAVs have been applied successfully in various models of PR dystrophies. However, unknown barriers still limit AAV's efficient application in several forms of severe PR degenerations due to insufficient transgene expression and/or treated cells at the time of injection. Optimizations of PR gene therapy strategies will likely benefit from the identification of the cellular factors that influence PR transduction. Interestingly, recent studies have shown that the AAV transduction profile of PRs differs significantly between neonatal and adult mouse retinas after subretinal injection. This phenomenon may provide clues to identify host factors that influence the efficiency of AAV-mediated PR transduction. This study demonstrates that rod outer segments are critical modulators of efficient AAV-mediated rod transduction. During retinal development, rod transduction correlated temporally and spatially with the differentiation order of PRs when vectors were introduced subretinally but not when introduced intravitreally. All subretinally injected vectors had an initial preference to transduce cones in the absence of formed rod outer segments and then displayed a preference for rods as the cells matured, independently of the expression cassette or AAV serotype. Consistent with this observation, altered development of rod outer segments was associated with a strong reduction of rod transduction and an increase in the percentage of transduced cones by 2- to 2.8-fold. A similar increase of cone transduction was observed in the adult retinal degeneration 1 (rd1) retina compared to wild-type mice. These results suggest that the loss of rod outer segments in diseased retinas could markedly affect gene transfer efficiency of AAV vectors by limiting the ability of AAVs to infect dying rods efficiently. This information could be exploited for the development of more efficient AAV-based PR gene delivery procedures.
基于腺相关病毒(AAV)的载体目前是在大小动物中将基因递送至光感受器(PR)的首选工具。AAV已成功应用于多种PR营养不良模型。然而,由于注射时转基因表达不足和/或处理的细胞数量不足,未知的障碍仍然限制了AAV在几种严重PR变性形式中的有效应用。PR基因治疗策略的优化可能受益于对影响PR转导的细胞因子的识别。有趣的是,最近的研究表明,视网膜下注射后,新生小鼠和成年小鼠视网膜中PR的AAV转导谱存在显著差异。这种现象可能为识别影响AAV介导的PR转导效率的宿主因子提供线索。本研究表明,视杆细胞外段是AAV介导的有效视杆细胞转导的关键调节因子。在视网膜发育过程中,当通过视网膜下注射引入载体时,视杆细胞转导在时间和空间上与PR的分化顺序相关,而通过玻璃体注射引入时则不相关。在没有形成视杆细胞外段的情况下,所有视网膜下注射的载体最初都倾向于转导视锥细胞,然后随着细胞成熟而表现出对视杆细胞的偏好,这与表达盒或AAV血清型无关。与这一观察结果一致,视杆细胞外段发育的改变与视杆细胞转导的显著降低以及转导视锥细胞的百分比增加2至2.8倍有关。与野生型小鼠相比,在成年视网膜变性1(rd1)视网膜中也观察到了类似的视锥细胞转导增加。这些结果表明,患病视网膜中视杆细胞外段的丧失可能通过限制AAV有效感染濒死视杆细胞的能力而显著影响AAV载体的基因转移效率。这一信息可用于开发更有效的基于AAV的PR基因递送程序。