San Raffaele Telethon Institute for Gene Therapy, IRCCS San Raffaele Scientific Institute, Milan, Italy.
Vita-Salute San Raffaele University, Milan, Italy.
EMBO Mol Med. 2024 Jun;16(6):1427-1450. doi: 10.1038/s44321-024-00072-8. Epub 2024 Apr 29.
Lentiviral vectors (LV) are efficient vehicles for in vivo gene delivery to the liver. LV integration into the chromatin of target cells ensures their transmission upon proliferation, thus allowing potentially life-long gene therapy following a single administration, even to young individuals. The glycoprotein of the vesicular stomatitis virus (VSV.G) is widely used to pseudotype LV, as it confers broad tropism and high stability. The baculovirus-derived GP64 envelope protein has been proposed as an alternative for in vivo liver-directed gene therapy. Here, we perform a detailed comparison of VSV.G- and GP64-pseudotyped LV in vitro and in vivo. We report that VSV.G-LV transduced hepatocytes better than GP64-LV, however the latter showed improved transduction of liver sinusoidal endothelial cells (LSEC). Combining GP64-pseudotyping with the high surface content of the phagocytosis inhibitor CD47 further enhanced LSEC transduction. Coagulation factor VIII (FVIII), the gene mutated in hemophilia A, is naturally expressed by LSEC, thus we exploited GP64-LV to deliver a FVIII transgene under the control of the endogenous FVIII promoter and achieved therapeutic amounts of FVIII and correction of hemophilia A mice.
慢病毒载体 (LV) 是将基因递送至肝脏的有效工具。LV 整合到靶细胞的染色质中可确保其在增殖时传递,从而允许在单次给药后进行潜在的终身基因治疗,甚至可以用于年轻人。水疱性口炎病毒 (VSV.G) 的糖蛋白广泛用于假型 LV,因为它赋予广泛的嗜性和高稳定性。杆状病毒衍生的 GP64 包膜蛋白已被提议作为体内肝定向基因治疗的替代物。在这里,我们在体外和体内对 VSV.G-和 GP64-假型 LV 进行了详细比较。我们报告说,VSV.G-LV 比 GP64-LV 更好地转导肝细胞,但后者显示出对肝窦内皮细胞 (LSEC) 的转导改善。将 GP64 假型化与吞噬抑制剂 CD47 的高表面含量结合使用,进一步增强了 LSEC 的转导。凝血因子 VIII (FVIII) 是血友病 A 中突变的基因,天然由 LSEC 表达,因此我们利用 GP64-LV 在内源性 FVIII 启动子的控制下传递 FVIII 转基因,并实现了治疗量的 FVIII 和纠正血友病 A 小鼠。