双突变视紫红质蛋白（Rho-R135G/G188R）转基因斑马鱼视网膜色素变性模型的建立
摘要
目的：建立一种视紫红质双突变-R135G/G188R暂时性(transient)转基因斑马鱼视网膜色素变性(retinitis pigmentosa, RP)模型,并检验该突变所致视网膜变性的特征。
方法：将两种经基因工程改造的质粒pXOP1.3-EGFP-rho和pXOP1.3-EGFP-rho-R135G/G188R通过显微注射的方法导入野生型斑马鱼胚胎,制造出两种暂时性转基因斑马鱼(F0代),分别表达外源性的野生型斑马鱼视紫红质或其双突变体(Rho-R135G/G188R)。用活体常规荧光显微镜观察法筛选出增强型绿色荧光蛋白(EGFP)阳性的F0代转基因幼鱼。并用荧光和/或免疫荧光冰冻切片分析技术检测转基因视紫红质的水平和其在视网膜上的分布,以及视网膜变性的表现。
结果：在本试验中,1.3-kb的爪蟾视紫红质启动子(Xenopus rhodopsin promoter,XOP)片段足以替代全长5.5-kb的XOP启动转基因构造表达。通过常规荧光显微镜下观察完整活体斑马鱼幼鱼眼睛荧光表达情况以及图像采集,可鉴别出成功的转基因个体：三次显微注射试验总计约1000枚胚胎中,平均约5%-7%的幼鱼呈EGFP阳性。冰冻切片分析显示,鱼卵受精后第5天(5days post fertilization,5dpf) Rho-R135G/G188R表达水平即下降,相反,对照转基因幼鱼外源性视紫红质表达水平却明显升高；结合EGFP在视网膜上的分布位置分析,可能是转入的双突变视紫红质的表达导致了视杆细胞特异性的视网膜变性,考虑到对照外源性转基因视紫红质没有引起此现象,提示视杆细胞死亡是呈R135G/G188R突变特异性的。
结论：利用由细胞特异性启动子加上带有EGFP编码序列标签的两种Rho CDS片段组成的质粒,可以成功制备出两种暂时性转基因斑马鱼RP模型；该模型证实1.3-kbXOP片段是一种跨物种性的足够强的启动子,也为Ⅱ类视紫红质突变——R135G/G188R导致视紫红质蛋白折叠错误进而发生以视杆细胞死亡为表型的RP这一说法提供了支持。这些转基因斑马鱼有望作为人类视网膜色素变性遗传病的模型,以其EGFP-Rho融合蛋白为探针来揭示视网膜变性的潜在病理机制,甚至可能为探索该病的基因治疗方法提供实验材料。
PURPOSE. To generate a transgenic zebrafish model of retinitis pigmentosa (RP), in which the retinal degeneration was induced by rhodopsin double mutation-R135G/G188R, and to investigate the characteristics of its retinal degeneration.
METHODS. Transient transgenic zebrafish (FO generations) were generated via embryo microinjection with genetically engineered pXOP1.3-EGFP-rho and pXOP1.3-EGFP-rho R135G/G188R plasmids that expressed the wild-type zebrafish rhodopsin or its double mutants, respectively. Those EGFP-positive FO generation transgenic larvae were screened out via in vivo conventional fluorescence microscope observation. Using fluorescence or immunofluorescence cryosection assay, transgenic rhodopsin levels and its localizations in the retina, as well as the characteristics of retinal degeneration was determined by fluorescence microscopy.
RESULTS. The1.3-kb Xenopus rhodopsin promoter fragment instead of the whole5.5-kb XOP was sufficient to promote the expression of our transgenic constructs. And it was possible to identify the transgenic animals by obtaining fluorescence images of the eyes of intact, living zebrafish larvae under conventional fluorescence microscope:during three rounds of injections, an approximate5%-7%mean EGFP-positive rate was obtained in about1000embryos. On cryosection assay, Rhodopsin-R135G/G188R expression levels decreased by the time of5dpf as opposed to that of the control transgenic rhodopsin, which obviously increased. Along with the retinal EGFP distribution feature, this observation suggested that the double mutated rhodopsin might have induced rod-specific retinal degeneration; whereas the control transgenic rhodopsin did not, indicating that rod photoreceptor death was specific to the R135G/G188R mutation.
CONCLUSIONS. Using plasmids consisting of cell-specific promoter plus EGFP CDS tagged double mutant of the Rho CDS, a transient transgenic zebrafish RP model was generated. This model verified that the1.3kb XOP fragment was a sufficient rod-specific promoter across species, and supported a role for the possibility that Class Ⅱ rhodopsin mutation-R135G/G188R caused misfolding in RP with a phenotype of rod death. Meanwhile, these zebrafish implicated the potential of being used as models of human inherited retinitis pigmentosa and the utility of EGFP-Rho fusion protein as a probe for discovering the underlying pathogenic mechanisms of this disease, or even being used as experimental materials to explore its gene therapy.
方法：将两种经基因工程改造的质粒pXOP1.3-EGFP-rho和pXOP1.3-EGFP-rho-R135G/G188R通过显微注射的方法导入野生型斑马鱼胚胎,制造出两种暂时性转基因斑马鱼(F0代),分别表达外源性的野生型斑马鱼视紫红质或其双突变体(Rho-R135G/G188R)。用活体常规荧光显微镜观察法筛选出增强型绿色荧光蛋白(EGFP)阳性的F0代转基因幼鱼。并用荧光和/或免疫荧光冰冻切片分析技术检测转基因视紫红质的水平和其在视网膜上的分布,以及视网膜变性的表现。
结果：在本试验中,1.3-kb的爪蟾视紫红质启动子(Xenopus rhodopsin promoter,XOP)片段足以替代全长5.5-kb的XOP启动转基因构造表达。通过常规荧光显微镜下观察完整活体斑马鱼幼鱼眼睛荧光表达情况以及图像采集,可鉴别出成功的转基因个体：三次显微注射试验总计约1000枚胚胎中,平均约5%-7%的幼鱼呈EGFP阳性。冰冻切片分析显示,鱼卵受精后第5天(5days post fertilization,5dpf) Rho-R135G/G188R表达水平即下降,相反,对照转基因幼鱼外源性视紫红质表达水平却明显升高；结合EGFP在视网膜上的分布位置分析,可能是转入的双突变视紫红质的表达导致了视杆细胞特异性的视网膜变性,考虑到对照外源性转基因视紫红质没有引起此现象,提示视杆细胞死亡是呈R135G/G188R突变特异性的。
结论：利用由细胞特异性启动子加上带有EGFP编码序列标签的两种Rho CDS片段组成的质粒,可以成功制备出两种暂时性转基因斑马鱼RP模型；该模型证实1.3-kbXOP片段是一种跨物种性的足够强的启动子,也为Ⅱ类视紫红质突变——R135G/G188R导致视紫红质蛋白折叠错误进而发生以视杆细胞死亡为表型的RP这一说法提供了支持。这些转基因斑马鱼有望作为人类视网膜色素变性遗传病的模型,以其EGFP-Rho融合蛋白为探针来揭示视网膜变性的潜在病理机制,甚至可能为探索该病的基因治疗方法提供实验材料。
PURPOSE. To generate a transgenic zebrafish model of retinitis pigmentosa (RP), in which the retinal degeneration was induced by rhodopsin double mutation-R135G/G188R, and to investigate the characteristics of its retinal degeneration.
METHODS. Transient transgenic zebrafish (FO generations) were generated via embryo microinjection with genetically engineered pXOP1.3-EGFP-rho and pXOP1.3-EGFP-rho R135G/G188R plasmids that expressed the wild-type zebrafish rhodopsin or its double mutants, respectively. Those EGFP-positive FO generation transgenic larvae were screened out via in vivo conventional fluorescence microscope observation. Using fluorescence or immunofluorescence cryosection assay, transgenic rhodopsin levels and its localizations in the retina, as well as the characteristics of retinal degeneration was determined by fluorescence microscopy.
RESULTS. The1.3-kb Xenopus rhodopsin promoter fragment instead of the whole5.5-kb XOP was sufficient to promote the expression of our transgenic constructs. And it was possible to identify the transgenic animals by obtaining fluorescence images of the eyes of intact, living zebrafish larvae under conventional fluorescence microscope:during three rounds of injections, an approximate5%-7%mean EGFP-positive rate was obtained in about1000embryos. On cryosection assay, Rhodopsin-R135G/G188R expression levels decreased by the time of5dpf as opposed to that of the control transgenic rhodopsin, which obviously increased. Along with the retinal EGFP distribution feature, this observation suggested that the double mutated rhodopsin might have induced rod-specific retinal degeneration; whereas the control transgenic rhodopsin did not, indicating that rod photoreceptor death was specific to the R135G/G188R mutation.
CONCLUSIONS. Using plasmids consisting of cell-specific promoter plus EGFP CDS tagged double mutant of the Rho CDS, a transient transgenic zebrafish RP model was generated. This model verified that the1.3kb XOP fragment was a sufficient rod-specific promoter across species, and supported a role for the possibility that Class Ⅱ rhodopsin mutation-R135G/G188R caused misfolding in RP with a phenotype of rod death. Meanwhile, these zebrafish implicated the potential of being used as models of human inherited retinitis pigmentosa and the utility of EGFP-Rho fusion protein as a probe for discovering the underlying pathogenic mechanisms of this disease, or even being used as experimental materials to explore its gene therapy.
引文
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