• 文献检索
  • 文档翻译
  • 深度研究
  • 学术资讯
  • Suppr Zotero 插件Zotero 插件
  • 邀请有礼
  • 套餐&价格
  • 历史记录
应用&插件
Suppr Zotero 插件Zotero 插件浏览器插件Mac 客户端Windows 客户端微信小程序
定价
高级版会员购买积分包购买API积分包
服务
文献检索文档翻译深度研究API 文档MCP 服务
关于我们
关于 Suppr公司介绍联系我们用户协议隐私条款
关注我们

Suppr 超能文献

核心技术专利：CN118964589B侵权必究
粤ICP备2023148730 号-1Suppr @ 2026

文献检索

告别复杂PubMed语法，用中文像聊天一样搜索，搜遍4000万医学文献。AI智能推荐，让科研检索更轻松。

立即免费搜索

文件翻译

保留排版，准确专业，支持PDF/Word/PPT等文件格式，支持 12+语言互译。

免费翻译文档

深度研究

AI帮你快速写综述，25分钟生成高质量综述，智能提取关键信息，辅助科研写作。

立即免费体验

视网膜色素变性小型猪模型中Pro23His视网膜病变的进展

Progression of Pro23His Retinopathy in a Miniature Swine Model of Retinitis Pigmentosa.

作者信息

Scott Patrick A, de Castro Juan P Fernandez, DeMarco Paul J, Ross Jason W, Njoka Josephat, Walters Eric, Prather Randall S, McCall Maureen A, Kaplan Henry J

机构信息

Department of Ophthalmology & Visual Sciences, University of Louisville, Louisville, KY, USA ; Department of Anatomical Sciences and Neurobiology, University of Louisville, Louisville, KY, USA.

Department of Ophthalmology & Visual Sciences, University of Louisville, Louisville, KY, USA.

出版信息

Transl Vis Sci Technol. 2017 Mar 15;6(2):4. doi: 10.1167/tvst.6.2.4. eCollection 2017 Mar.

DOI:10.1167/tvst.6.2.4
PMID:28316877
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5354474/
Abstract

PURPOSE

We characterize the progression of retinopathy in Filial 1 (F1) progeny of a transgenic (Tg) founder miniswine exhibiting severe Pro23His (P23H) retinopathy.

METHODS

The F1 TgP23H miniswine progeny were created by crossing TgP23H founder miniswine 53-1 with wild type (WT) inbred miniature swine. Scotopic (rod-driven) and photopic (cone-driven) retinal functions were evaluated in F1 TgP23H and WT littermates using full field electroretinograms (ffERGs) at 1, 2, 3, 6, 9, 12, and 18 months of age, as well as the Tg founder miniswine at 6 years of age. Miniswine were euthanized and their retinas processed for morphologic evaluation at the light and electron microscopic level. Retinal morphology of a 36-month-old Tg miniswine also was examined.

RESULTS

Wild type littermates reached mature scotopic and photopic retinal function by 3 months, while TgP23H miniswine showed abnormal scotopic ffERGs at the earliest time point, 1 month, and depressed photopic ffERGs after 2 months. Rod and cone photoreceptors (PR) exhibited morphologic abnormalities and dropout from the outer nuclear layer at 1 month, with only a monolayer of cone PR somata remaining after 2 months. The retinas showed progressive neural remodeling of the outer retina that included dendritic retraction of rod bipolar cells and glial seal formation by Müller cells. The TgP23H founder miniswine showed cone PR with relatively intact morphology exclusive to the area centralis.

CONCLUSIONS

The F1 Tg miniswine and the TgP23H founder miniswine exhibit similar retinopathy.

TRANSLATIONAL RELEVANCE

TgP23H miniswine are a useful large-eye model to study pathogenesis and preservation cone PRs in humans with retinitis pigmentosa.

摘要

目的

我们对表现出严重Pro23His（P23H）视网膜病变的转基因（Tg）创始小型猪的子一代（F1）后代的视网膜病变进展进行了特征描述。

方法

通过将TgP23H创始小型猪53-1与野生型（WT）近交小型猪杂交，培育出F1 TgP23H小型猪后代。在1、2、3、6、9、12和18月龄时，使用全视野视网膜电图（ffERG）对F1 TgP23H和WT同窝仔猪的暗视（视杆细胞驱动）和明视（视锥细胞驱动）视网膜功能进行评估，同时在6岁时对Tg创始小型猪进行评估。对小型猪实施安乐死，并对其视网膜进行处理，以进行光镜和电镜水平的形态学评估。还检查了一只36月龄Tg小型猪的视网膜形态。

结果

野生型同窝仔猪在3个月时达到成熟的暗视和明视视网膜功能，而TgP23H小型猪在最早的时间点即1个月时就表现出异常的暗视ffERG，2个月后明视ffERG降低。视杆和视锥光感受器（PR）在1个月时出现形态异常并从外核层脱落，2个月后仅剩下单层视锥PR胞体。视网膜显示外层视网膜进行性神经重塑，包括视杆双极细胞的树突回缩和Müller细胞形成胶质封闭。TgP23H创始小型猪的视锥PR在中央凹区域形态相对完整。

结论

F1 Tg小型猪和TgP23H创始小型猪表现出相似的视网膜病变。

转化相关性

TgP23H小型猪是研究人类色素性视网膜炎发病机制和保存视锥PR的有用的大眼睛模型。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6425/5354474/fc0b6bbbe6e2/i2164-2591-6-2-4-f11.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6425/5354474/9432f953ebde/i2164-2591-6-2-4-f01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6425/5354474/ebbb77c819d3/i2164-2591-6-2-4-f02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6425/5354474/dc7bce303a29/i2164-2591-6-2-4-f03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6425/5354474/6802b91dab13/i2164-2591-6-2-4-f04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6425/5354474/021e84eb9fd6/i2164-2591-6-2-4-f05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6425/5354474/68885abc741f/i2164-2591-6-2-4-f06.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6425/5354474/2be8c5cafed0/i2164-2591-6-2-4-f07.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6425/5354474/b0443bd8b2b3/i2164-2591-6-2-4-f08.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6425/5354474/03cbabaf2961/i2164-2591-6-2-4-f09.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6425/5354474/a04b05485d50/i2164-2591-6-2-4-f10.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6425/5354474/fc0b6bbbe6e2/i2164-2591-6-2-4-f11.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6425/5354474/9432f953ebde/i2164-2591-6-2-4-f01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6425/5354474/ebbb77c819d3/i2164-2591-6-2-4-f02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6425/5354474/dc7bce303a29/i2164-2591-6-2-4-f03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6425/5354474/6802b91dab13/i2164-2591-6-2-4-f04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6425/5354474/021e84eb9fd6/i2164-2591-6-2-4-f05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6425/5354474/68885abc741f/i2164-2591-6-2-4-f06.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6425/5354474/2be8c5cafed0/i2164-2591-6-2-4-f07.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6425/5354474/b0443bd8b2b3/i2164-2591-6-2-4-f08.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6425/5354474/03cbabaf2961/i2164-2591-6-2-4-f09.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6425/5354474/a04b05485d50/i2164-2591-6-2-4-f10.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6425/5354474/fc0b6bbbe6e2/i2164-2591-6-2-4-f11.jpg

相似文献

1
Progression of Pro23His Retinopathy in a Miniature Swine Model of Retinitis Pigmentosa.视网膜色素变性小型猪模型中Pro23His视网膜病变的进展
Transl Vis Sci Technol. 2017 Mar 15;6(2):4. doi: 10.1167/tvst.6.2.4. eCollection 2017 Mar.
2
Cone photoreceptors develop normally in the absence of functional rod photoreceptors in a transgenic swine model of retinitis pigmentosa.在色素性视网膜炎的转基因猪模型中，当杆状光感受器失去功能时，圆锥状光感受器仍能正常发育。
Invest Ophthalmol Vis Sci. 2014 Apr 17;55(4):2460-8. doi: 10.1167/iovs.13-13724.
3
A Pro23His mutation alters prenatal rod photoreceptor morphology in a transgenic swine model of retinitis pigmentosa.一种 Pro23His 突变改变了视网膜色素变性转基因猪模型中的产前杆状光感受器形态。
Invest Ophthalmol Vis Sci. 2014 Apr 28;55(4):2452-9. doi: 10.1167/iovs.13-13723.
4
Prenatal Exposure to Curcumin Protects Rod Photoreceptors in a Transgenic Pro23His Swine Model of Retinitis Pigmentosa.产前暴露于姜黄素可保护视网膜色素变性转基因Pro23His猪模型中的视杆光感受器。
Transl Vis Sci Technol. 2015 Sep 16;4(5):5. doi: 10.1167/tvst.4.5.5. eCollection 2015 Sep.
5
Generation of an inbred miniature pig model of retinitis pigmentosa.致盲性眼病——色素性视网膜炎的近交系小型猪模型的建立
Invest Ophthalmol Vis Sci. 2012 Jan 31;53(1):501-7. doi: 10.1167/iovs.11-8784.
6
Noninvasive Imaging and Correlative Histology of Cone Photoreceptor Structure in the Pig Retina.猪视网膜中视锥光感受器结构的无创成像与相关组织学研究
Transl Vis Sci Technol. 2019 Dec 18;8(6):38. doi: 10.1167/tvst.8.6.38. eCollection 2019 Nov.
7
Retinal Phenotype in the rd9 Mutant Mouse, a Model of X-Linked RP.rd9突变小鼠的视网膜表型，一种X连锁视网膜色素变性模型。
Front Neurosci. 2019 Sep 19;13:991. doi: 10.3389/fnins.2019.00991. eCollection 2019.
8
Long-term preservation of cone photoreceptors and visual acuity in rd10 mutant mice exposed to continuous environmental enrichment.长期暴露于持续环境富集的rd10突变小鼠中视锥光感受器和视力的保存
Mol Vis. 2014 Nov 5;20:1545-56. eCollection 2014.
9
Astrocytes and Müller Cell Alterations During Retinal Degeneration in a Transgenic Rat Model of Retinitis Pigmentosa.视网膜色素变性转基因大鼠模型视网膜变性过程中星形胶质细胞和米勒细胞的改变
Front Cell Neurosci. 2015 Dec 22;9:484. doi: 10.3389/fncel.2015.00484. eCollection 2015.
10
Integration of Perforated Subretinal Prostheses With Retinal Tissue.穿孔性视网膜下假体与视网膜组织的整合。
Transl Vis Sci Technol. 2015 Aug 14;4(4):5. doi: 10.1167/tvst.4.4.5. eCollection 2015 Aug.

引用本文的文献

1
Animal models for the evaluation of retinal stem cell therapies.用于评估视网膜干细胞疗法的动物模型。
Prog Retin Eye Res. 2025 May;106:101356. doi: 10.1016/j.preteyeres.2025.101356. Epub 2025 Apr 14.
2
Synchronized Photoactivation of T4K Rhodopsin Causes a Chromophore-Dependent Retinal Degeneration That Is Moderated by Interaction with Phototransduction Cascade Components.T4K 视紫红质的光激活同步化会导致依赖发色团的视网膜变性，这种变性会受到与光转导级联成分相互作用的调节。
J Neurosci. 2024 Sep 4;44(36):e0453242024. doi: 10.1523/JNEUROSCI.0453-24.2024.
3
CRISPRi-Mediated Treatment of Dominant Rhodopsin-Associated Retinitis Pigmentosa.

本文引用的文献

1
Prenatal Exposure to Curcumin Protects Rod Photoreceptors in a Transgenic Pro23His Swine Model of Retinitis Pigmentosa.产前暴露于姜黄素可保护视网膜色素变性转基因Pro23His猪模型中的视杆光感受器。
Transl Vis Sci Technol. 2015 Sep 16;4(5):5. doi: 10.1167/tvst.4.5.5. eCollection 2015 Sep.
2
Cone photoreceptors develop normally in the absence of functional rod photoreceptors in a transgenic swine model of retinitis pigmentosa.在色素性视网膜炎的转基因猪模型中，当杆状光感受器失去功能时，圆锥状光感受器仍能正常发育。
Invest Ophthalmol Vis Sci. 2014 Apr 17;55(4):2460-8. doi: 10.1167/iovs.13-13724.
3
A Pro23His mutation alters prenatal rod photoreceptor morphology in a transgenic swine model of retinitis pigmentosa.
CRISPRi 介导的显性视紫红质相关视网膜色素变性治疗。
CRISPR J. 2023 Dec;6(6):502-513. doi: 10.1089/crispr.2023.0039.
4
A Novel Porcine Model of CLN2 Batten Disease that Recapitulates Patient Phenotypes.一种新型 CLN2 神经鞘脂贮积症猪模型，可重现患者表型。
Neurotherapeutics. 2022 Oct;19(6):1905-1919. doi: 10.1007/s13311-022-01296-7. Epub 2022 Sep 13.
5
POLYRETINA restores light responses in vivo in blind Göttingen minipigs.POLYRETINA 可恢复活体失明豚鼠的光反应。
Nat Commun. 2022 Jun 27;13(1):3678. doi: 10.1038/s41467-022-31180-z.
6
Metabolic rescue of cone photoreceptors in retinitis pigmentosa.视网膜色素变性中视锥光感受器的代谢挽救
Taiwan J Ophthalmol. 2021 Dec 6;11(4):331-335. doi: 10.4103/tjo.tjo_46_21. eCollection 2021 Oct-Dec.
7
An experimental pig model with outer retinal degeneration induced by temporary intravitreal loading of N-methyl-N-nitrosourea during vitrectomy.玻璃体切割术中临时玻璃体内注射 N-甲基-N-亚硝脲诱导的外视网膜变性实验猪模型。
Sci Rep. 2021 Jan 8;11(1):258. doi: 10.1038/s41598-020-79437-1.
8
Metabolic and Redox Signaling of the Nucleoredoxin-Like-1 Gene for the Treatment of Genetic Retinal Diseases.核蛋白样 1 基因的代谢和氧化还原信号转导在遗传性视网膜疾病治疗中的作用。
Int J Mol Sci. 2020 Feb 27;21(5):1625. doi: 10.3390/ijms21051625.
9
Noninvasive Imaging and Correlative Histology of Cone Photoreceptor Structure in the Pig Retina.猪视网膜中视锥光感受器结构的无创成像与相关组织学研究
Transl Vis Sci Technol. 2019 Dec 18;8(6):38. doi: 10.1167/tvst.8.6.38. eCollection 2019 Nov.
10
Two-photon polymerized poly(caprolactone) retinal cell delivery scaffolds and their systemic and retinal biocompatibility.双光子聚合聚己内酯视网膜细胞递药支架及其系统和视网膜生物相容性。
Acta Biomater. 2019 Aug;94:204-218. doi: 10.1016/j.actbio.2019.04.057. Epub 2019 May 3.
一种 Pro23His 突变改变了视网膜色素变性转基因猪模型中的产前杆状光感受器形态。
Invest Ophthalmol Vis Sci. 2014 Apr 28;55(4):2452-9. doi: 10.1167/iovs.13-13723.
4
Generation of an inbred miniature pig model of retinitis pigmentosa.致盲性眼病——色素性视网膜炎的近交系小型猪模型的建立
Invest Ophthalmol Vis Sci. 2012 Jan 31;53(1):501-7. doi: 10.1167/iovs.11-8784.
5
Mislocalization and degradation of human P23H-rhodopsin-GFP in a knockin mouse model of retinitis pigmentosa.人 P23H-视紫红质-GFP 在视网膜色素变性 knockin 小鼠模型中的定位错误和降解。
Invest Ophthalmol Vis Sci. 2011 Dec 28;52(13):9728-36. doi: 10.1167/iovs.11-8654.
6
Probing mechanisms of photoreceptor degeneration in a new mouse model of the common form of autosomal dominant retinitis pigmentosa due to P23H opsin mutations.研究 P23H 视蛋白突变引起的常染色体显性遗传视网膜色素变性新型小鼠模型中光感受器变性的机制。
J Biol Chem. 2011 Mar 25;286(12):10551-67. doi: 10.1074/jbc.M110.209759. Epub 2011 Jan 11.
7
A battery of cell- and structure-specific markers for the adult porcine retina.一组用于成年猪视网膜的细胞和结构特异性标记物。
J Histochem Cytochem. 2010 Apr;58(4):377-89. doi: 10.1369/jhc.2009.954933. Epub 2010 Jan 19.
8
Remodeling of cone photoreceptor cells after rod degeneration in rd mice.视网膜变性小鼠杆状细胞退化后视锥光感受器细胞的重塑。
Exp Eye Res. 2009 Mar;88(3):589-99. doi: 10.1016/j.exer.2008.11.022. Epub 2008 Dec 6.
9
ISCEV Standard for full-field clinical electroretinography (2008 update).国际临床视觉电生理学会全视野临床视网膜电图标准（2008年更新版）
Doc Ophthalmol. 2009 Feb;118(1):69-77. doi: 10.1007/s10633-008-9155-4. Epub 2008 Nov 22.
10
Photoreceptor density of the domestic pig retina.
Vet Ophthalmol. 1999;2(3):179-184. doi: 10.1046/j.1463-5224.1999.00077.x.