ArticleInvestigative ophthalmology & visual science2012
Generation of an inbred miniature pig model of retinitis pigmentosa.
Article in Investigative ophthalmology & visual science, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 99 papers.
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99 citing papers in PubMed, 154 citations in OpenAlex.
- Retinitis pigmentosa elicits neurodegeneration within the visual pathway in REEP6 knockout mice.Neural regeneration research · 2026Article
- A porcine commotio retinae model for preclinical evaluation of posttraumatic photoreceptor degeneration.JCI insight · 2026Article
- Subretinal Injection Volume Correlates to Persistent Outer Retinal Thinning in the Pig Eye.Investigative ophthalmology & visual science · 2025Article
- Graphene oxide increases the phototransduction efficiency of copolymeric nanoimplants and rescues visual functions in rat and pig models of Retinitis pigmentosa.Nature communications · 2025Article
- RHO1-2 meganuclease gene editing targets human P23H rhodopsin-induced retinitis pigmentosa to rejuvenate rods and maintain cones.bioRxiv : the preprint server for biology · 2025Article
- Metabolic regulation of visual acuity.Science advances · 2025Article
- Robust generation of photoreceptor-dominant retinal organoids from porcine induced pluripotent stem cells.Stem cell reports · 2025Article
- Rabbit model of oxidative stress-induced retinal degeneration.Free radical biology & medicine · 2025Article
- Mechanisms of Rhodopsin-Related Inherited Retinal Degeneration and Pharmacological Treatment Strategies.Cells · 2025Review
- Pig Models in Retinal Research and Retinal Disease.Cold Spring Harbor perspectives in medicine · 2024Review
- An Automated Visual Psychophysics Method to Measure Visual Function in Swine Preclinical Animal Model.Translational vision science & technology · 2024Article
- CRISPRi-Mediated Treatment of Dominant Rhodopsin-Associated Retinitis Pigmentosa.The CRISPR journal · 2023Article
- Metabolic transcriptomics dictate responses of cone photoreceptors to retinitis pigmentosa.Cell reports · 2023Article
- A versatile laser-induced porcine model of outer retinal and choroidal degeneration for preclinical testing.JCI insight · 2023Article
- Testing spatial working memory in pigs using an automated T-maze.Oxford open neuroscience · 2023Article
- Network biology analysis of P23H rhodopsin interactome identifies protein and mRNA quality control mechanisms.Scientific reports · 2022Article
- The p-ERG spatial acuity in the biomedical pig under physiological conditions.Scientific reports · 2022Article
- Gene Therapy for Rhodopsin Mutations.Cold Spring Harbor perspectives in medicine · 2022Article
- In vivo study to assess dosage of allogeneic pig retinal progenitor cells: Long-term survival, engraftment, differentiation and safety.Journal of cellular and molecular medicine · 2022Article
- Subcellular localization of mutant P23H rhodopsin in an RFP fusion knock-in mouse model of retinitis pigmentosa.Disease models & mechanisms · 2022Article
39 more citing papers are in PubMed but not listed here.
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16 authors at 5 institutions in 2 countries.
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Abstract
purposeThe Pro23His (P23H) rhodopsin (RHO) mutation underlies the most common form of human autosomal dominant retinitis pigmentosa (adRP). The objective of this investigation was to establish a transgenic miniature swine model of RP using the human P23H RHO gene.
methodsSomatic cell nuclear transfer (SCNT) was used to create transgenic miniature pigs that expressed the human P23H RHO mutation. From these experiments, six transgenic founders were identified whose retinal function was studied with full-field electroretinography (ffERG) from 3 months through 2 years. Progeny from one founder were generated and genotyped to determine transgene inheritance pattern. Retinal mRNA was isolated, and the ratio of P23H to wild-type pig RHO was measured.
resultsA single transgene integration site was observed for five of the six founders. All founders had abnormal scotopic and photopic ffERGs after 3 months. The severity of the ffERG phenotype was grouped into moderately and severely affected groups. Offspring of one founder inherited the transgene as an autosomal dominant mutation. mRNA analyses demonstrated that approximately 80% of total RHO was mutant P23H.
conclusionsExpression of the human RHO P23H transgene in the retina creates a miniature swine model with an inheritance pattern and retinal function that mimics adRP. This large-animal model can serve as a novel tool for the study of the pathogenesis and therapeutic intervention in the most common form of adRP.
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