Temporal Properties of Flicker ERGs in Rabbit Model of Retinitis Pigmentosa

Okado, Satoshi; Ueno, Shinji; Kominami, Taro; Nakanishi, Atsuko; Inooka, Daiki; Sayo, Akira; Kondo, Mineo; Terasaki, Hiroko

doi:10.1167/iovs.17-22332

Cited by 6 publications

(2 citation statements)

References 28 publications

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“…However, most of these rabbit models were induced experimentally, which limited their application in hereditary eye diseases. Due to the lack of a technology to generate targeted gene modifications in rabbits, for many years, genetically modified rabbit models for ocular disease studies were restricted to transgenic rabbits only (Kondo et al, 2009;Yokoyama et al, 2010;Hirota et al, 2012;Ueno et al, 2013Ueno et al, , 2019Asakawa et al, 2015Asakawa et al, , 2016Nagai et al, 2016;Nakagami et al, 2016;Kominami et al, 2017Kominami et al, , 2019Okado et al, 2017;Roy et al, 2019).…”

Section: Gene Edited Rabbits For Translational Studies Of Ocular Diseasesmentioning

confidence: 99%

Gene Editing in Rabbits: Unique Opportunities for Translational Biomedical Research

Zhang

Yang

et al. 2021

Front. Genet.

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The rabbit is a classic animal model for biomedical research, but the production of gene targeted transgenic rabbits had been extremely challenging until the recent advent of gene editing tools. More than fifty gene knockout or knock-in rabbit models have been reported in the past decade. Gene edited (GE) rabbit models, compared to their counterpart mouse models, may offer unique opportunities in translational biomedical research attributed primarily to their relatively large size and long lifespan. More importantly, GE rabbit models have been found to mimic several disease pathologies better than their mouse counterparts particularly in fields focused on genetically inherited diseases, cardiovascular diseases, ocular diseases, and others. In this review we present selected examples of research areas where GE rabbit models are expected to make immediate contributions to the understanding of the pathophysiology of human disease, and support the development of novel therapeutics.

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Section: Gene Edited Rabbits For Translational Studies Of Ocular Diseasesmentioning

confidence: 99%

Gene Editing in Rabbits: Unique Opportunities for Translational Biomedical Research

Zhang

Yang

et al. 2021

Front. Genet.

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“…Rabbits, compared with mice, are closer to humans in terms of phylogenesis, anatomical features, physiology, and pathophysiological responses [10][11][12][13][14] , and are used as a classic lab animal species to develop novel therapeutics for humans and refine medical and surgical equipment 13,[15][16][17][18][19] . Historically, retinal degeneration has been studied in rhodopsin Pro347Leu transgenic rabbits, a model of RP [20][21][22][23][24][25][26][27][28][29][30][31] . Recently, the emerging gene editing technology in rabbits has greatly increased their value to biomedicine, motivating our efforts to develop rabbits that carry the disease causing mutations found in human patients, as models to replicate human diseases more precisely [32][33][34] .…”

Section: Introductionmentioning

confidence: 99%

USH2A gene mutations in rabbits lead to progressive retinal degeneration and hearing loss

Nguyen

Song

Prieskorn

et al. 2022

Preprint

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Mutations in USH2A gene are responsible for the greatest proportion of hearing and vision loss among individuals with Usher Syndrome (USH) and for autosomal recessive non-syndromic retinitis pigmentosa. Mutations on USH2A exon 13 account for more than 35% of the disease causing USH2A variants including the most prevalence point mutation, c.2299delG, a frameshift mutation. The lack of a clinically relevant animal model has been a bottleneck for the development of therapeutics for USH2A related vision loss. Using CRSPR/Cas9 technology, this study establishes a rabbit line carrying an USH2A frameshift mutation on exon12 (equivalent to human USH2A Exon 13) as a novel mammalian animal model of USH2A. The bi-allelic mutant rabbits exhibit hyper reflective signals in FAF indicating RPE damage and OCT changes indicating photoreceptor degeneration as early as 4 months of age. ERG signals of both rod and cone function were reduced in the USH2A mutant rabbits starting from 7 months old and further decreased at 15-22 months old, indicating progressive retinal photoreceptor degeneration, which is further confirmed by retinal histopathology examination. ABR examination showed moderate to server hearing loss in the USH2A mutant rabbits. These results indicated that disruption of USH2A gene in rabbits is sufficient to induce hearing loss and progressive photoreceptor degeneration. To our knowledge, this is the first mammalian animal model of USH2 which closely recapitulates the phenotype of retinitis pigmentosa in human patients. This study supports the use of rabbits as a clinically relevant animal model to understand the pathogenesis and to develop novel therapeutics for Usher Syndrome.

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Course of loss of photoreceptor function and progressive Müller cell gliosis in rhodopsin P347L transgenic rabbits

Ueno

Kominami

Okado

et al. 2019

Experimental Eye Research

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Temporal Properties of Flicker ERGs in Rabbit Model of Retinitis Pigmentosa

Abstract: The temporal properties of the flicker ERGs of Tg rabbits were affected markedly by the remodeling of the retinal neurons. Evaluations of the flicker ERGs in RP eyes must be done with careful considerations of the current findings.

Cited by 6 publications

References 28 publications

Gene Editing in Rabbits: Unique Opportunities for Translational Biomedical Research

Gene Editing in Rabbits: Unique Opportunities for Translational Biomedical Research

USH2A gene mutations in rabbits lead to progressive retinal degeneration and hearing loss

Course of loss of photoreceptor function and progressive Müller cell gliosis in rhodopsin P347L transgenic rabbits

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