ArticleThe Journal of neuroscience : the official journal of the Society for Neuroscience2011
Regeneration of photopigment is enhanced in mouse cone photoreceptors expressing RPE65 protein.
Article in The Journal of neuroscience : the official journal of the Society for Neuroscience, 2011. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 21 papers.
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Who cites it
21 citing papers in PubMed, 23 citations in OpenAlex.
- Long-term effect of gene therapy on Leber's congenital amaurosis.The New England journal of medicine · 2015Trial
- Complement C3 knockout protects photoreceptors in the sodium iodate model.Experimental eye research · 2025Article
- A comparison of optophysiological biomarkers of photoreceptor stress and phototoxicity in BALB/cJ, B6 (Cg)-Tyrc-2J/J, and C57Bl/6J mouse strains.Frontiers in ophthalmology · 2023Article
- Retinal pigment epithelium 65 kDa protein (RPE65): An update.Progress in retinal and eye research · 2022Review
- An inducible Cre mouse for studying roles of the RPE in retinal physiology and disease.JCI insight · 2021Article
- The microphthalmia-associated transcription factor (Mitf) gene and its role in regulating eye function.Scientific reports · 2019Article
- Examining the Role of Cone-expressed RPE65 in Mouse Cone Function.Scientific reports · 2018Article
- A subpopulation of activated retinal macrophages selectively migrated to regions of cone photoreceptor stress, but had limited effect on cone death in a mouse model for type 2 Leber congenital amaurosis.Molecular and cellular neurosciences · 2017Article
- The retina visual cycle is driven by cis retinol oxidation in the outer segments of cones.Visual neuroscience · 2017Article
- Evolution of mammalian Opn5 as a specialized UV-absorbing pigment by a single amino acid mutation.The Journal of biological chemistry · 2014Article
- Immuno-histochemical analysis of rod and cone reaction to RPE65 deficiency in the inferior and superior canine retina.PloS one · 2014Article
- Persistence of non-viral vector mediated RPE65 expression: case for viability as a gene transfer therapy for RPE-based diseases.Journal of controlled release : official journal of the Controlled Release Society · 2013Article
- Receptor interacting protein kinase-mediated necrosis contributes to cone and rod photoreceptor degeneration in the retina lacking interphotoreceptor retinoid-binding protein.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2013Article
- Complementation test of Rpe65 knockout and tvrm148.Investigative ophthalmology & visual science · 2013Article
- RPE65 gene therapy slows cone loss in Rpe65-deficient dogs.Gene therapy · 2013Article
- New insights into retinoid metabolism and cycling within the retina.Progress in retinal and eye research · 2013Review
- Cultured Müller cells from mammals can synthesize and accumulate retinyl esters.Experimental eye research · 2012Article
- Metabolism of carotenoids and retinoids related to vision.The Journal of biological chemistry · 2012Review
- Gene therapy for leber congenital amaurosis caused by RPE65 mutations: safety and efficacy in 15 children and adults followed up to 3 years.Archives of ophthalmology (Chicago, Ill. : 1960) · 2012Article
- RPE65 is present in human green/red cones and promotes photopigment regeneration in an in vitro cone cell model.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2011Article
Corrections and comments
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Authors and funding
3 authors at 2 institutions in 1 country.
Funding
Abstract
As cone photoreceptors mediate vision in bright light, their photopigments are bleached at a rapid rate and require substantial recycling of the chromophore 11-cis-retinal (RAL) for continued function. The retinal pigment epithelium (RPE) supplies 11-cis-RAL to both rod and cone photoreceptors; however, stringent demands imposed by the function of cones in bright light exceed the output from this source. Recent evidence has suggested that cones may be able to satisfy this demand through privileged access to an additional source of chromophore located within the inner retina. In this study, we demonstrate that the protein RPE65, previously identified in RPE as the isomerohydrolase of the RPE-retinal visual cycle, is found within cones of the rod-dominant mouse retina, and the level of RPE65 in cones is inversely related to the level in the RPE. The light sensitivity of cone ERGs of BALB/c mice, which had an undetectable level of cone RPE65, was enhanced by approximately threefold with administration of exogenous chromophore, indicating that the cones of these animals are chromophore deficient. This enhancement with chromophore administration was not observed in C57BL/6 mice, whose cones contain RPE65. These results demonstrate that RPE65 within cones may be essential for the efficient regeneration of cone photopigments under bright-light conditions.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.