Research TopicsGenomes and Genes
| Rpe65SummaryGene Symbol: Rpe65 Description: RPE65, retinoid isomerohydrolase Alias: retinoid isomerohydrolase, all-trans-retinyl-palmitate hydrolase, meso-zeaxanthin isomerase, retinal pigment epithelium 65, retinal pigment epithelium 65-like, retinal pigment epithelium-specific 65 kDa protein, retinal pigment epithelium-specific protein 65, retinol isomerase Species: rat Products: Rpe65 Top Publications
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- Beatrice J, Wenzel A, REme C, Grimm C. Increased light damage susceptibility at night does not correlate with RPE65 levels and rhodopsin regeneration in rats. Exp Eye Res. 2003;76:695-700 pubmed..The rate of rhodopsin regeneration is at least partly controlled by RPE65, a protein expressed in the retinal pigment epithelium...
- Bowne S, Humphries M, Sullivan L, Kenna P, Tam L, Kiang A, et al. A dominant mutation in RPE65 identified by whole-exome sequencing causes retinitis pigmentosa with choroidal involvement. Eur J Hum Genet. 2011;19:1074-81 pubmed publisher..8 Mb region on 1p31. Of 50 known genes in the region, 11 candidates, including RPE65 and PDE4B, were sequenced using di-deoxy capillary electrophoresis...
- Van Hooser J, Liang Y, Maeda T, Kuksa V, Jang G, He Y, et al. Recovery of visual functions in a mouse model of Leber congenital amaurosis. J Biol Chem. 2002;277:19173-82 pubmed..For sustained vision the 11-cis-chromophore must be regenerated from all-trans-retinal. This requires RPE65, a dominant retinal pigment epithelium protein...
- Kiser P, Golczak M, Maeda A, Palczewski K. Key enzymes of the retinoid (visual) cycle in vertebrate retina. Biochim Biophys Acta. 2012;1821:137-51 pubmed publisher..This article is part of a Special Issue entitled: Retinoid and Lipid Metabolism. ..
- Wang N, Tosi J, Kasanuki J, Chou C, Kong J, Parmalee N, et al. Transplantation of reprogrammed embryonic stem cells improves visual function in a mouse model for retinitis pigmentosa. Transplantation. 2010;89:911-9 pubmed publisher..pigment epithelial (RPE) cells in vitro and then restore retinal function in a model for retinitis pigmentosa: Rpe65/Rpe65 C57BL6 mice...
- Bridges C. Distribution of retinol isomerase in vertebrate eyes and its emergence during retinal development. Vision Res. 1989;29:1711-7 pubmed..Therefore, the expression in the RPE of retinol isomerase, which is essential for the formation of rhodopsin in the developing photoreceptors, is coordinated with the ..
- Chen Y, Moiseyev G, Takahashi Y, Ma J. RPE65 gene delivery restores isomerohydrolase activity and prevents early cone loss in Rpe65-/- mice. Invest Ophthalmol Vis Sci. 2006;47:1177-84 pubmedRecent in vitro evidence has shown that RPE65 is the isomerohydrolase that converts all-trans retinyl ester to 11-cis retinal, the chromophore for visual pigments in vertebrates...
- Wright A, Chakarova C, Abd El Aziz M, Bhattacharya S. Photoreceptor degeneration: genetic and mechanistic dissection of a complex trait. Nat Rev Genet. 2010;11:273-84 pubmed publisher..Although genetic and mechanistic diversity creates challenges for therapy, some approaches--particularly gene-replacement therapy--are showing considerable promise. ..
- Tanito M, Kaidzu S, Anderson R. Delayed loss of cone and remaining rod photoreceptor cells due to impairment of choroidal circulation after acute light exposure in rats. Invest Ophthalmol Vis Sci. 2007;48:1864-72 pubmed..hours and 7 days after light exposure, and ERG amplitude, ONL and choroidal thickness and area, and rhodopsin and RPE65 expression were lower 7 or more days after light exposure than in phenyl-N-tert-butylnitrone (PBN)-injected ..
- Mandal M, Moiseyev G, Elliott M, Kasus Jacobi A, Li X, Chen H, et al. Alpha-phenyl-N-tert-butylnitrone (PBN) prevents light-induced degeneration of the retina by inhibiting RPE65 protein isomerohydrolase activity. J Biol Chem. 2011;286:32491-501 pubmed publisher..PBN-mediated retinal protection probably occurs by slowing down the rate of rhodopsin regeneration by inhibiting RPE65 activity. PBN (50 mg/kg) protected albino Sprague-Dawley rat retinas when injected 0...
- Nakamura H, Yamaki K, Kondo I, Sakuragi S. Experimental autoimmune uveitis induced by immunization with retinal pigment epithelium-specific 65-kDa protein peptides. Curr Eye Res. 2005;30:673-80 pubmedTo investigate the pathogenic potential and sites of retinal pigment epithelium-specific 65-kDa protein (RPE65) for inducing experimental autoimmune uveitis (EAU) in Lewis rats...
- Kirwin S, Kanaly S, Hansen C, Cairns B, Ren M, Edelman J. Retinal gene expression and visually evoked behavior in diabetic long evans rats. Invest Ophthalmol Vis Sci. 2011;52:7654-63 pubmed publisher..including lecithin/retinol acyltransferase (LRAT), retinal pigment epithelium (RPE)-specific protein 65 kDa (RPE65), and RPE retinal G protein-coupled receptor (RGR)...
- Samardzija M, von Lintig J, Tanimoto N, Oberhauser V, Thiersch M, Reme C, et al. R91W mutation in Rpe65 leads to milder early-onset retinal dystrophy due to the generation of low levels of 11-cis-retinal. Hum Mol Genet. 2008;17:281-92 pubmedb>RPE65 is a retinal pigment epithelial protein essential for the regeneration of 11-cis-retinal, the chromophore of cone and rod visual pigments...
- Wenzel A, von Lintig J, Oberhauser V, Tanimoto N, Grimm C, Seeliger M. RPE65 is essential for the function of cone photoreceptors in NRL-deficient mice. Invest Ophthalmol Vis Sci. 2007;48:534-42 pubmed..In addition, these mice were bred to retinal pigment epithelial protein 65 (Rpe65(-/-))-deficient mice to study the role of RPE65...
- Bridges C, Alvarez R. The visual cycle operates via an isomerase acting on all-trans retinol in the pigment epithelium. Science. 1987;236:1678-80 pubmed..chromatography have now been used to demonstrate the existence of an eye-specific, membrane-bound enzyme (retinol isomerase) that converts all-trans to 11-cis retinol in the dark...
- Kondo H, Qin M, Mizota A, Kondo M, Hayashi H, Hayashi K, et al. A homozygosity-based search for mutations in patients with autosomal recessive retinitis pigmentosa, using microsatellite markers. Invest Ophthalmol Vis Sci. 2004;45:4433-9 pubmed..1145T-->C, F382S) and CNGB1 (c.3444 + 1G-->A). The other was a mutation in RPE65 (c.1543C-->T, R515W), which is known to cause Leber's congenital amaurosis...
- Shyam R, Gorusupudi A, Nelson K, Horvath M, Bernstein P. RPE65 has an additional function as the lutein to meso-zeaxanthin isomerase in the vertebrate eye. Proc Natl Acad Sci U S A. 2017;114:10882-10887 pubmed publisher..In the present study, we show that RPE65, the isomerohydrolase enzyme of the vertebrate visual cycle that catalyzes the isomerization of all-trans-..