Keywords: Light induced retinal degeneration; Oxidative stress; RPE65; Met450; OCT volume; RPE morphology; Retina;
مقالات ISI (ترجمه نشده)
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Keywords: Retinal ageing; Cyclodextrin; RPE65; Retinal inflammation; Retinal amyloid beta; Lipid;
Keywords: Retinal stem cells; Retinal neurospheres; RPE65; LRAT; Bestrophin 1; Beta5-integrin; OA1; CRALBP; MITF
A novel RPE65 inhibitor CU239 suppresses visual cycle and prevents retinal degeneration
Keywords: RPE; retinal pigment epithelium; IPM; inter-photoreceptor matrix; 11-cis-RAL; 11-cis-retinaldehyde; all-trans-RAL; all-trans-retinaldehyde; ABCA4; ATP-cassette transporter 4; atRDH; all-trans-retinol dehydrogenases; all-trans-ROL; all-trans-retinol; IRBP;
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
Keywords: Microglia; Macrophage; Dendritic cell; Neurodegeneration; Retina; RPE65; Photoreceptors;
Span poly-L-arginine nanoparticles are efficient non-viral vectors for PRPF31 gene delivery: An approach of gene therapy to treat retinitis pigmentosa
Keywords: Nanoparticle; Span; Gene therapy; Ophthalmology; Retinitis pigmentosa; AAV; adeno-associated viral; AAV2; adeno-associated viral vectors serotype 2; adRP; autosomal dominant retinitis pigmentosa; ANOVA; analysis of variance; c/d; cycles/degree; DAPI; 4â²
Bardet-Biedl syndrome: A model for translational research in rare diseases
Keywords: AAV; adeno-associated vector; ADPKD; autosomal dominant polycystic kidney disease; BBS; Bardet-Biedl Syndrome; CDE; common data element; CRIBBS; Clinical Registry Investigating Bardet-Biedl Syndrome; DDAVP; desmopressin acetate; GRDR®; Global Rare Di
Using patient-specific induced pluripotent stem cells to interrogate the pathogenicity of a novel retinal pigment epithelium-specific 65Â kDa cryptic splice site mutation and confirm eligibility for enrollment into a clinical gene augmentation trial
Keywords: Best1; Bestrophin1; bFGF; basic fibroblast growth factor; DMEM; Dulbecco's Modified Eagle Medium; DNMT; DNA methyltransferases; ERG; Electroretinography; KLF4; Kruppel-like factor 4; LB; Lysogeny broth; LCA; Leber congenital amaurosis; MEM; minimum essent
A Phe-rich region in short-wavelength sensitive opsins is responsible for their aggregation in the absence of 11-cis-retinal
Keywords: Opsin aggregation; Short-wavelength sensitive opsins (SWS); Medium/long-wavelength sensitive opsins (M/LWS); Leber congenital amaurosis (LCA); Cone degeneration; G-protein-coupled receptors (GPCRs); RPE65; LRAT;
Persistence of non-viral vector mediated RPE65 expression: Case for viability as a gene transfer therapy for RPE-based diseases
Keywords: RPE65; Non-viral gene therapy; Retinal pigment epithelium; DNA nanoparticle; S/MAR;
Flow cytometry assessment of the purity of human retinal pigment epithelial primary cell cultures
Keywords: Flow cytometry; hRPE; Purity; RPE65; Epithelial and fibroblast proteins
A reduced zinc diet or zinc transporter 3 knockout attenuate light induced zinc accumulation and retinal degeneration
Keywords: rat; mouse; rhodopsin; RPE65; ZnT3; photoreceptorsTPEN, d-N,N,N'N′-tetrakis(-)[2-pyridylmethyl]-ethylenediamine; GCL, ganglion cell layer; LD, light damage; INL, inner nuclear layer; ONL, outer nuclear layer; IPL, inner plexiform layer; OPL, outer plexifo
A review of therapeutic prospects of non-viral gene therapy in the retinal pigment epithelium
Keywords: RPE65; Non-viral gene therapy; Retinal pigment epithelium; DNA nanoparticle
Structural basis of carotenoid cleavage: From bacteria to mammals
Keywords: Carotenoid oxygenase; Carotenoid; Apocarotenoid; ACO; VP14; RPE65;
Mammalian Carotenoid-oxygenases: Key players for carotenoid function and homeostasis
Keywords: C/EBPα; CCAAT/enhancer-binding protein α; BCMO1; β,β-carotene-15,15â²-monooxygenase 1; BCDO2; β,β-carotene-9,10-dioxygenase 2; CCE; carotenoid cleaving enzyme; CD36 (SCARB3); Cluster of Differentiation 36; FABP4 (aP2); fatty acid-binding protein 4;
Key enzymes of the retinoid (visual) cycle in vertebrate retina
Keywords: RPE65; Retinol dehydrogenase; Visual cycle; Retinoid cycle; Retinal degeneration; Lecithin:retinol acyltransferase;
S-opsin protein is incompletely modified during N-glycan processing in Rpe65−/− mice
Keywords: RPE65; 11-cis-retinal; S-opsin; N-glycosylation; degradation
Membrane-binding and enzymatic properties of RPE65
Keywords: Retinoid isomerase; RPE65; Membrane protein; Metalloenzyme; Retinal pigment epithelium; Leber congenital amaurosisACO, Apocarotenoid oxygenase; CCO, Carotenoid cleavage oxygenase; CHAPS, 3-[(3-cholamidopropyl)-dimethylammonio]-1-propane sulfonate; CRALBP,
Leber congenital amaurosis due to RPE65 mutations and its treatment with gene therapy
Keywords: Rod and cone photoreceptors; RPE65; Visual (retinoid) cycle; Retinal degeneration; Electroretinogram (ERG); Retinal pigment epithelium (RPE); Optical coherence tomography (OCT); Transient pupillary light reflex (TPLR); Cortical plasticity; Perimetry; Func
Elucidating the phenomenon of HESC-derived RPE: Anatomy of cell genesis, expansion and retinal transplantation
Keywords: Age-related macular degeneration; Human embryonic stem cells; Retinal Pigment Epithelium; OTX2; Pax6; PMEL17; Cytokeratin 8; RPE65; RCS dystrophic rat; Phagocytosis
Neural retinal regeneration in the anuran amphibian Xenopus laevis post-metamorphosis: Transdifferentiation of retinal pigmented epithelium regenerates the neural retina
Keywords: Regeneration; Retina; Lens; Xenopus laevis; Organ culture; RPE65; Pax6; BrdU
Organization, structure and activity of proteins in monolayers
Keywords: Protein; Membrane protein; Peripheral protein; Phospholipase; Monolayer; Air-water interface; Recoverin; RPE65; Rhodopsin; Bacteriorhodopsin; Gramicidin;
Impacts of two point mutations of RPE65 from Leber’s congenital amaurosis on the stability, subcellular localization and isomerohydrolase activity of RPE65
Keywords: CHX, cycloheximide; LCA, Leber’s congenital amaurosis; LRAT, lecithin retinol acyltransferase; MOI, multiplicity of infection; RPE, retinal pigment epithelium; ER, endoplasmic reticulumLRAT; Retinoid; Retinal degeneration; RPE; RPE65; Visual cycle
Structure prediction of the RPE65 protein
Keywords: RPE65; Protein structure; Protein structure prediction; Retina; Visual cycle
The level of thymic expression of RPE65 inversely correlates with its capacity to induce experimental autoimmune uveitis (EAU) in different rodent strains
Keywords: RPE65; autoimmunity; experimental autoimmune uveitis; immunopathology; immunotolerance; thymus; mouse strains
Amniotic membrane maintains the phenotype of rabbit retinal pigment epithelial cells in culture
Keywords: age-related macular degeneration; amniotic membrane; basement membrane; differentiation; epithelial cells; eye; in vitro; pigmentation; rabbit; retinal pigment epithelium; RPE65; tight junctions; transepithelial resistance;
Towards a better understanding of carotenoid metabolism in animals
Keywords: Vitamin A; Carotenoid metabolism in animal; bco; bco2; rpe65; Retinoic acid signaling;
Molecular mechanisms of light-induced photoreceptor apoptosis and neuroprotection for retinal degeneration
Keywords: A2E; N-retinyl-N-retinylidene ethanolamine; AA; arachidonic acid; AAV; adeno-associated virus; ABCR; ATP-binding cassette transporter retina; AIF; apoptosis inducing factor; AMD; age-related macular degeneration; AP-1; activator protein-1; AP24; Mr 24; 00