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https://www.readbyqxmd.com/read/28985805/antigen-specificity-of-antiretinal-antibodies-in-patients-with-noninfectious-uveitis
#1
Ebrima Gibbs, Joanne Matsubara, Sijia Cao, Jing Cui, Farzin Forooghian
OBJECTIVE: Antiretinal antibodies (ARAs) have previously been described in noninfectious uveitis. However, the antigen specificity of these ARAs has not been investigated. The purpose of this study was to identify antigen-specific ARAs in noninfectious uveitis. METHODS: A total of 18 patients with noninfectious uveitis were enrolled. Surface plasmon resonance was used to measure binding responses of patient and control sera against several uveitogenic proteins: recoverin, S-antigen, interphotoreceptor retinoid binding (IRBP), retinal-pigment-epithelium-specific 65-kDa protein (RPE65), tyrosinase-related protein 1 (TRYP1), and tyrosinase-related protein 2 (TRYP2)...
October 2017: Canadian Journal of Ophthalmology. Journal Canadien D'ophtalmologie
https://www.readbyqxmd.com/read/28973952/rpe65-takes-on-another-role-in-the-vertebrate-retina
#2
T Michael Redmond
No abstract text is available yet for this article.
October 2, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28962184/bioinformatic-analysis-of-retinal-gene-function-and-expression-in-diabetic-rats
#3
Wenjuan Zhao, Dong Wang, Jun Zhao, Wenqing Zhao
The aim of the present study was to investigate the changes in retinal gene expression at three time points and assess the underlying molecular mechanisms of diabetic retinopathy (DR) in a streptozotocin (STZ)-induced diabetes rat model using bioinformatics analysis. The gene expression profile of GSE28831 was extracted from the Gene Expression Omnibus database and differentially expressed genes (DEGs) were identified at three different time points (1, 4 and 12 weeks) using the limma package in R language. Gene ontology (GO) enrichment analysis of DEGs was performed followed by a principal component and pathway enrichment analysis of the selected DEGs along with protein-protein interaction network construction at the three time points...
September 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/28955745/bioengineered-bruch-s-like-extracellular-matrix-promotes-retinal-pigment-epithelial-differentiation
#4
Samuel McLenachan, Erwei Hao, Dan Zhang, Ling Zhang, Michael Edel, Fred Chen
In the eye, the retinal pigment epithelium (RPE) adheres to a complex protein matrix known as Bruch's membrane (BrM). The aim of this study was to provide enriched conditions for RPE cell culture through the production of a BrM-like matrix. Our hypothesis was that a human RPE cell line would deposit an extracellular matrix (ECM) resembling BrM. The composition and structure of ECM deposited by ARPE19 cells (ARPE19-ECM) was characterized. To produce ARPE19-ECM, ARPE19 cells were cultured in the presence dextran sulphate...
July 2017: Biochemistry and Biophysics Reports
https://www.readbyqxmd.com/read/28945494/identification-of-the-genetic-determinants-responsible-for-retinal-degeneration-in-families-of-mexican-descent
#5
Adda Villanueva, Pooja Biswas, Kameron Kishaba, John Suk, Keerti Tadimeti, Pongali B Raghavendra, Karine Nadeau, Bruno Lamontagne, Lambert Busque, Steve Geoffroy, Ian Mongrain, Géraldine Asselin, Sylvie Provost, Marie-Pierre Dubé, Eric Nudleman, Radha Ayyagari
PURPOSE: To investigate the clinical characteristics and genetic basis of inherited retinal degeneration (IRD) in six unrelated pedigrees from Mexico. METHODS: A complete ophthalmic evaluation including measurement of visual acuities, Goldman kinetic or Humphrey dynamic perimetry, Amsler test, fundus photography, and color vision testing was performed. Family history and blood samples were collected from available family members. DNA from members of two pedigrees was examined for known mutations using the APEX ARRP genotyping microarray and one pedigree using the APEX LCA genotyping microarray...
September 25, 2017: Ophthalmic Genetics
https://www.readbyqxmd.com/read/28943836/evaluating-efficiencies-of-dual-aav-approaches-for-retinal-targeting
#6
Livia S Carvalho, Heikki T Turunen, Sarah J Wassmer, María V Luna-Velez, Ru Xiao, Jean Bennett, Luk H Vandenberghe
Retinal gene therapy has come a long way in the last few decades and the development and improvement of new gene delivery technologies has been exponential. The recent promising results from the first clinical trials for inherited retinal degeneration due to mutations in RPE65 have provided a major breakthrough in the field and have helped cement the use of recombinant adeno-associated viruses (AAV) as the major tool for retinal gene supplementation. One of the key problems of AAV however, is its limited capacity for packaging genomic information to a maximum of around 4...
2017: Frontiers in Neuroscience
https://www.readbyqxmd.com/read/28889993/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
#7
Peter H Tang, Mark J Pierson, Neal D Heuss, Dale S Gregerson
BACKGROUND: Studies of antigen presentation in retina using mice that expressed green fluorescent protein (GFP) from a transgenic CD11c promoter found that retinal GFP(hi) cells possessed antigen presentation function. Subsequent studies found that these high GFP(hi) cells preferentially localized to sites of retinal injury, consistent with their APC function. Interest in the roles of macrophages in degenerative CNS diseases led us to study the GFP(hi) cells in a retinal model of neurodegeneration...
September 8, 2017: Molecular and Cellular Neurosciences
https://www.readbyqxmd.com/read/28880021/long-term-expression-of-melanopsin-and-channelrhodopsin-causes-no-gross-alterations-in-the-dystrophic-dog-retina
#8
B Ameline, K-T Tshilenge, M Weber, M Biget, L Libeau, R Caplette, A Mendes-Madeira, N Provost, C Guihal, S Picaud, P Moullier, V Pichard, T Cronin, C Isiegas
Several preclinical studies have investigated the potential of algal channelrhodopsin and human melanopsin as optogenetic tools for vision restoration. In the present study, we assessed the potentially deleterious effects of long-term expression of these optogenes on the diseased retina in a large animal model of retinal degeneration, the RPE65-deficient Briard dog model of Leber congenital amaurosis. Intravitreal injection of adeno-associated virus vectors expressing channelrhodopsin and melanopsin had no effect on retinal thickness over a 16-month period post injection...
September 7, 2017: Gene Therapy
https://www.readbyqxmd.com/read/28874556/rpe65-has-an-additional-function-as-the-lutein-to-meso-zeaxanthin-isomerase-in-the-vertebrate-eye
#9
Rajalekshmy Shyam, Aruna Gorusupudi, Kelly Nelson, Martin P Horvath, Paul S Bernstein
Carotenoids are plant-derived pigment molecules that vertebrates cannot synthesize de novo that protect the fovea of the primate retina from oxidative stress and light damage. meso-Zeaxanthin is an ocular-specific carotenoid for which there are no common dietary sources. It is one of the three major carotenoids present at the foveal center, but the mechanism by which it is produced in the eye is unknown. An isomerase enzyme is thought to be responsible for the transformation of lutein to meso-zeaxanthin by a double-bond shift mechanism, but its identity has been elusive...
September 5, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28754419/evaluation-of-tolerance-to-lentiviral-lv-rpe65-gene-therapy-vector-after-subretinal-delivery-in-non-human-primates
#10
Alexandre Matet, Corinne Kostic, Alexis-Pierre Bemelmans, Alexandre Moulin, Serge G Rosolen, Samia Martin, Fulvio Mavilio, Vazrik Amirjanians, Knut Stieger, Birgit Lorenz, Francine Behar-Cohen, Yvan Arsenijevic
Several approaches have been developed for gene therapy in RPE65-related Leber congenital amaurosis. To date, strategies that have reached the clinical stages rely on adeno-associated viral vectors and two of them documented limited long-term effect. We have developed a lentiviral-based strategy of RPE65 gene transfer that efficiently restored protein expression and cone function in RPE65-deficient mice. In this study, we evaluated the ocular and systemic tolerances of this lentiviral-based therapy (LV-RPE65) on healthy nonhuman primates (NHPs), without adjuvant systemic anti-inflammatory prophylaxis...
October 2017: Translational Research: the Journal of Laboratory and Clinical Medicine
https://www.readbyqxmd.com/read/28712537/efficacy-and-safety-of-voretigene-neparvovec-aav2-hrpe65v2-in-patients-with-rpe65-mediated-inherited-retinal-dystrophy-a-randomised-controlled-open-label-phase-3-trial
#11
Stephen Russell, Jean Bennett, Jennifer A Wellman, Daniel C Chung, Zi-Fan Yu, Amy Tillman, Janet Wittes, Julie Pappas, Okan Elci, Sarah McCague, Dominique Cross, Kathleen A Marshall, Jean Walshire, Taylor L Kehoe, Hannah Reichert, Maria Davis, Leslie Raffini, Lindsey A George, F Parker Hudson, Laura Dingfield, Xiaosong Zhu, Julia A Haller, Elliott H Sohn, Vinit B Mahajan, Wanda Pfeifer, Michelle Weckmann, Chris Johnson, Dina Gewaily, Arlene Drack, Edwin Stone, Katie Wachtel, Francesca Simonelli, Bart P Leroy, J Fraser Wright, Katherine A High, Albert M Maguire
BACKGROUND: Phase 1 studies have shown potential benefit of gene replacement in RPE65-mediated inherited retinal dystrophy. This phase 3 study assessed the efficacy and safety of voretigene neparvovec in participants whose inherited retinal dystrophy would otherwise progress to complete blindness. METHODS: In this open-label, randomised, controlled phase 3 trial done at two sites in the USA, individuals aged 3 years or older with, in each eye, best corrected visual acuity of 20/60 or worse, or visual field less than 20 degrees in any meridian, or both, with confirmed genetic diagnosis of biallelic RPE65 mutations, sufficient viable retina, and ability to perform standardised multi-luminance mobility testing (MLMT) within the luminance range evaluated, were eligible...
July 13, 2017: Lancet
https://www.readbyqxmd.com/read/28712536/gene-therapy-for-rpe65-mediated-inherited-retinal-dystrophy-completes-phase-3
#12
Helena Lee, Andrew Lotery
No abstract text is available yet for this article.
July 13, 2017: Lancet
https://www.readbyqxmd.com/read/28697496/spatially-resolved-spectral-sensitivities-as-a-potential-read-out-parameter-in-clinical-gene-therapeutic-trials
#13
Birgit Lorenz, Erika Wegscheider, Christian Hamel, Markus N Preising, Knut Stieger
PURPOSE: Spatially resolved functional assessment of rods and cones under photopic and scotopic conditions is desirable to evaluate the treatment outcome of gene therapeutic applications in inherited retinal disorders, such as early- onset severe retinal dystrophy (EOSRD) or achromatopsia. METHODS: A sample of 3 healthy subjects, 6 patients with RPE65 deficiency (aged 11-45 years), and 3 patients with cone dysfunction disorders underwent spectral sensitivity testing (SST) under conditions of dark and light adaptation using a Humphrey Field Analyzer modified perimeter...
July 12, 2017: Ophthalmic Research
https://www.readbyqxmd.com/read/28691584/fibrin-gel-as-a-scaffold-for-photoreceptor-cells-differentiation-from-conjunctiva-mesenchymal-stem-cells-in-retina-tissue-engineering
#14
Mostafa Soleimannejad, Somayeh Ebrahimi-Barough, Masoud Soleimani, Samad Nadri, Seyed Mohammad Tavangar, Ramak Roohipoor, Meysam Yazdankhah, Neda Bayat, Mohammad Riazi-Esfahani, Jafar Ai
Stem cell-based therapies are attraction approaches for regenerative medicine for treating retinal diseases. One of the limitations in cell therapy is cell death following post-injection whit preventing functional integration with retinal tissue. Fibrin gel, a bio-polymeric material with excellent biocompatibility, provides numerous advantages as a tissue engineering scaffold and a stem cell carrier. Therefore, current research is focusing on developing fibrin hydrogel scaffolds to protect stem cells during delivery and to stimulate endogenous regeneration through interactions of transplanted stem cells and retinal tissue...
July 10, 2017: Artificial Cells, Nanomedicine, and Biotechnology
https://www.readbyqxmd.com/read/28672005/fatty-acid-transport-protein-1-regulates-retinoid-metabolism-and-photoreceptor-development-in-mouse-retina
#15
Aurélie Cubizolle, Laurent Guillou, Bertrand Mollereau, Christian P Hamel, Philippe Brabet
In retinal pigment epithelium (RPE), RPE65 catalyzes the isomerization of all-trans-retinyl fatty acid esters to 11-cis-retinol in the visual cycle and controls the rhodopsin regeneration rate. However, the mechanisms by which these processes are regulated are still unclear. Fatty Acid Transport Protein 1 (FATP1) is involved in fatty acid uptake and lipid metabolism in a variety of cell types. FATP1 co-localizes with RPE65 in RPE and inhibits its isomerase activity in vitro. Here, we further investigated the role of FATP1 in the visual cycle using transgenic mice that overexpress human FATP1 specifically in the RPE (hFATP1TG mice)...
2017: PloS One
https://www.readbyqxmd.com/read/28662231/early-onset-progressive-degeneration-of-the-area-centralis-in-rpe65-deficient-dogs
#16
Freya M Mowat, Kristen J Gervais, Laurence M Occelli, Matthew J Annear, Janice Querubin, James W Bainbridge, Alexander J Smith, Robin R Ali, Simon M Petersen-Jones
Purpose: Retinal epithelium-specific protein 65 kDa (RPE65)-deficient dogs are a valuable large animal model species that have been used to refine gene augmentation therapy for Leber congenital amaurosis type-2 (LCA2). Previous studies have suggested that retinal degeneration in the dog model is slower than that observed in humans. However, the area centralis of the dog retina is a cone and rod photoreceptor rich region comparable to the human macula, and the effect of RPE65 deficiency specifically on this retinal region, important for high acuity vision, has not previously been reported...
June 1, 2017: Investigative Ophthalmology & Visual Science
https://www.readbyqxmd.com/read/28632489/the-effects-of-platelet-gel-on-cultured-human-retinal-pigment-epithelial-cells
#17
Sahar Balagholi, Shaban Alizadeh, Abouzar Bagheri, Yashar Amizadeh, Mozhgan Rezaei Kanavi
The positive role of platelet gel (PG) in tissue regeneration is well known, however, other characteristics of PG still remain to be determined. We investigated cellular and molecular changes in cultured human retinal pigment epithelial (hRPE) cells when treated with different concentrations of PG named PG1, PG2, and PG3. hRPE cells were isolated from donor eyes of two newborn children, within 24 hours after their death. The cells were treated with three concentrations of PG for 7 days: 3 × 104/ml (PG1), 6 × 104/ml (PG2), and 9 × 104/ml (PG3)...
June 20, 2017: Bosnian Journal of Basic Medical Sciences
https://www.readbyqxmd.com/read/28624218/targeted-multifunctional-lipid-eco-plasmid-dna-nanoparticles-as-efficient-non-viral-gene-therapy-for-leber-s-congenital-amaurosis
#18
Da Sun, Bhubanananda Sahu, Songqi Gao, Rebecca M Schur, Amita M Vaidya, Akiko Maeda, Krzysztof Palczewski, Zheng-Rong Lu
Development of a gene delivery system with high efficiency and a good safety profile is essential for successful gene therapy. Here we developed a targeted non-viral delivery system using a multifunctional lipid ECO for treating Leber's congenital amaurosis type 2 (LCA2) and tested this in a mouse model. ECO formed stable nanoparticles with plasmid DNA (pDNA) at a low amine to phosphate (N/P) ratio and mediated high gene transfection efficiency in ARPE-19 cells because of their intrinsic properties of pH-sensitive amphiphilic endosomal escape and reductive cytosolic release (PERC)...
June 16, 2017: Molecular Therapy. Nucleic Acids
https://www.readbyqxmd.com/read/28546339/bmp-induced-reprogramming-of-the-neural-retina-into-retinal-pigment-epithelium-requires-wnt-signalling
#19
Jörg Steinfeld, Ichie Steinfeld, Alexander Bausch, Nicola Coronato, Meggi-Lee Hampel, Heike Depner, Paul G Layer, Astrid Vogel-Höpker
In vertebrates, the retinal pigment epithelium (RPE) and photoreceptors of the neural retina (NR) comprise a functional unit required for vision. During vertebrate eye development, a conversion of the RPE into NR can be induced by growth factors in vivo at optic cup stages, but the reverse process, the conversion of NR tissue into RPE, has not been reported. Here, we show that bone morphogenetic protein (BMP) signalling can reprogram the NR into RPE at optic cup stages in chick. Shortly after BMP application, expression of Microphthalmia-associated transcription factor (Mitf) is induced in the NR and selective cell death on the basal side of the NR induces an RPE-like morphology...
July 15, 2017: Biology Open
https://www.readbyqxmd.com/read/28500718/rpe65-and-the-accumulation-of-retinyl-esters-in-mouse-retinal-pigment-epithelium
#20
Colleen Sheridan, Nicholas P Boyer, Rosalie K Crouch, Yiannis Koutalos
The RPE65 protein of the retinal pigment epithelium (RPE) enables the conversion of retinyl esters to the visual pigment chromophore 11-cis retinal. Fresh 11-cis retinal is generated from retinyl esters following photoisomerization of the visual pigment chromophore to all-trans during light detection. Large amounts of esters accumulate in Rpe65(-/-) mice, indicating their continuous formation when 11-cis retinal generation is blocked. We hypothesized that absence of light, by limiting the conversion of esters to 11-cis retinal, would also result in the build-up of retinyl esters in the RPE of wild-type mice...
May 2017: Photochemistry and Photobiology
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