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https://www.readbyqxmd.com/read/28632489/the-effects-of-platelet-gel-on-cultured-human-retinal-pigment-epithelial-cells
#1
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
#2
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-reprograming-of-the-retina-into-rpe-requires-wnt-signalling-in-the-developing-chick-optic-cup
#3
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 proteins (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...
May 25, 2017: Biology Open
https://www.readbyqxmd.com/read/28500718/rpe65-and-the-accumulation-of-retinyl-esters-in-mouse-retinal-pigment-epithelium
#4
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
https://www.readbyqxmd.com/read/28476927/rational-tuning-of-visual-cycle-modulator-pharmacodynamics
#5
Philip D Kiser, Jianye Zhang, Mohsen Badiee, Junzo Kinoshita, Neal S Peachey, Gregory P Tochtrop, Krzysztof Palczewski
Modulators of the visual cycle have been developed for treatment of various retinal disorders. These agents were designed to inhibit retinoid isomerase (RPE65), the rate-limiting enzyme of the visual cycle, based on the idea that attenuation of visual pigment regeneration could reduce formation of toxic retinal conjugates. Certain of these agents that contain primary amine groups also can also reversibly form retinaldehyde-Schiff base adducts, which contributes to their retinal protective activity. Direct inhibition of RPE65 as a therapeutic strategy is complicated by adverse effects resulting from slowed chromophore regeneration whereas effective retinal sequestration can require high drug doses with potential off-target effects...
May 5, 2017: Journal of Pharmacology and Experimental Therapeutics
https://www.readbyqxmd.com/read/28474714/-gene-therapy-for-vision-restoration-in-patients-with-leber-congenital-amaurosis-lca-due-to-rpe65-gene-mutations-beginning-the-phase-iv-trial
#6
Óscar Francisco Chacón-Camacho, Juan Carlos Zenteno
This is a significant time moment in the field of gene therapy in humans. Recently, results from a phase III clinical trial were published, demonstrating the first gene therapy success for a genetic disease. A clinical trial was carried out in patients suffering a hereditary blindness disease named Leber congenital amaurosis, caused by mutations in the RPE65 gene. Participating subjects received a subretinal injection of the normal RPE65 gene and one year after exhibited a significant improvement in visual acuity...
March 2017: Gaceta Médica de México
https://www.readbyqxmd.com/read/28428265/inhibition-of-thyroid-hormone-receptor-locally-in-the-retina-is-a-therapeutic-strategy-for-retinal-degeneration
#7
Hongwei Ma, Fan Yang, Michael R Butler, Joshua Belcher, T Michael Redmond, Andrew T Placzek, Thomas S Scanlan, Xi-Qin Ding
Thyroid hormone (TH) signaling regulates cell proliferation, differentiation, and metabolism. Recent studies have implicated TH signaling in cone photoreceptor viability. Using mouse models of retinal degeneration, we demonstrated that antithyroid drug treatment and targeting iodothyronine deiodinases (DIOs) to suppress cellular tri-iodothyronine (T3) production or increase T3 degradation preserves cones. In this work, we investigated the effectiveness of inhibition of the TH receptor (TR). Two genes, THRA and THRB, encode TRs; THRB2 has been associated with cone viability...
April 20, 2017: FASEB Journal: Official Publication of the Federation of American Societies for Experimental Biology
https://www.readbyqxmd.com/read/28418497/course-of-sodium-iodate-induced-retinal-degeneration-in-albino-and-pigmented-mice
#8
Guy Chowers, Matan Cohen, Devora Marks-Ohana, Shelly Stika, Ayala Eijzenberg, Eyal Banin, Alexey Obolensky
Purpose: To characterize the course of sodium iodate (SI)-induced retinal degeneration in young adult albino and pigmented mice. Methods: Single intraperitoneal (IP) injections of SI (25, 50, and 100 mg/kg) were performed in 7- to 8-week-old BALB/c and C57Bl/6J mice. Retinal function and structure was assessed at baseline, 24 hours, 3 days, 1, 2, 3, and 4 weeks postinjection by optokinetic tracking response, ERG, optical coherence tomography (OCT), and histologic and immunohistochemical techniques...
April 1, 2017: Investigative Ophthalmology & Visual Science
https://www.readbyqxmd.com/read/28393863/genetic-and-phenotypic-characteristics-of-four-chinese-families-with-fundus-albipunctatus
#9
Guoxing Yang, Zhiqiang Liu, Shipeng Xie, Chengquan Li, Lina Lv, Minglian Zhang, Jialiang Zhao
Fundus albipunctatus (FA) is a rare autosomal recessive form of stationary night blindness characterized by the presence of white or white-yellow dots in the perimacular area and the periphery of the retina, with or without macular involvement. In this study, we examined four Chinese families with FA. Patients were given complete ophthalmic examinations, and blood samples were collected for DNA extraction. Three genes, RDH5, RLBP1 and RPE65, were screened by direct sequencing. Mutations in RDH5 were identified in three families and mutations in RPE65 were identified in one family...
April 10, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28393043/leber-s-congenital-amaurosis-and-the-role-of-gene-therapy-in-congenital-retinal-disorders
#10
REVIEW
Walid Sharif, Zuhair Sharif
Leber's congenital amaurosis (LCA) and recent gene therapy advancement for treating inherited retinopathies were extensive literature reviewed using MEDLINE, PubMed and EMBASE. Adeno-associated viral vectors were the most utilised vectors for ocular gene therapy. Cone photoreceptor cells might use an alternate pathway which was not reliant of the retinal pigment epithelium (RPE) derived retinoid isomerohydrolase (RPE65) to access the 11-cis retinal dehydechromophore. Research efforts dedicated on the progression of a gene-based therapy for the treatment of LCA2...
2017: International Journal of Ophthalmology
https://www.readbyqxmd.com/read/28356702/appropriately-differentiated-arpe-19-cells-regain-phenotype-and-gene-expression-profiles-similar-to-those-of-native-rpe-cells
#11
William Samuel, Cynthia Jaworski, Olga A Postnikova, R Krishnan Kutty, Todd Duncan, Li Xuan Tan, Eugenia Poliakov, Aparna Lakkaraju, T Michael Redmond
PURPOSE: The RPE cell line ARPE-19 provides a dependable and widely used alternative to native RPE. However, replication of the native RPE phenotype becomes more difficult because these cells lose their specialized phenotype after multiple passages. Compounding this problem is the widespread use of ARPE-19 cells in an undifferentiated state to attempt to model RPE functions. We wished to determine whether suitable culture conditions and differentiation could restore the RPE-appropriate expression of genes and proteins to ARPE-19, along with a functional and morphological phenotype resembling native RPE...
2017: Molecular Vision
https://www.readbyqxmd.com/read/28351499/retina-tissue-engineering-by-conjunctiva-mesenchymal-stem-cells-encapsulated-in-fibrin-gel-hypotheses-on-novel-approach-to-retinal-diseases-treatment
#12
Mostafa Soleimannejad, Somayeh Ebrahimi-Barough, Samad Nadri, Mohammad Riazi-Esfahani, Masoud Soleimani, Seyed Mohammad Tavangar, Jafar Ai
BACKGROUND: Retinitis pigmentosa (RP) and age related macular degeneration (AMD) are two retinal diseases that progress by photoreceptor cells death. In retinal transplantation studies, stem and progenitor cells inject into the sub retinal space or vitreous and then these cells can be migrate to the site of retinal degeneration and locate in the host retina and restitute vision. PRESENTATION OF THE HYPOTHESIS: Our hypothesis suggests that using human conjunctiva stem cells (as the source for increasing the number of human stem cells progenitor cells in retina dysfunction diseases) with fibrin gel and also assessing its relating in vitro (cellular and molecular processes) and in vivo (vision tests and pathology) could be a promising strategy for treatment of AMD and RP disorders...
April 2017: Medical Hypotheses
https://www.readbyqxmd.com/read/28339981/transcriptome-analysis-of-comb-and-testis-from-rose-comb-silky-chicken-r1-r1-and-beijing-fatty-wild-type-chicken-r-r
#13
Y Wang, J Li, C Feng, Y Zhao, X Hu, N Li
Rose-comb was one of the chicken comb-variants first used by Bateson and Punnet in 1902 to demonstrate Mendelian inheritance in animals. Rose-comb is a monogenic trait that has been widely described in chickens. It is caused by a large structural rearrangement that leads to mis-expression of transcription factor MNR2 on chromosome 7. Rose-comb has pleiotropic effects in homozygous roosters, which is associated with poor sperm mobility. It was postulated that this is caused by the disruption of the CCDC108 gene located at the distal inversion breakpoint...
June 1, 2017: Poultry Science
https://www.readbyqxmd.com/read/28336262/increased-susceptibility-to-fundus-camera-delivered-light-induced-retinal-degeneration-in-mice-deficient-in-oxidative-stress-response-proteins
#14
Yi Ding, Bogale Aredo, Xin Zhong, Cynthia X Zhao, Rafael L Ufret-Vincenty
Oxidative stress is an important contributor to the pathogenesis of many retinal diseases including age-related macular degeneration and retinal dystrophies. Light-induced retinal degeneration (LIRD) can serve as a model in which to study the response of the retina to stress. Of note, many genetic mutant mice are in a C57BL/6 J background and are thus resistant to the usual LIRD models. We recently developed a new model of fundus camera-delivered light-induced retinal degeneration (FCD-LIRD) which is effective in strains of mice expressing the light-resistant variant of RPE65 (450Met), including C57BL/6 J...
March 20, 2017: Experimental Eye Research
https://www.readbyqxmd.com/read/28300845/digoxin-induced-retinal-degeneration-depends-on-rhodopsin
#15
Britta Landfried, Marijana Samardzija, Maya Barben, Christian Schori, Katrin Klee, Federica Storti, Christian Grimm
Na,K-ATPases are energy consuming ion pumps that are required for maintaining ion homeostasis in most cells. In the retina, Na,K-ATPases are especially important to sustain the dark current in photoreceptor cells needed for rapid hyperpolarization of rods and cones in light. Cardiac glycosides like digoxin inhibit the activity of Na,K-ATPases by targeting their catalytic alpha subunits. This leads to a disturbed ion balance, which can affect cellular function and survival. Here we show that the treatment of wild-type mice with digoxin leads to severe retinal degeneration and loss of vision...
March 16, 2017: Cell Death & Disease
https://www.readbyqxmd.com/read/28298893/bis-retinoid-a2e-induces-an-increase-of-basic-fibroblast-growth-factor-via-inhibition-of-extracellular-signal-regulated-kinases-1-2-pathway-in-retinal-pigment-epithelium-cells-and-facilitates-phagocytosis
#16
Delphine Balmer, Linda Bapst-Wicht, Aswin Pyakurel, Martine Emery, Natacha Nanchen, Christian G Bochet, Raphael Roduit
Age-related macular degeneration (ARMD) is the leading cause of vision loss in developed countries. Hallmarks of the disease are well known; indeed, this pathology is characterized by lipofuscin accumulation, is principally composed of lipid-containing residues of lysosomal digestion. The N-retinyl-N-retinylidene ethanolamine (A2E) retinoid which is thought to be a cytotoxic component for RPE is the best-characterized component of lipofuscin so far. Even if no direct correlation between A2E spatial distribution and lipofuscin fluorescence has been established in aged human RPE, modified forms or metabolites of A2E could be involved in ARMD pathology...
2017: Frontiers in Aging Neuroscience
https://www.readbyqxmd.com/read/28286579/brain-xanthophyll-content-and-exploratory-gene-expression-analysis-subspecies-differences-in-rhesus-macaque
#17
Emily S Mohn, John W Erdman, Martha Neuringer, Matthew J Kuchan, Elizabeth J Johnson
BACKGROUND: The dietary xanthophylls, lutein and zeaxanthin, accumulate in primate retina and brain, and emerging evidence indicates neural lutein content may be beneficial for cognition. Neural xanthophyll content in primates varies greatly among individuals, and genetic factors are likely to be significant contributors. Subspecies of rhesus macaques originating from different geographic locations are known to differ genetically, but the effect of origin on gene expression and carotenoid status has not been determined...
2017: Genes & Nutrition
https://www.readbyqxmd.com/read/28282423/age-related-macular-degeneration-phenotypes-are-associated-with-increased-tumor-necrosis-alpha-and-subretinal-immune-cells-in-aged-cxcr5-knockout-mice
#18
Hu Huang, Ying Liu, Lei Wang, Wen Li
The role of chemokine receptor in age-related macular degeneration (AMD) remains elusive. The objective of this study is to investigate the role of chemokine receptor Cxcr5 in the pathogenesis of AMD. Cxcr5 gene expression levels (mRNA and protein) are higher in the retina and retinal pigment epithelium (RPE) of aged C57BL/6 wild type mice than younger ones. Vascular and glial cells express Cxcr5 and its ligand Cxcl13 in mouse retina. Aged Cxcr5 knockout (-/-) mice develop both early and late AMD-like pathological features...
2017: PloS One
https://www.readbyqxmd.com/read/28280483/outer-plexiform-layer-structures-are-not-altered-following-aav-mediated-gene-transfer-in-healthy-rat-retina
#19
Bert C Giers, Daniela Klein, Alexandra Mendes-Madeira, Carolina Isiegas, Birgit Lorenz, Silke Haverkamp, Knut Stieger
Ocular gene therapy approaches have been developed for a variety of different diseases. In particular, clinical gene therapy trials for RPE65 mutations, X-linked retinoschisis, and choroideremia have been conducted at different centers in recent years, showing that adeno-associated virus (AAV)-mediated gene therapy is safe, but limitations exist as to the therapeutic benefit and long-term duration of the treatment. The technique of vector delivery to retinal cells relies on subretinal injection of the vector solution, causing a transient retinal detachment...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28238097/igf-1-mediated-survival-from-induced-death-of-human-primary-cultured-retinal-pigment-epithelial-cells-is-mediated-by-an-akt-dependent-signaling-pathway
#20
Wenhua Zheng, Qian Meng, Haitao Wang, Fengxia Yan, Peter J Little, Xinguo Deng, Shaofen Lin
Degeneration of the human retinal pigmented epithelium (hRPE) is involved in several eye disorders such as age-related macular degeneration (AMD). In this study, we investigated the protective effect of IGF-1 on human primary cultured RPE cells and its underlying mechanism. IGF-1 dose- and time-dependently promoted the survival of RPE cells from serum deprivation. Western blot showed that IGF-1 stimulated the activation of the PI3K/Akt and MAPK pathways in hRPE. Inhibition of the PI3K/Akt pathway by the PI3K-specific inhibitor, LY294002 or inhibition of Akt by Akt-specific inhibitors Akt inhibitor VIII or SN-38, or downregulation Akt with siRNA specific for Akt blocked the effect of IGF-1 on hRPE...
February 25, 2017: Molecular Neurobiology
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