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https://www.readbyqxmd.com/read/29574166/stem-cells-from-apical-papilla-promote-differentiation-of-human-pluripotent-stem-cells-towards-retinal-cells
#1
Fereshteh Karamali, Mohammad-Hossein Nasr Esfahani, Sara Taleahmad, Leila Satarian, Hossein Baharvand
Recently, we have found that human stem cells from apical papilla (SCAP) show a stromal cell-derived inducing activity (SDIA). To examine SDIA competence for retinal cells differentiation, we co-cultured SCAP with human pluripotent stem cells (hPSCs). In comparison with Matrigel-cultured hPSCs, SCAP significantly induces hPSCs to differentiate into rostral neural cells as demonstrated by upregulation of OTX2 and PAX6 and down-regulation of EN1, HOXB4 and HOXC8. Furthermore, the differentiated cells on SCAP significantly expressed eye-field markers, RAX, PAX6, LHX2 and SIX3 and showed five folds pigmented colonies...
March 2, 2018: Differentiation; Research in Biological Diversity
https://www.readbyqxmd.com/read/29570997/gene-therapy-for-retinal-degeneration
#2
Rajendra S Apte
Biallelic mutations in the RPE65 gene are associated with inherited retinal degenerations/dystrophies (IRD) and disrupt the visual cycle, leading to loss of vision. A new adenoviral vector-based gene therapy surgically delivered to retinal cells provides normal human RPE65 protein that can restore the visual cycle and some vision. To view this Bench to Bedside, open or download the PDF.
March 22, 2018: Cell
https://www.readbyqxmd.com/read/29569173/ultrastructural-changes-and-expression-of-pcna-and-rpe65-in-sodium-iodate-induced-acute-retinal-pigment-epithelium-degeneration-model
#3
Hong-Lim Kim, Sung Min Nam, Byung-Joon Chang, Sang-Soep Nahm, Jong-Hwan Lee
Alteration in retinal pigment epithelium (RPE) results in the visual dysfunction and blindness of retinal degenerative diseases. Injection of sodium iodate (NaIO3 ) generates degeneration of RPE. We analyzed the sequential ultrastructure and expression of proliferating cell nuclear antigen (PCNA) and retina-specific RPE65 in NaIO3 -induced retinal degeneration model. Adult male rats were injected 1% NaIO3 (50 mg/kg) and eyes were enucleated at 1, 3, 5, 7 and 14 days post-injection (DPI), fixed, and processed for histological analysis...
March 22, 2018: Neurochemical Research
https://www.readbyqxmd.com/read/29564403/prospect-of-retinal-gene-therapy-following-commercialization-of-voretigene-neparvovec-rzyl-for-retinal-dystrophy-mediated-by-rpe65-mutation
#4
EDITORIAL
Hossein Ameri
No abstract text is available yet for this article.
March 2018: Journal of Current Ophthalmology
https://www.readbyqxmd.com/read/29542350/pharmacotherapy-of-retinal-disease-with-visual-cycle-modulators
#5
Rehan M Hussain, Ninel Z Gregori, Thomas A Ciulla, Byron L Lam
Pharmacotherapy with visual cycle modulators (VCMs) is under investigation for retinitis pigmentosa (RP), Leber congenital amaurosis (LCA), Stargardt macular dystrophy (SMD) and nonexudative age-related macular degeneration (AMD), all blinding diseases that lack effective treatment options. Areas covered: The authors review investigational VCMs, including oral retinoids, 9-cis-retinyl-acetate (zuretinol) and 9-cis-β-carotene, which restore 11-cis-retinal levels in RP and LCA caused by LRAT and RPE65 gene mutations, and may improve visual acuity and visual fields...
March 15, 2018: Expert Opinion on Pharmacotherapy
https://www.readbyqxmd.com/read/29540715/mutant-best1-expression-and-impaired-phagocytosis-in-an-ipsc-model-of-autosomal-recessive-bestrophinopathy
#6
Alan D Marmorstein, Adiv A Johnson, Lori A Bachman, Cynthia Andrews-Pfannkoch, Travis Knudsen, Benjamin J Gilles, Matthew Hill, Jarel K Gandhi, Lihua Y Marmorstein, Jose S Pulido
Autosomal recessive bestrophinopathy (ARB) is caused by mutations in the gene BEST1 which encodes bestrophin 1 (Best1), an anion channel expressed in retinal pigment epithelial (RPE) cells. It has been hypothesized that ARB represents the human null phenotype for BEST1 and that this occurs due to nonsense mediated decay (NMD). To test this hypothesis, we generated induced pluripotent stem cells (iPSCs) from a patient with ARB and her parents. After differentiation to retinal pigment epithelial (iPSC-RPE) cells, both BEST1 mRNA and Best1 protein expression were compared to controls...
March 14, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29523386/the-effects-of-zinc-supplementation-on-primary-human-retinal-pigment-epithelium
#7
Po-Jung Pao, Eszter Emri, Safiya Bishar Abdirahman, Talha Soorma, Hui-Hui Zeng, Stefanie M Hauck, Richard B Thompson, Imre Lengyel
Population-based and interventional studies have shown that elevated zinc levels can reduce the progression to advanced age-related macular degeneration. The objective of this study was to assess whether elevated extracellular zinc has a direct effect on retinal pigment epithelial cells (RPE), by examining the phenotype and molecular characteristics of increased extracellular zinc on human primary RPE cells. Monolayers of human foetal primary RPE cells were grown on culture inserts and maintained in medium supplemented with increasing total concentrations of zinc (0, 75, 100, 125 and 150 μM) for up to 4 weeks...
March 1, 2018: Journal of Trace Elements in Medicine and Biology
https://www.readbyqxmd.com/read/29506236/retinal-gene-therapy
#8
Neruban Kumaran, Michel Michaelides, Alexander J Smith, Robin R Ali, James W B Bainbridge
Introduction: Inherited retinal diseases are the leading cause of sight impairment in people of working age in England and Wales, and the second commonest in childhood. Gene therapy offers the potential for benefit. Sources of data: Pubmed and clinicaltrials.gov. Areas of agreement: Gene therapy can improve vision in RPE65-associated Leber Congenital Amaurosis (RPE65-LCA). Potential benefit depends on efficient gene transfer and is limited by the extent of retinal degeneration...
March 1, 2018: British Medical Bulletin
https://www.readbyqxmd.com/read/29500274/retinoid-isomerase-inhibitors-impair-but-do-not-block-mammalian-cone-photoreceptor-function
#9
Philip D Kiser, Jianye Zhang, Aditya Sharma, Juan M Angueyra, Alexander V Kolesnikov, Mohsen Badiee, Gregory P Tochtrop, Junzo Kinoshita, Neal S Peachey, Wei Li, Vladimir J Kefalov, Krzysztof Palczewski
Visual function in vertebrates critically depends on the continuous regeneration of visual pigments in rod and cone photoreceptors. RPE65 is a well-established retinoid isomerase in the pigment epithelium that regenerates rhodopsin during the rod visual cycle; however, its contribution to the regeneration of cone pigments remains obscure. In this study, we use potent and selective RPE65 inhibitors in rod- and cone-dominant animal models to discern the role of this enzyme in cone-mediated vision. We confirm that retinylamine and emixustat-family compounds selectively inhibit RPE65 over DES1, the putative retinoid isomerase of the intraretinal visual cycle...
March 2, 2018: Journal of General Physiology
https://www.readbyqxmd.com/read/29453250/retinal-chitosan-conjugates-effectively-deliver-active-chromophores-to-retinal-photoreceptor-cells-in-blind-mice-and-dogs
#10
Songqi Gao, Shirin Kahremany, Jianye Zhang, Beata Jastrzebska, Janice Querubin, Simon M Petersen-Jones, Krzysztof Palczewski
The retinoid (visual) cycle consists of a series of biochemical reactions needed to regenerate the visual chromophore, 11-cis-retinal and sustain vision. Genetic or environmental factors affecting chromophore production can lead to blindness. Using animal models that mimic human retinal diseases, we previously demonstrated that mechanism-based pharmacological interventions can maintain vision in otherwise incurable genetic diseases of the retina. Here, we report that after 9-cis-retinal administration to lecithin:retinol acyltransferase-deficient (Lrat-/-) mice, the drug was rapidly absorbed and then cleared within 1-2 h...
February 16, 2018: Molecular Pharmacology
https://www.readbyqxmd.com/read/29332120/severe-loss-of-tritan-color-discrimination-in-rpe65-associated-leber-congenital-amaurosis
#11
Neruban Kumaran, Caterina Ripamonti, Angelos Kalitzeos, Gary S Rubin, James W B Bainbridge, Michel Michaelides
Purpose: RPE65-associated Leber congenital amaurosis (RPE65-LCA) is a progressive severe retinal dystrophy with early profound dysfunction of rod photoreceptors followed by progressive cone photoreceptor degeneration. We aim to provide detailed information about how cone dysfunction affects color discrimination. Methods: Seven adults (aged 16-21) with RPE65-LCA underwent monocular color discrimination assessment using the Trivector and Ellipse versions of three computerized tests: Cambridge Colour Test (CCT), low vision version of the Cambridge Colour Test (lvvCCT), and the Universal Colour Discrimination Test (UCDT)...
January 1, 2018: Investigative Ophthalmology & Visual Science
https://www.readbyqxmd.com/read/29248533/gene-therapy-for-rpe65-mediated-retinal-dystrophies
#12
Aaron Nagiel
No abstract text is available yet for this article.
December 15, 2017: Survey of Ophthalmology
https://www.readbyqxmd.com/read/29210653/human-umbilical-cord-mesenchymal-stem-cells-subpopulations-and-their-difference-in-cell-biology-and-effects-on-retinal-degeneration-in-rcs-rats
#13
L Wang, P Li, Y Tian, Z Li, C Lian, Q Ou, C Jin, F Gao, J-Y Xu, J Wang, F Wang, J Zhang, J Zhang, W Li, H Tian, L Lu, G-T Xu
BACKGROUND: Human umbilical cord mesenchymal stem cells (hUC-MSCs) are potential candidates for treating retinal degeneration (RD). OBJECTIVE: To further study the biology and therapeutic effects of the hUC-MSCs on retinal degeneration. METHODS: Two hUC-MSC subpopulations, termed hUC-MSC1 and hUC-MSC2, were isolated by single-cell cloning method and their therapeutic functions were compared in RCS rat, a RD model. RESULTS: Although both subsets satisfied the basic requirements for hUC-MSCs, they were significantly different in morphology, proliferation rate, differentiation capacity, phenotype and gene expression...
2017: Current Molecular Medicine
https://www.readbyqxmd.com/read/29160102/improving-the-transduction-of-bone-marrow-derived-cells-with-an-integrase-defective-lentiviral-vector
#14
S Louise Pay, Xiaoping Qi, Jeffrey F Willard, Juliana Godoy, Kavya Sankhavaram, Ranier Horton, Sayak K Mitter, Judith L Quigley, Lung-Ji Chang, Maria B Grant, Michael E Boulton
In lentiviral vector (LV) applications where transient transgene expression is sufficient, integrase-defective lentiviral vectors (IDLVs) are beneficial for reducing the potential for off-target effects associated with insertional mutagenesis. It was previously demonstrated that human RPE65 mRNA expression from an integrating lentiviral vector (ILV) induces endogenous Rpe65 and Cralbp mRNA expression in murine bone marrow-derived cells (BMDCs), initiating programming of the cells to retinal pigment epithelium (RPE)-like cells...
February 2018: Human Gene Therapy Methods
https://www.readbyqxmd.com/read/29133760/a-gene-scan-study-of-rpe65-in-chinese-patients-with-leber-congenital-amaurosis
#15
Jing Liu, Juan Bu
BACKGROUND: Leber congenital amaurosis (LCA) is a visual disease which is caused by RPE65 mutations and results in retinal degeneration and severe vision loss in early infancy. According to previous researches, mutations of the RPE65 gene account for 16% of all cases of LCA. This study aimed to identify RPE65 gene mutations in Chinese patients with LCA. METHODS: We recruited 52 sporadic patients from Peking University Third Hospital in 2016 and applied Sanger sequencing to identify variants among exons responsible for the disease...
November 20, 2017: Chinese Medical Journal
https://www.readbyqxmd.com/read/29097191/molecular-genetics-and-emerging-therapies-for-retinitis-pigmentosa-basic-research-and-clinical-perspectives
#16
REVIEW
Marina França Dias, Kwangsic Joo, Jessica A Kemp, Silvia Ligório Fialho, Armando da Silva Cunha, Se Joon Woo, Young Jik Kwon
Retinitis Pigmentosa (RP) is a hereditary retinopathy that affects about 2.5 million people worldwide. It is characterized with progressive loss of rods and cones and causes severe visual dysfunction and eventual blindness in bilateral eyes. In addition to more than 3000 genetic mutations from about 70 genes, a wide genetic overlap with other types of retinal dystrophies has been reported with RP. This diversity of genetic pathophysiology makes treatment extremely challenging. Although therapeutic attempts have been made using various pharmacologic agents (neurotrophic factors, antioxidants, and anti-apoptotic agents), most are not targeted to the fundamental cause of RP, and their clinical efficacy has not been clearly proven...
March 2018: Progress in Retinal and Eye Research
https://www.readbyqxmd.com/read/29057462/insulin-like-growth-factor-1-activates-pi3k-akt-signalling-to-protect-human-retinal-pigment-epithelial-cells-from-amiodarone-induced-oxidative-injury
#17
Rifang Liao, Fengxia Yan, Zhuanping Zeng, Haitao Wang, Kaifeng Qiu, Jinying Xu, Wenhua Zheng
BACKGROUND AND PURPOSE: Amiodarone is one of the most effective anti-arrhythmic drugs available, but its clinical applications are limited by toxic side effects including optic toxicity. The purpose of this study was to investigate the toxic effect of amiodarone on D407 cells (a human retinal pigmented epithelial (RPE) cell line) and the mechanisms of the protective effect of insulin-like growth factor-1 (IGF-1). EXPERIMENTAL APPROACH: The involvement of the kinases, Akt and ERK, was analysed by Western blot...
January 2018: British Journal of Pharmacology
https://www.readbyqxmd.com/read/29042966/effects-of-rpe-conditioned-medium-on-the-differentiation-of-hadscs-into-rpe-cells-and-their-proliferation-and-migration
#18
Yi Zhang, Dandan Zhang, Wei Wei, Bingqiao Shen, Yuyao Wang, Yingjie Zhang, Yidan Zhang, Jing Ji, Hao Sun, Min Luo, Ping Gu
Age-related macular degeneration (AMD) is associated with the dysfunction and death of the retinal pigment epithelium (RPE). Recently, there has been increasing interest in stem cell-derived RPE cells for cell replacement therapies, such as those for AMD. The present study investigated whether RPE-conditioned medium (RPECM) could promote the differentiation of human adipose tissue-derived mesenchymal stromal cells (hADSCs) into RPE cells, and enhance the proliferation and migration of these cells. Reverse-transcription quantitative polymerase chain reaction analysis demonstrated that RPECM induced hADSCs to differentiate into cells expressing RPE markers, including retinoid isomerohydrolase (RPE65), cytokeratin (CK8) and Bestrophin, which were identified to be significantly upregulated by ~10-fold, 3...
October 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/29038443/phylogenetic-analysis-of-the-metazoan-carotenoid-oxygenase-superfamily-a-new-ancestral-gene-assemblage-of-bco-like-bcol-proteins
#19
Eugenia Poliakov, Joseph Soucy, Susan Gentleman, Igor B Rogozin, T Michael Redmond
Here we describe a new family of carotenoid cleavage oxygenases (CCOs) in metazoans, the BCO2-like (BCOL) clade, which contains lancelet, nematode, and molluscan carotenoid oxygenase sequences. Phylogenetic analysis of CCOs in all kingdoms of life confirmed that the BCOL enzymes are an independent clade of ancient origin. One of the predicted lancelet BCOL proteins, cloned and analyzed for carotenoid cleavage activity in a bacterial carotenoid expression system, had activity similar to lancelet BCO2 proteins, although with a preference for cis isomers...
October 16, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29038159/loss-of-extracellular-signal-regulated-kinase-1-2-in-the-retinal-pigment-epithelium-leads-to-rpe65-decrease-and-retinal-degeneration
#20
Aswin Pyakurel, Delphine Balmer, Marc K Saba-El-Leil, Caroline Kizilyaprak, Jean Daraspe, Bruno M Humbel, Laure Voisin, Yun Z Le, Johannes von Lintig, Sylvain Meloche, Raphaël Roduit
Recent work suggested that the activity of extracellular signal-regulated kinase 1/2 (ERK1/2) is increased in the retinal pigment epithelium (RPE) of age-related macular degeneration (ARMD) patients and therefore could be an attractive therapeutic target. Notably, ERK1/2 pathway inhibitors are used in cancer therapy, with severe and noncharacterized ocular side effects. To decipher the role of ERK1/2 in RPE cells, we conditionally disrupted the Erk1 and Erk2 genes in mouse RPE. The loss of ERK1/2 activity resulted in a significant decrease in the level of RPE65 expression, a decrease in ocular retinoid levels concomitant with low visual function, and a rapid disorganization of RPE cells, ultimately leading to retinal degeneration...
December 15, 2017: Molecular and Cellular Biology
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