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https://www.readbyqxmd.com/read/30116628/peripheral-cone-dystrophy-expanded-clinical-spectrum-multimodal-and-ultrawide-field-imaging-and-genomic-analysis
#1
Robert A Sisk, Robert B Hufnagel, Ailee Laham, Elizabeth S Wohler, Nara Sobreira, Zubair M Ahmed
Purpose: To present new clinical features, multimodal and ultrawide-field imaging characteristics of peripheral cone dystrophy (PCD), and results of laboratory and genetic investigation to decipher the etiology. Methods: Retrospective observational case-series. Results: Three patients with PCD presented with bilateral paracentral scotomas and a mean visual acuity of 20/25. All exhibited confluent macular hyperautofluorescence with a central bull's eye lesion...
2018: Journal of Ophthalmology
https://www.readbyqxmd.com/read/30116590/sustained-and-targeted-episcleral-delivery-of-celecoxib-in-a-rabbit-model-of-retinal-and-choroidal-neovascularization
#2
Luiz H Lima, Michel E Farah, Glenwood Gum, Pamela Ko, Ricardo A de Carvalho
Background: To evaluate the efficacy of selective episcleral delivery of celecoxib formulated in a sustained-release episcleral exoplant on a model of retinal and choroidal neovascularization induced in rabbits by subretinal injection of matrigel combined with vascular endothelial growth factor (VEGF) and basic fibroblast growth factor (bFGF). Methods: Nine New Zealand white rabbits were randomly assigned to three groups (episcleral celecoxib exoplant, intravitreal bevacizumab injection and control group)...
2018: International Journal of Retina and Vitreous
https://www.readbyqxmd.com/read/30112688/m%C3%A3-ller-glial-dysfunction-during-diabetic-retinopathy-in-rats-is-reduced-by-the-acrolein-scavenging-drug-2-hydrazino-4-6-dimethylpyrimidine
#3
Rosemary E McDowell, Peter Barabas, Josy Augustine, Olivier Chevallier, Philip McCarron, Mei Chen, J Graham McGeown, Tim M Curtis
AIMS/HYPOTHESIS: Recent studies suggest that abnormal function in Müller glial cells plays an important role in the pathogenesis of diabetic retinopathy. This is associated with the selective accumulation of the acrolein-derived advanced lipoxidation end-product, Nε -(3-formyl-3,4-dehydropiperidino)lysine (FDP-lysine), on Müller cell proteins. The aim of the current study was to identify more efficacious acrolein-scavenging drugs and determine the effects of the most potent on Müller cell FDP-lysine accumulation and neuroretinal dysfunction during diabetes...
August 15, 2018: Diabetologia
https://www.readbyqxmd.com/read/30110696/intravitreous-delivery-of-%C3%AE-b-crystallin-ameliorates-n-methyl-n-nitrosourea-induced-photoreceptor-degeneration-in-mice-an-in-vivo-and-ex-vivo-study
#4
Ye Tao, Lei Ding, Anhui Yao, Zhen Yang, Qinghua Yang, Limin Qin, Linjun Yu, Yanling Gao, Yi Fei Huang, Zhengwei Li, Dengke Teng
BACKGROUND/AIMS: αB -crystallin (αBC) belongs to the family of small heat shock proteins that are necessary for maintaining oxygen homeostasis. This study was designed to explore the possible effects of αBC on N-methyl- N-nitrosourea (MNU) induced retinal degeneration and the underlying mechanisms. METHODS: The αBC was injected into the vitreous bodies of MNU administered mice. The retinal morphology and visual function of experimental animals were analyzed by electroretinography (ERG), Spectral domain optical coherence tomography (SD-OCT), fundus photographs, optokinetic testing and immunohistochemistry assay...
August 15, 2018: Cellular Physiology and Biochemistry
https://www.readbyqxmd.com/read/30109754/optical-coherence-tomography-and-molecular-analysis-of-sudden-acquired-retinal-degeneration-syndrome-sards-eyes-suggests-the-immune-mediated-nature-of-retinal-damage
#5
Sinisa D Grozdanic, Tatjana Lazic, Helga Kecova, Kabhilan Mohan, Markus H Kuehn
OBJECTIVE: To perform detailed analysis of retinal changes in dogs with SARDS using optical coherence tomography (OCT), funduscopy, and molecular analysis. ANIMALS: Subjects were 29 dogs from 12 US states and Canada diagnosed with SARDS by 8 ophthalmologists. An additional 7 eyes from 5 deceased SARDS dogs were used for molecular and histological analysis. PROCEDURES: Dogs were evaluated using chromatic pupil light reflex testing (cPLR), and electroretinography (ERG); subjects underwent complete ophthalmic examination, including funduscopy, retinal photography, and OCT, in addition to complete laboratory analysis, blood pressure evaluation, abdominal and thoracic radiographs, and computerized tomography (CT) imaging to assess possible systemic abnormalities...
August 15, 2018: Veterinary Ophthalmology
https://www.readbyqxmd.com/read/30105490/predicting-visual-function-after-an-ocular-bee-sting
#6
Masih Ahmed, Chang Sup Lee, Brian McMillan, Priyanka Jain, Lee Wiley, J Vernon Odom, Monique Leys
PURPOSE: To report a case of toxic optic neuropathy caused by an ocular bee sting. METHODS: Case report and literature review. RESULTS: A 44-year-old female presented with no light perception vision 2 days after a corneal bee sting in her right eye. She was found to have diffuse cornea edema with overlying epithelial defect and a pinpoint penetrating laceration at 6 o'clock. There was an intense green color to the cornea. The pupil was fixed and dilated with an afferent pupillary defect...
August 13, 2018: International Ophthalmology
https://www.readbyqxmd.com/read/30098196/microrna-124-dysregulation-is-associated-with-retinal-inflammation-and-photoreceptor-death-in-the-degenerating-retina
#7
Joshua A Chu-Tan, Matt Rutar, Kartik Saxena, Riemke Aggio-Bruce, Rohan W Essex, Krisztina Valter, Haihan Jiao, Nilisha Fernando, Yvette Wooff, Michele C Madigan, Jan Provis, Riccardo Natoli
Purpose: We sought to determine the role and retinal cellular location of microRNA-124 (miR-124) in a neuroinflammatory model of retinal degeneration. Further, we explored the anti-inflammatory relationship of miR-124 with a predicted messenger RNA (mRNA) binding partner, chemokine (C-C motif) ligand 2 (Ccl2), which is crucially involved in inflammatory cell recruitment in the damaged retina. Methods: Human AMD donor eyes and photo-oxidative damaged (PD) mice were labeled for miR-124 expression using in situ hybridization...
August 1, 2018: Investigative Ophthalmology & Visual Science
https://www.readbyqxmd.com/read/30090179/preoperative-electrophysiological-characterization-of-patients-with-primary-macula-involving-rhegmatogenous-retinal-detachment
#8
Jonathan B Lin, Julia Sein, Gregory P Van Stavern, Rajendra S Apte
Purpose: To determine 1) which components of retinal function are impaired after rhegmatogenous retinal detachment, 2) which outer retinal pathways (rod- or cone-driven) are more severely affected, and 3) whether there is concomitant inner retinal dysfunction. Methods: We conducted a prospective observational study in a large academic institution. We performed preoperative electroretinography on eight patients to assess outer and inner retinal function. In all cases, a comparison between the eye with the detached retina and the control fellow eye was made...
July 2018: Journal of Ophthalmic & Vision Research
https://www.readbyqxmd.com/read/30090174/electroretinography-and-rhegmatogenous-retinal-detachment
#9
EDITORIAL
Mohammad Mehdi Parvaresh
No abstract text is available yet for this article.
July 2018: Journal of Ophthalmic & Vision Research
https://www.readbyqxmd.com/read/30079774/3-5-dimethoxy-4-hydroxy-myricanol-ameliorates-photoreceptor-cell-degeneration-in-pde6b-rd10-mouse-model
#10
Yan-Yan Wang, Chen-Guang Wang, Shou-Nan Qi, Zao-Xia Liu, Guan-Fang Su, Ya-Juan Zheng
Retinitis pigmentosa (RP) caused by the photoreceptor cell degeneration is currently incurable and leads to partial or complete blindness eventually. 3,5-dimethoxy-4-hydroxy myricanol (DM) is a novel compound isolated from the leaves of Micromelum integerrimum, with proliferative activities on NIH3T3 cells. This study was to investigate whether DM could mitigate retinal degeneration of rd10 mice, a well-characterized mouse model of RP. Rd10 mice were treated with DM daily by intraperitoneal injection from postnatal day 12 (P12) to P26...
August 6, 2018: Cutaneous and Ocular Toxicology
https://www.readbyqxmd.com/read/30074097/transplantation-of-rat-embryonic-stem-cell-derived-retinal-cells-restores-visual-function-in-the-royal-college-of-surgeons-rats
#11
Hongxi Wu, Jia Li, Xinbang Mao, Guodong Li, Lin Xie, Zhipeng You
AIM OF STUDY: To evaluate the feasibility of transplantation of embryonic stem cell (ESC)-derived retinal cells in the treatment of retinal degeneration. MATERIALS AND METHODS: Rat ESCs were isolated and induced into retinal progenitor cells (RPCs) in vitro, which were subsequently induced into retinal pigment epithelium cells (RPEs) and photoreceptors (PRCs). All cells were identified by Western blot detection of their specific markers. RPEs and PRCs were, respectively, injected into the retina of Royal College of Surgeons (RCSs) rats...
August 3, 2018: Documenta Ophthalmologica. Advances in Ophthalmology
https://www.readbyqxmd.com/read/30060493/expanding-the-mutation-spectrum-in-abca4-sixty-novel-disease-causing-variants-and-their-associated-phenotype-in-a-large-french-stargardt-cohort
#12
Marco Nassisi, Saddek Mohand-Saïd, Claire-Marie Dhaenens, Fiona Boyard, Vanessa Démontant, Camille Andrieu, Aline Antonio, Christel Condroyer, Marine Foussard, Cécile Méjécase, Chiara Maria Eandi, José-Alain Sahel, Christina Zeitz, Isabelle Audo
Here we report novel mutations in ABCA4 with the underlying phenotype in a large French cohort with autosomal recessive Stargardt disease. The DNA samples of 397 index subjects were analyzed in exons and flanking intronic regions of ABCA4 (NM_000350.2) by microarray analysis and direct Sanger sequencing. At the end of the screening, at least two likely pathogenic mutations were found in 302 patients (76.1%) while 95 remained unsolved: 40 (10.1%) with no variants identified, 52 (13.1%) with one heterozygous mutation, and 3 (0...
July 27, 2018: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/30055837/joubert-syndrome-ophthalmological-findings-in-correlation-with-genotype-and-hepatorenal-disease-in-99-patients-prospectively-evaluated-at-a-single-center
#13
Brian P Brooks, Wadih M Zein, Amy H Thompson, Maryam Mokhtarzadeh, Daniel A Doherty, Melissa Parisi, Ian A Glass, May C Malicdan, Thierry Vilboux, Meghana Vemulapalli, James C Mullikin, William A Gahl, Meral Gunay-Aygun
PURPOSE: Joubert syndrome (JS) is caused by mutations in >34 genes that encode proteins involved with primary (nonmotile) cilia and the cilium basal body. This study describes the varying ocular phenotypes in JS patients, with correlation to systemic findings and genotype. DESIGN: Patients were systematically and prospectively examined at the National Institutes of Health (NIH) Clinical Center in the setting of a dedicated natural history clinical trial. PARTICIPANTS: Ninety-nine patients with JS examined at a single center...
July 25, 2018: Ophthalmology
https://www.readbyqxmd.com/read/30054542/long-term-rescue-of-photoreceptors-in-a-rodent-model-of-retinitis-pigmentosa-associated-with-mertk-mutation
#14
H Lorach, S Kang, R Dalal, M B Bhuckory, Y Quan, D Palanker
MERTK mutation reduces the ability of retinal pigment epithelial (RPE) cells to phagocytize the photoreceptor outer segments, which leads to accumulation of debris separating photoreceptors from RPE cells, resulting in their degeneration and loss of vision. In a rat model of Retinitis Pigmentosa due to MERTK mutation, we demonstrate that surgical removal of debris performed when about half of photoreceptors are lost (P38), allows the remaining photoreceptor cells to renew their outer segments and survive for at least 6 months - 3 times longer than in untreated eyes...
July 27, 2018: Scientific Reports
https://www.readbyqxmd.com/read/30051304/phenotypic-characterization-of-complete-csnb-in-the-inbred-research-beagle-how-common-is-csnb-in-research-and-companion-dogs
#15
Annie Oh, Ellis R Loew, Melanie L Foster, Michael G Davidson, Robert V English, Kristen J Gervais, Ian P Herring, Freya M Mowat
PURPOSE: Although congenital stationary night blindness (CSNB) has been described in a Japanese beagle dog research colony, certain clinical correlates with human CSNB have not yet been described, nor has an estimate of frequency of the condition been made in inbred and outbred beagle populations. METHODS: A beagle with CSNB obtained from a commercial research dog supplier in the USA and matched control dogs (n = 3) underwent examination, refraction, ocular imaging, assessment of visual navigation ability and detailed electroretinography (ERG)...
July 26, 2018: Documenta Ophthalmologica. Advances in Ophthalmology
https://www.readbyqxmd.com/read/30048621/a-cell-penetrating-peptide-from-socs-1-prevents-ocular-damage-in-experimental-autoimmune-uveitis
#16
Chulbul M Ahmed, Michael T Massengill, Emily E Brown, Cristhian J Ildefonso, Howard M Johnson, Alfred S Lewin
We describe an immunosuppressive peptide corresponding to the kinase inhibitory region (KIR) of the intracellular checkpoint protein suppressor of cytokine signaling 1 (SOCS-1) that binds to the phospho-tyrosine containing regions of the tyrosine kinases JAK2 and TYK2 and the adaptor protein MAL, and thereby inhibits signaling downstream from these signaling mediators. The peptide, SOCS1-KIR, is thus capable of downregulating overactive JAK/STAT or NF-kB signaling in somatic cells, including those in many compartments of the eye...
July 23, 2018: Experimental Eye Research
https://www.readbyqxmd.com/read/30030392/identification-of-a-novel-pathogenic-missense-mutation-in-prpf31-using-whole-exome-sequencing-a-case-report
#17
Laura Bryant, Olga Lozynska, Anson Marsh, Tyler E Papp, Lucas van Gorder, Leona W Serrano, Xiaowu Gai, Albert M Maguire, Tomas S Aleman, Jean Bennett
BACKGROUND: Variants in PRPF31 , which encodes pre-mRNA processing factor 31 homolog, are known to cause autosomal-dominant retinitis pigmentosa (adRP) with incomplete penetrance. However, the majority of mutations cause null alleles, with only two proven pathogenic missense mutations. We identified a novel missense mutation in PRPF31 in a family with adRP. METHODS: We performed whole exome sequencing to identify possible pathogenic mutations in the proband of a family with adRP...
July 20, 2018: British Journal of Ophthalmology
https://www.readbyqxmd.com/read/30027431/clinical-and-genetic-findings-of-a-japanese-patient-with-rp1-related-autosomal-recessive-retinitis-pigmentosa
#18
Kentaro Kurata, Katsuhiro Hosono, Yoshihiro Hotta
PURPOSE: This study reports the ophthalmic and genetic findings of a Japanese patient with autosomal recessive retinitis pigmentosa (arRP) caused by retinitis pigmentosa 1 (RP1) mutations. PATIENT AND METHODS: The 34-year-old female patient and her unaffected parents underwent comprehensive ophthalmic examinations, including visual acuity measurements, perimetry, electroretinography (ERG), and optical coherence tomography. Fundus autofluorescence was also evaluated in the patient...
August 2018: Documenta Ophthalmologica. Advances in Ophthalmology
https://www.readbyqxmd.com/read/30025123/systemic-7-8-dihydroxyflavone-treatment-protects-immature-retinas-against-hypoxic-ischemic-injury-via-m%C3%A3-ller-glia-regeneration-and-mapk-erk-activation
#19
Hsiu-Mei Huang, Chao-Ching Huang, Meng-Han Tsai, Yi-Chieh Poon, Ying-Chao Chang
Purpose: Perinatal hypoxic-ischemic (HI) injury causes significant damages in the immature retina. The brain-derived neurotrophic factor is well known for its neuroprotective role but has limited clinical applications. A selective agonist of tyrosine kinase receptor B, 7,8-dihydroxyflavone (DHF), is a powerful therapeutic tool, when administered systemically. However, it remains unclear whether DHF treatment can protect the immature retinas against HI injury. Methods: Postnatal (P) day 7 rat pups were intraperitoneally injected with DHF or vehicle 2 hours before and 18 hours after being subjected to HI injury...
June 1, 2018: Investigative Ophthalmology & Visual Science
https://www.readbyqxmd.com/read/30025090/retinal-basal-laminar-deposits-in-complement-fh-fp-mouse-model-of-dense-deposit-disease
#20
Delu Song, Imran Mohammed, Rupak Bhuyan, Takashi Miwa, Allison Lesher Williams, Damodar Gullipalli, Sayaka Sato, Ying Song, Joshua L Dunaief, Wen-Chao Song
Purpose: Dense deposit disease (DDD) is caused by dysregulation of the alternative pathway of the complement cascade and characterized by electron-dense deposits in the kidney glomerular basement membrane (GBM) and drusen in Bruch's membrane (BrM). Complement factor H (fH) and factor properdin (fP) regulate complement activation; fH inhibits alternative pathway (AP) activation, whereas fP promotes it. We report pathologic changes in eyes of an fH and fP double-mutant mouse, which we previously showed have dense deposits in the GBM and early mortality from nephropathy...
July 2, 2018: Investigative Ophthalmology & Visual Science
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