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https://www.readbyqxmd.com/read/29145636/localization-and-functional-characterization-of-the-p-asn965ser-n965s-abca4-variant-in-mice-reveal-pathogenic-mechanisms-underlying-stargardt-macular-degeneration
#1
Laurie L Molday, Daniel Wahl, Marinko Sarunic, Robert S Molday
ABCA4 is a member of the superfamily of ATP-binding cassette (ABC) proteins that transports N-retinylidene-phosphatidylethanolamine (N-Ret-PE) across outer segment disc membranes thereby facilitating the removal of potentially toxic retinoid compounds from photoreceptor cells. Mutations in the gene encoding ABCA4 are responsible for Stargardt disease (STGD1), an autosomal recessive retinal degenerative disease that causes severe vision loss. To define the molecular basis for STGD1 associated with the p.Asn965Ser (N965S) mutation in the Walker A motif of nucleotide binding domain 1 (NBD1), we generated a p...
November 14, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/29145208/curcumin-delays-retinal-degeneration-by-regulating-microglia-activation-in-the-retina-of-rd1-mice
#2
Yanhe Wang, Zhiyuan Yin, Lixiong Gao, Dayu Sun, Xisu Hu, Langyue Xue, Jiaman Dai, YuXiao Zeng, Siyu Chen, Boju Pan, Min Chen, Jing Xie, Haiwei Xu
BACKGROUND/AIMS: Retinitis pigmentosa (RP) is characterized by degeneration of photoreceptors, and there are currently no effective treatments for this disease. However, curcumin has shown neuroprotectant efficacy in a RP rat and swine model, and thus, may have neuroprotective effects in this disease. METHODS: Immunofluorescence staining, electroretinogram recordings, and behavioral tests were used to analyze the effects of curcumin and the underlying mechanism in retinal degeneration 1 (rd1) mice...
November 17, 2017: Cellular Physiology and Biochemistry
https://www.readbyqxmd.com/read/29143216/self-perception-and-determinants-of-color-vision-in-parkinson-s-disease
#3
Alexander U Brandt, Hanna G Zimmermann, Timm Oberwahrenbrock, Justine Isensee, Thomas Müller, Friedemann Paul
Visual dysfunction is common in patients with Parkinson's disease (PD). The objective of this study was to investigate the perceived impact of visual dysfunction and especially color vision loss on PD patients, and to identify retinal and disease factors associated with color vision. Thirty PD patients and thirty-four healthy controls were included. Participants performed the Farnsworth-Munsell Hue-100 test (FMT). Patients answered the National Eye Institute Visual Function Questionnaire (NEI-VFQ), Unified Parkinson's Disease Rating Scale (UPDRS) assessment, and underwent optical coherence tomography with measurement of retinal nerve fiber layer, ganglion cell layer + inner plexiform layer (GCIPL), and outer nuclear and photoreceptor layer...
November 15, 2017: Journal of Neural Transmission
https://www.readbyqxmd.com/read/29142592/advances-in-age-related-macular-degeneration-understanding-and-therapy
#4
Joan W Miller, Saghar Bagheri, Demetrios G Vavvas
While the development of anti-vascular endothelial growth factor (anti-VEGF) as a therapy for neovascular age-related macular degeneration (AMD) was a great success, the pathologic processes underlying dry AMD that eventually leads to photoreceptor dysfunction, death, and vision loss remain elusive to date, with a lack of effective therapies and increasing prevalence of the disease. There is an overwhelming need to improve the classification system of AMD, to increase our understanding of cell death mechanisms involved in both neovascular and non-neovascular AMD, and to develop better biomarkers and clinical endpoints to eventually be able to identify better therapeutic targets-especially early in the disease process...
2017: US Ophthalmic Review
https://www.readbyqxmd.com/read/29142497/expression-of-ccl2-and-its-receptor-in-activation-and-migration-of-microglia-and-monocytes-induced-by-photoreceptor-apoptosis
#5
ChaoYi Feng, Xin Wang, TianJin Liu, Meng Zhang, GeZhi Xu, YingQin Ni
Purpose: To explore the effect of the CCL2 and CCR2 system on the activation and migration of microglia and monocytes in light-induced photoreceptor apoptosis. Methods: At 1 day, 3 days, 7 days, and 14 days after light exposure, OX42 and ED1 immunostaining were used to label the activation and migration of microglia and monocytes. Double immunostaining of CCL2 with terminal deoxynucleotidyl transferase dUTP nick end labeling (TUNEL), OX42, or glial fibrillary acidic protein (GFAP) was applied to explore the relationships among CCL2, apoptotic photoreceptors, activated microglia and monocytes, and macroglial cells (Müller cells and astrocytes)...
2017: Molecular Vision
https://www.readbyqxmd.com/read/29142075/cone-myoid-elongation-involves-unidirectional-microtubule-movement-mediated-by-dynein-1
#6
Tylor R Lewis, Mariusz Zareba, Brian A Link, Joseph C Besharse
Teleosts and amphibians exhibit retinomotor movements, morphological changes in photoreceptors regulated by light and circadian rhythms. Cone myoid elongation occurs during dark adaptation leading to the positioning of the cone outer segment closer to the retinal pigment epithelium. While it has been shown that microtubules are essential for cone myoid elongation, the underlying mechanism has not been established. In this work, we generated a transgenic line of zebrafish expressing a photoconvertible form of α-tubulin (tdEOS-tubulin) specifically in cone photoreceptors...
November 15, 2017: Molecular Biology of the Cell
https://www.readbyqxmd.com/read/29141877/giving-invertebrates-an-eye-exam-an-ophthalmoscope-that-utilizes-the-autofluorescence-of-photoreceptors
#7
Annette Stowasser, Madeline Owens, Elke K Buschbeck
One of the most important functional features of eyes is focusing light, as both nearsightedness and farsightedness have major functional implications. Accordingly, refractive errors are frequently assessed in vertebrates, but not in the very small invertebrate eyes. We describe a micro-ophthalmoscope that takes advantage of autofluorescent properties of invertebrate photoreceptors and test the device on the relatively well-understood eyes of jumping spiders and flies. In each case, our measurements confirmed previous findings with a greater degree of accuracy...
November 15, 2017: Journal of Experimental Biology
https://www.readbyqxmd.com/read/29141686/anisotropic-m%C3%A3-ller-glial-scaffolding-supports-a-multiplex-lattice-mosaic-of-photoreceptors-in-zebrafish-retina
#8
Mikiko Nagashima, Jeremy Hadidjojo, Linda K Barthel, David K Lubensky, Pamela A Raymond
BACKGROUND: The multiplex, lattice mosaic of cone photoreceptors in the adult fish retina is a compelling example of a highly ordered epithelial cell pattern, with single cell width rows and columns of cones and precisely defined neighbor relationships among different cone types. Cellular mechanisms patterning this multiplex mosaic are not understood. Physical models can provide new insights into fundamental mechanisms of biological patterning. In earlier work, we developed a mathematical model of photoreceptor cell packing in the zebrafish retina, which predicted that anisotropic mechanical tension in the retinal epithelium orients planar polarized adhesive interfaces to align the columns as cone photoreceptors are generated at the retinal margin during post-embryonic growth...
November 15, 2017: Neural Development
https://www.readbyqxmd.com/read/29140861/sealing-of-corneal-lacerations-using-photoactivated-rose-bengal-dye-and-amniotic-membrane
#9
Timothy A Soeken, Hong Zhu, Sheri DeMartelaere, Brett W Davies, Mirang Kim, Heuy-Ching Wang, James Aden, Rose Grimm, Clemens Alt, Irene E Kochevar, Anthony J Johnson
PURPOSE: Watertight closure of perforating corneoscleral lacerations is necessary to prevent epithelial ingrowth, infection, and potential loss of the eye. Complex lacerations can be difficult to treat, and repair with sutures alone is often inadequate. In this study, we evaluated a potentially sutureless technology for sealing complex corneal and scleral lacerations that bonds the amniotic membrane (AM) to the wound using only green light and rose bengal dye. METHODS: The AM was impregnated with rose bengal and then sealed over lacerations using green light to bond the AM to the deepithelialized corneal surface...
November 14, 2017: Cornea
https://www.readbyqxmd.com/read/29140328/retina-simulating-phantom-produced-by-photolithography
#10
Denise Valente, Brian Vohnsen
Cone photoreceptors have a narrow acceptance angle that is well matched to the size of the eye pupil and dampens the visual impact of aberrations and scattering. However, the structure of the human retina is not replicated in existing eye models used to test refractive designs or retinal implants that restore partial vision to the blind. Here, we report on an artificial waveguide-based retinal phantom manufactured by photolithography in photoresist film with dimensions and refractive index contrast similar to the retinal receptor layer...
November 15, 2017: Optics Letters
https://www.readbyqxmd.com/read/29139167/the-zinc-finger-gene-nolz1-regulates-the-formation-of-retinal-progenitor-cells-and-suppresses-the-lim3-lhx3-phenotype-of-retinal-bipolar-cells-in-chicken-retina
#11
Maria K E Blixt, Dardan Konjusha, Henrik Ring, Finn Hallböök
BACKGROUND: The zinc-finger transcription factor Nolz1 regulates spinal cord neuron development by interacting with the transcription factors Isl1, Lim1 and Lim3, which are also important for photoreceptors, horizontal and bipolar cells during retinal development. We therefore studied Nolz1 during retinal development. RESULTS: Nolz1 expression was seen in two waves during development: one early (peak at embryonic day 3-4.5) in retinal progenitors and one late (embryonic day 8) in newly differentiated cells in the inner nuclear layer...
November 15, 2017: Developmental Dynamics: An Official Publication of the American Association of Anatomists
https://www.readbyqxmd.com/read/29139123/new-insights-into-the-mechanisms-of-phytochrome-cryptochrome-coaction
#12
REVIEW
Qin Wang, Qing Liu, Xu Wang, Zecheng Zuo, Yoshito Oka, Chentao Lin
Contents I. II. III. IV. SUMMARY: Plants perceive and respond to light signals by multiple sensory photoreceptors, including phytochromes and cryptochromes, which absorb different wavelengths of light to regulate genome expression and plant development. Photophysiological analyses have long revealed the coordinated actions of different photoreceptors, a phenomenon referred to as the photoreceptor coaction. The mechanistic explanations of photoreceptor coactions are not fully understood...
November 15, 2017: New Phytologist
https://www.readbyqxmd.com/read/29138314/imaging-and-quantifying-ganglion-cells-and-other-transparent-neurons-in-the-living-human-retina
#13
Zhuolin Liu, Kazuhiro Kurokawa, Furu Zhang, John J Lee, Donald T Miller
Ganglion cells (GCs) are fundamental to retinal neural circuitry, processing photoreceptor signals for transmission to the brain via their axons. However, much remains unknown about their role in vision and their vulnerability to disease leading to blindness. A major bottleneck has been our inability to observe GCs and their degeneration in the living human eye. Despite two decades of development of optical technologies to image cells in the living human retina, GCs remain elusive due to their high optical translucency...
November 14, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/29137925/polycomb-repression-complex-2-is-required-for-the-maintenance-of-retinal-progenitor-cells-and-balanced-retinal-differentiation
#14
Naoko Fujimura, Andrea Kuzelova, Anja Ebert, Hynek Strnad, Jitka Lachova, Ondrej Machon, Meinrad Busslinger, Zbynek Kozmik
Polycomb repressive complexes maintain transcriptional repression of genes encoding crucial developmental regulators through chromatin modification. Here we investigated the role of Polycomb repressive complex 2 (PRC2) in retinal development by inactivating its key components Eed and Ezh2. Conditional deletion of Ezh2 resulted in a partial loss of PRC2 function and accelerated differentiation of Müller glial cells. In contrast, inactivation of Eed led to the ablation of PRC2 function at early postnatal stage...
November 11, 2017: Developmental Biology
https://www.readbyqxmd.com/read/29134295/rod-versus-cone-driven-ergs-at-different-stimulus-sizes-in-normal-subjects-and-retinitis-pigmentosa-patients
#15
Avinash J Aher, Declan J McKeefry, Neil R A Parry, John Maguire, I J Murray, Tina I Tsai, Cord Huchzermeyer, Jan Kremers
PURPOSE: To study how rod- and cone-driven responses depend on stimulus size in normal subjects and patients with retinitis pigmentosa (RP), and to show that comparisons between responses to full-field (FF) and smaller stimuli can be useful in diagnosing and monitoring disorders of the peripheral retina without the need for lengthy dark adaptation periods. METHOD: The triple silent substitution technique was used to isolate L-cone-, M-cone- and rod-driven ERGs with 19, 18 and 33% photoreceptor contrasts, respectively, under identical mean luminance conditions...
November 13, 2017: Documenta Ophthalmologica. Advances in Ophthalmology
https://www.readbyqxmd.com/read/29134201/pushing-the-limits-of-photoreception-in-twilight-conditions-the-rod-like-cone-retina-of-the-deep-sea-pearlsides
#16
Fanny de Busserolles, Fabio Cortesi, Jon Vidar Helvik, Wayne I L Davies, Rachel M Templin, Robert K P Sullivan, Craig T Michell, Jessica K Mountford, Shaun P Collin, Xabier Irigoien, Stein Kaartvedt, Justin Marshall
Most vertebrates have a duplex retina comprising two photoreceptor types, rods for dim-light (scotopic) vision and cones for bright-light (photopic) and color vision. However, deep-sea fishes are only active in dim-light conditions; hence, most species have lost their cones in favor of a simplex retina composed exclusively of rods. Although the pearlsides, Maurolicus spp., have such a pure rod retina, their behavior is at odds with this simplex visual system. Contrary to other deep-sea fishes, pearlsides are mostly active during dusk and dawn close to the surface, where light levels are intermediate (twilight or mesopic) and require the use of both rod and cone photoreceptors...
November 2017: Science Advances
https://www.readbyqxmd.com/read/29133761/reduction-on-off-responses-of-electroretinogram-in-monkeys-with-long-term-high-intraocular-pressure
#17
Ke-Gao Liu, Xiao-Yan Peng, Zheng Zhang, Hua Sun, Di-Ya Yang, Ning-Li Wang
BACKGROUND: There are ON- and OFF-pathways in the normal vertebrate retina. Short- and long-flash electroretinogram (ERG) are suitable methods to observe the function of ON- and OFF-pathways in vivo, respectively. It is clear that high intraocular pressure (IOP) might cause dysfunction of cone-dominated photopic negative response (PhNR) in monkeys with high IOP in ON-pathway. However, whether cone-dominated OFF-responses are also affected is less known. The aim of this study was to observe photopic OFF-responses of ERG in monkeys with high IOP...
November 20, 2017: Chinese Medical Journal
https://www.readbyqxmd.com/read/29133431/restoration-of-dendritic-complexity-functional-connectivity-and-diversity-of-regenerated-retinal-bipolar-neurons-in-adult-zebrafish
#18
Timothy E McGinn, Diana M Mitchell, Peter C Meighan, Natalie Partington, Dylan C Leoni, Christina E Jenkins, Michael D Varnum, Deborah L Stenkamp
Adult zebrafish (Danio rerio) are capable of regenerating retinal neurons that have been lost due to mechanical, chemical, or light damage. In the case of chemical damage, there is evidence that visually-mediated behaviors are restored following regeneration, consistent with recovery of retinal function. However, the extent to which regenerated retinal neurons attain appropriate morphologies and circuitry after such tissue-disrupting lesions has not been investigated. Adult zebrafish of both sexes were subjected to intravitreal injections of ouabain, which destroys the inner retina...
November 13, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/29133122/involvement-of-cannabinoid-receptor-type-2-in-light-induced-degeneration-of-cells-from-mouse-retinal-cell-line-in-vitro-and-mouse-photoreceptors-in-vivo
#19
Tomoyo Imamura, Kazuhiro Tsuruma, Yuki Inoue, Tomohiro Otsuka, Yuta Ohno, Shiho Ogami, Shinsaku Yamane, Masamitsu Shimazawa, Hideaki Hara
Earlier studies showed that the expressions of the agonists of the cannabinoid receptors are reduced in the vitreous humor of patients with age-related macular degeneration (AMD), and the cannabinoid type 2 receptor is present in the retinas of rats and monkeys. The purpose of this study was to determine whether the cannabinoid type 2 receptor is involved in the light-induced death of cultured 661W cells, an immortalized murine retinal cell line, and in the light-induced retinal degeneration in mice. Time-dependent changes in the expression and location of retinal cannabinoid type 2 receptor were determined by Western blot and immunostaining...
November 10, 2017: Experimental Eye Research
https://www.readbyqxmd.com/read/29131863/long-term-retinal-cone-rescue-using-a-capsid-mutant-aav8-vector-in-a-mouse-model-of-cnga3-achromatopsia
#20
Xufeng Dai, Ying He, Hua Zhang, Yangyang Zhang, Yan Liu, Muran Wang, Hao Chen, Ji-Jing Pang
Adeno-associated virus (AAV) vectors are important gene delivery tools for the treatment of many recessively inherited retinal diseases. For example, a wild-type (WT) AAV5 vector can deliver a full-length Cnga3 (cyclic nucleotide-gated channel alpha-3) cDNA to target cells of the cone photoreceptor function loss 5 (cpfl5) mouse, a spontaneous animal model of achromatopsia with a Cnga3 mutation. Gene therapy restores cone-mediated function and blocks cone degeneration in the mice. However, since transgene expression delivered by an AAV vector shows relatively short-term effectiveness, this cannot be regarded as a very successful therapy...
2017: PloS One
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