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Prognostication in Stargardt Disease Using Fundus Autofluorescence: Improving Patient Care

OPHTHALMOLOGY 2023年第11期130卷 1182-1190页

作者： Varela, Malena Daich Laich, Yannik Hashem, Shaima Awadh Mahroo, Omar A. Webster, Andrew R. Michaelides, Michel Moorfields Eye Hosp London England UCL UCL Inst Ophthalmol London England Univ Freiburg Fac Med Eye Ctr Med Ctr Freiburg Germany UCL Inst Ophthalmol 11-43 Bath St London EC1V 9EL England

Purpose: To explore fundus autofluorescence (FAF) imaging as an alternative to electroretinography as a noninvasive, quick, and readily interpretable method to predict disease progression in Stargardt disease (STGD).Design: Retrospective case series of patients who attended Moorfields Eye Hospital (London, United Kingdom).Participants: Patients with STGD who met the following criteria were included: (1) biallelic disease-causing variants in ABCA4, (2) electroretinography testing performed in house with an unequivocal electroretinography group classification, and (3) ultrawidefield (UWF) FAF imaging performed up to 2 years before or after the ***: Patients were divided into 3 electroretinography groups based on retinal function and 3 FAF groups according to the extent of hypoautofluorescence and retinal background appearance. Fundus auto-fluorescence images of 30 degrees and 55 degrees were reviewed *** Outcome Measures: Electroretinography and FAF concordance and its association with baseline visual acuity (VA) and ***: Two hundred thirty-four patients were included in the cohort. One hundred seventy patients (73%) were in electroretinography and FAF groups of the same severity, 33 (14%) were in a milder FAF than electro-retinography group, and 31 (13%) were in a more severe FAF than electroretinography group. Children retinal involvement and thereby inform prognostication by comparing FAF with the current gold standard of electro-retinography. In 80% of p

关键词： Autofluorescence Electroretinogram Genetics retina Stargardt

来源： SCIE

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CYCLIC LIGHT-INTENSITY THRESHOLD FOR retinaL DAMAGE IN ALBINO-RATS RAISED UNDER 6-LX

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EXPERIMENTAL EYE RESEARCH 1987年第5期44卷 643-661页

作者： SEMPLEROWLAND, SL DAWSON, WW UNIV FLORIDA J HILLIS MILLER HLTH CTRSCH MEDDEPT OPHTHALMOLGAINESVILLEFL 32610

This study determined the minimum cyclic (12L:12D) light intensity which produces retinal damage in albino (Sprague-Dawley) rats raised from birth to 15 weeks of age under 6 lx (12L:12D). Four experimental light intensities were tested, viz. 1345, 270, 130 and 65 lx. Control animals remained under 6 lx. After various durations of exposure to one of the intensities tested, the retinas of groups of six rats were evaluated for physiological and morphological evidence of light damage. The indices of damage were: (1) histological and morphometric changes in the retina, and (2) changes in the amplitude and latency of the b-wave of the electroretinogram. Light intensities of 1345- or 270 lx severely damage retinas of albino rats raised from birth under 6 lx within 3-7 days of the initiation of light exposure. Exposure to 130- or 65 lx produced much less dramatic changes in the responsiveness and morphology of the retina that did not appear to be permanent. Based on these results, a reasonable estimate for the threshold cyclic-light intensity which produces damage to retinas of albino rats raised under 6 lx lies between 65- and 130 lx, or slightly more than 1 log unit above the light intensity under which the animals were raised. The effects of an animal's light history on retinal susceptibility to light damage are discussed.

关键词： albino rat cyclic electrophysiology light damage morphology retina threshold

来源： SCIE

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EXPRESSION OF GLUTAMATE RECEPTOR GENES IN THE MAMMALIAN retina - THE LOCALIZATION OF GLUR1 THROUGH GLUR7 MESSENGER-RNAS

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JOURNAL OF NEUROSCIENCE 1993年第5期13卷 1888-1898页

作者： HAMASSAKIBRITTO, DE HERMANSBORGMEYER, I HEINEMANN, S HUGHES, TE UNIV CALIF SAN DIEGO DEPT NEUROSCILA JOLLACA 92093 SALK INST BIOL STUDIES LA JOLLACA 92037

Seven distinct cDNAs encoding functional subunits of the AMPA/kainate-type glutamate receptors have been recently cloned. This in situ hybridization study was done to determine which subunits are expressed in the retina and, where possible, which neurons express them. Hybridization of S-35-UTP-labeled cRNA probes with transverse sections revealed that mRNAs for all seven receptor subunits (GluR1-GluR7) are expressed in both cat and rat retinas. GluR1 and GluR2 produced labeling over the entire inner nuclear layer (INL) and ganglion cell layer (GCL). GluR3-GluR7 have more limited distributions, indicative of expression by only a subset of neurons. All of the subunits are expressed by the cells at the inner edge of the INL, where amacrine cells reside, yet the layers with the horizontal, bipolar, and ganglion cells contain different subsets of subunits. These findings suggest that these glutamate receptor subunits are employed at many of the retinal synapses, including the photoreceptor input to the outer plexiform layer and the bipolar cell's contacts with the processes at the INL. It is also possible that some glial cells in the INL express some of the subunits. Since different combinations of GluR1-GluR3 have been shown to play an important role in the calcium permeability in response to glutamate, we investigated whether single cells coexpressed those subunits. By hybridizing adjacent semithin (1 mum) sections of the cat retina with probes for GluR1-GluR3, it was possible to observe coexpression of all three subunits, or of pairs of these subunits, in cells within the INL and GCL.

关键词： EXCITATORY AMINO ACID GLUTAMATE GLUTAMATE RECEPTORS INSITU HYBRIDIZATION RECEPTOR SUBUNITS retina

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PHOTORECEPTOR DAMAGE FOLLOWING EXPOSURE TO EXCESS RIBOFLAVIN

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EXPERIENTIA 1993年第12期49卷 1084-1087页

作者： ECKHERT, CD HSU, MH PANG, N 1. Department of Environmental Health Sciences University of California 90024-1772 Los Angeles California USA

Flavins generate oxidants during metabolism and when exposed to light(1). Here we report that the photoreceptor layer of retinas from black-eyed rats is reduced in size by a dietary regime containing excess riboflavin. The effect of excess riboflavin was dose-dependent and was manifested by a decrease in photoreceptor length. This decrease was due in part to a reduction in the thickness of the outer nuclear layer, a structure formed from stacked photoreceptor nuclei. These changes were accompanied by an increase in photoreceptor outer segment autofluorescence following illumination at 328 nm, a wavelength that corresponds to the excitation maxima of oxidized lipopigments of the retinal pigment epithelium(2).

关键词： RIBOFLAVIN FLAVINS TISSUE DAMAGE retina retinaL DEGENERATION

来源： SCIE

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Effects of intravitreous injection of preserved and nonpreserved triamcinolone in rabbit retina

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ARCHIVES OF OPHTHALMOLOGY 2006年第11期124卷 1666-1666页

作者： Carvounis, Petros E. El Barr, Muhammad Abd Albini, Thomas A. Holz, Eric R. Baylor Coll Med Cullen Eye Inst Houston TX 77030 USA

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DEVELOPMENT OF NEUROPEPTIDE Y-IMMUNOREACTIVE NEURONS IN THE RAT retina

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DEVELOPMENTAL BRAIN RESEARCH 1989年第1期48卷 19-26页

作者： FERRIERO, DM SAGAR, SM VET ADM MED CTR SAN FRANCISCOCA 94122

Neuropeptide Y-like immunoreactivity (NPY-LI) was examined in the rat retina by radioimmunoassay and immunocytochemistry during prenatal and postnatal development. NPY-LI appears late in gestation (embryonic day [E18]), at which time it is present in small quantities (0.038 +/- 0.005 pm/mg protein) and the NPY-LI is confined to cells in the ganglion cell layer. The concentration of NPY-LI rises steadily over pre- and postnatal development; and on postnatal day 6 (P6), immunoreactive cells first appear in the inner nuclear layer. At eye opening (P13), there is a large increase in NPY-LI (0.207 +/- 0.035 pm/mg protein), and immunoreactive cells can be seen in the innermost row of the inner nuclear layer (INL) as well as in the ganglion cell layer (GCL). As the retina matures, the levels of NPY-LI fall to adult levels (0.080 +/- 0.019 pm/mg protein) and the peptide is confined to two subpopulations of cells, one in the INL and one in the GCL. The transient increase in NPY-LI at eye opening suggests that it may have a role at this time in modulating developing retinal circuitry. This pattern is very different from that of somatostatin-like immunoreactivity which appears earlier in development in high quantities and decreases prior to synaptogenesis and eye opening.

关键词： Development retina Neuropeptide Y Peptide Ontogeny Immunocytochemistry

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MOLECULAR-CLONING OF TOPAP - A TOPOGRAPHICALLY GRADED PROTEIN IN THE DEVELOPING CHICK VISUAL-SYSTEM

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NEURON 1995年第2期14卷 253-261页

作者： SAVITT, JW TRISLER, D HILT, DC UNIV MARYLAND SCH MEDDEPT BIOL CHEMBALTIMOREMD 21201 NHLBI BIOCHEM GENET LABBETHESDAMD 20892

Topographically graded molecules representing position-specific differences among otherwise similar cells are thought to play a role in the patterning of the developing nervous system. In the embryonic chick visual system, a 40 kDa protein, TOPAP, is expressed in a posterior > anterior gradient in the retina and in an inverted anterior > posterior gradient in the optic tectum, the major retinal projection area. Here we report the isolation and nucleotide sequencing of a complementary DNA clone encoding the chick TOPAP protein and demonstrate that the mRNA encoding this coiled-coil integral membrane protein is topographically graded within the retina and is present in a variety of chick tissues.

关键词： Amino Acid Sequence Animals Base Sequence Brain Chick Embryo Cloning Molecular Comparative Study DNA Primers Eye Proteins Gene Expression Membrane Proteins Molecular Sequence Data Molecular Weight Polymerase Chain Reaction RNA Messenger Recombinant Proteins Restriction Mapping retina Sequence Homology Amino Acid Subcellular Fractions Superior Colliculus

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Stereo Matching in Address-Event-Representation (AER) Bio-Inspired Binocular Systems in a Field-Programmable Gate Array (FPGA)

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ELECTRONICS 2019年第4期8卷 410-410页

作者： Dominguez-Morales, Manuel Dominguez-Morales, Juan P. Jimenez-Fernandez, Angel Linares-Barranco, Alejandro Jimenez-Moreno, Gabriel Univ Seville Robot & Comp Technol Lab E-41012 Seville Spain

In stereo-vision processing, the image-matching step is essential for results, although it involves a very high computational cost. Moreover, the more information is processed, the more time is spent by the matching algorithm, and the more inefficient it is. Spike-based processing is a relatively new approach that implements processing methods by manipulating spikes one by one at the time they are transmitted, like a human brain. The mammal nervous system can solve much more complex problems, such as visual recognition by manipulating neuron spikes. The spike-based philosophy for visual information processing based on the neuro-inspired address-event-representation (AER) is currently achieving very high performance. The aim of this work was to study the viability of a matching mechanism in stereo-vision systems, using AER codification and its implementation in a field-programmable gate array (FPGA). Some studies have been done before in an AER system with monitored data using a computer;however, this kind of mechanism has not been implemented directly on hardware. To this end, an epipolar geometry basis applied to AER systems was studied and implemented, with other restrictions, in order to achieve good results in a real-time scenario. The results and conclusions are shown, and the viability of its implementation is proven.

关键词： address-event-representation spike neuromorphic engineering stereo vision epipolar geometry dynamic vision sensors retina FPGA

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EDGE-CURVATURE DISCRIMINABILITY ARGUES AGAINST EXPLICIT CURVATURE DETECTORS

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JOURNAL OF THE OPTICAL SOCIETY OF AMERICA A-OPTICS IMAGE SCIENCE AND VISION 1995年第2期12卷 202-213页

作者： DEHAAN, E []Utrecht Biophysics Research Institute Faculty of Physics Astronomy Princetonplein 5 3584 CC Utrecht The Netherlands

Human discrimination of edge curvature was investigated over a 2-decade range of curvatures. The luminance profile of the edge pattern was either sharp or blurred on a scale that varied over a range of almost 2 decades. The experiments with curved blurred stimuli can also be regarded as investigations of contrast discrimination in which a blurred bar is superimposed orthogonally upon a blurred edge. The discrimination thresholds for the blurred stimuli show substantial size invariance, which can be explained in terms of contrast discrimination and masking with orthogonally oriented patterns rather than in terms of curvature per se. No such size invariance is found with sharp stimuli. All results can be described by a simple model based only upon local contrast.

关键词： Curvature Photometry retina Spatial frequency Visual perception Visual system

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The lipid intermediate pathway in the retina for the activation of carbohydrates involved in the glycosylation of rhodopsin

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Neurochemistry International 1980年第C期1卷 59-68页

作者： Kean, Edward L. Lorand V. Johnson Laboratory for Research in Ophthalmology Department of Surgery Division of Ophthalmology the Department of Biochemistry Cleveland OH 44106 United States

The presence of and many of the properties of enzymes in the retina involved in the biosynthesis of key intermediates in the dolichol pathway of carbohydrate activation has been demonstrated. The formation of dolichol phosphate-mannose and of mannose-containing oligosaccharide-polyprenols, and their use as substrates in further biosynthetic reactions was shown. The biosynthesis of the core-region glucosamine-containing mono, di and tri-saccharide pyrophosphoryl polyprenols, and the novel function of GDP-mannose as a regulator in their biosynthesis, was described. The participation of the dolichol pathway in the glycosylation of rhodopsin was demonstrated by the inhibition of core-region glycosylation of this glycoprotein by the antibiotic, tunicamycin. Preliminary evidence was obtained that glycosylation of rhodopsin was not a requirement for its insertion into disc membranes. © 1980.

关键词： carbohydrate-activation dolichol pathway dolichol phosphate-mannose glucosaminyl-polyprenols glycoprotein biosynthesis glycosylation Polyprenols retina rhodopsin

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