electroretinography

..To use the global flash multifocal electroretinogram (mfERG) in patients with asymmetric glaucoma to determine whether retinal function is affected in fellow eyes that have no glaucomatous visual field defects...

To demonstrate the clinical feasibility of using surface electrodes for recording flash electroretinography (ERG), using the stimulation standards of the International Society for the Clinical Electrophysiology of Vision (ISCEV), without ..

..These 2007 guidelines, from the International Society for Clinical Electrophysiology of Vision (ISCEV: http://www.iscev.org ), replace the ISCEV guidelines for the mfERG published in 2003...

..The guidelines presented here are applicable to a wide range of investigations of retinal disease in rodent models...

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..Clinical Electrophysiology of Vision (ISCEV), presents an updated and revised ISCEV Standard for clinical electroretinography (ERG)...

..For therapeutic application to postmitotic tissues, this system substantially reduces the risk of insertional mutagenesis...

..These results lay the groundwork for the development of PDEβ-RP gene therapy trial and suggest that tyrosine-capsid mutant AAV vectors may be effective for treating other rapidly degenerating models of retinal degeneration...

..protect against insults to axons, strongly protects against DBA/2J glaucoma and preserves RGC activity as measured by pattern electroretinography. These experiments provide strong evidence for a local insult to axons in the optic nerve.

..With a size of 2 Mb, it is one of the largest human genes, and it is by far the largest retinal dystrophy gene. The discovery of EYS might shed light on a critical component of photoreceptor morphogenesis...

..Identification of TRPM1 as a mGluR6-coupled cation channel reveals a key step in vision, expands the role of the TRP channel family in sensory perception, and presents insights into the evolution of vertebrate vision...

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..To characterize the process by which overexpression of normal opsin leads to photoreceptor degeneration...

..Finally, we showed that detailed electroretinography is an effective way to discriminate among patients with mutations in either TRPM1 or GRM6, another ..

..However, an overall slower decay of retinal structure and function predicts that rd10 mice might become excellent models for rescue approaches...

..It may also provide a better model for experimental pharmaceutical-based therapy for RP because of its later onset and milder retinal degeneration than rd1 and nmf137...

..These data suggest that GABAC receptors determine the time course and extent of inhibition at bipolar cell terminals that, in turn, modulates the magnitude of excitatory transmission from bipolar cells to ganglion cells...

..It is speculated that a markedly diminished second-order kernel is diagnostic of outer plexiform layer damage, not inner plexiform layer damage as is commonly assumed...

..To describe the clinical and genetic characteristics of six Japanese families with Bietti's crystalline corneoretinal dystrophy (BCD)...

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..The purpose of this study was to test whether delivery of functional GC1 to cone cells of the postnatal GC1KO mouse could restore function to these cells...

..To study cone photoreceptor structure and function in patients with inherited retinal degenerations treated with sustained-release ciliary neurotrophic factor (CNTF)...

..To evaluate the involvement of NR2E3 in inherited retinal degenerative diseases in the Israeli and Palestinian populations and to study phenotypic variability in patients who are homozygous for the same mutation...

..To describe the phenotype of members of a large Caucasian British family affected by autosomal dominant cone-rod dystrophy due to an R838C mutation in the guanylate cyclase 2D (GUCY2D) gene encoding retinal guanylate cyclase-1 (RETGC-1)...

..These findings suggest an important role of this specific cation channel for the normal function of ON bipolar cells in the human retina...

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..autosomal-recessive congenital stationary night blindness (CSNB) and can be assessed by standard full-field electroretinography (ERG), showing severely reduced rod b-wave amplitude and slightly altered cone responses...

..an ophthalmic evaluation, which included a fundus examination, fluorescein angiography, full-field electroretinography (ERG), multifocal ERG, time-domain optical coherence tomography (TD-OCT), and visual field testing...

..Among patients on vitamin A for at least 2 years, a diet rich in omega-3 fatty acids (> or =0.20 g/d) slowed the decline in visual field sensitivity...

..To test the hypothesis that oxidative injury to the retinal pigment epithelium (RPE) may lead to retinal damage similar to that associated with the early stages of age-related macular degeneration (AMD)...

..However, vascular and neural abnormalities did not correlate...

..Furthermore, as the length of the abnormal amino acid sequence following ORF15 frameshift mutations increased, the severity of disease increased...

..To test AAV-mediated gene therapy in the rd10 mouse, a natural model of recessive RP caused by mutation of the beta-subunit of rod photoreceptor cGMP phosphodiesterase...

..Whether CNTF-induced changes in rods are through the same mechanism that mediates light-induced photoreceptor plasticity remains to be answered...

..The mechanisms for these side effects and proper doses of CNTF administration should be determined before human clinical trials are considered for the amelioration of inherited retinal degenerations with CNTF...

..In summary, in this study it was found that in Nob mice, postreceptoral neurons from the Off pathway make a positive-going contribution to the light-adapted flash ERG, and contribute substantially to sinusoidal flicker ERG...

..Our results also provide functional evidence that the feedback may be involved in temporal processing in the mammalian retina...

..Our data suggest that in visual information processing, shortening and shaping of light responses by activation of HCN1 at the level of the photoreceptors is an important step in both scotopic and photopic pathways...

..For the first time, high-resolution imaging provided in vivo evidence of RPE and photoreceptor involvement in ARB...

..To identify human transient receptor potential cation channel, subfamily M, member 1 (TRPM1) gene mutations in patients with congenital stationary night blindness (CSNB)...

..Although this technique is relatively new and standards are still being developed, centers capable of recording reliable mfERG responses can be found in hundreds of locations around the world...

..These studies reveal that, similar to rods, deletion of GCAP1 and GCAP2 delays the recovery of light responses in cones and GCAP1 restores the recovery of cone responses in the absence of GCAP2...

..We propose that changes at rod-RB synapses compensate for the partial loss of rod photoreceptors in senescence and in early stages of retinal degeneration...

..To report a novel mutation in the GUCY2D gene in a Japanese family with autosomal dominant cone-rod dystrophy (adCORD), and to examine the possible use of arrayed primer extension (APEX)-based genotyping chip in detecting mutations...

..CONCLUSIONS: The photopic hill in the primate ERG results mainly from two factors: the reduction of the ON-component amplitude at higher intensities and the delay in the positive peak of the OFF-component at higher intensities...

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..Treatments or genetic manipulations directed toward amelioration of proliferative retinopathy need to address not only the vascular changes but also the alterations in neuronal and macro- and microglial function...

..To describe two novel mutations in the eyes shut homolog (EYS) gene in two families with autosomal recessive retinitis pigmentosa (arRP) from Pakistan and Indonesia...

..To characterize the role of EYS, a recently identified retinal disease gene, in families with inherited retinal degenerations in the Israeli and Palestinian populations...

..These results demonstrate that GLAST is required for normal signal transmission between photoreceptors and bipolar cells and that both GLAST and GLT-1 play a neuroprotective role during ischemia in the retina...

..To evaluate the relation between refractive error and electrophysiological retinal abnormalities in children referred for investigation of reduced vision...

..To describe the clinical features and genetic analysis of a family with an autosomal dominant cone dystrophy (adCD)...

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..To establish mouse models for RPGR-associated diseases by generating and characterizing an Rpgr mutation (in-frame deletion of exon 4) in two different genetic backgrounds (BL/6 and BALB/c)...

..To evaluate the efficiency and safety of AAV-mediated gene delivery of a normal human ND4 complex I subunit in the mouse visual system...

..Low docosahexaenoic acid (DHA) in X-linked retinitis pigmentosa (XLRP) may influence retinal function. The goals of this study were to elevate blood DHA levels and determine the effect on the rate of disease progression...

..In the male patient, expression of both mutant alleles may compensate for the malfunction of each mutated protein and could explain why a milder form of BCD results from compound heterozygosity...

..Aiming towards an understanding of the molecular background of retinitis pigmentosa, this paper describes the phenotype of a Swedish family with a mutation in IMPDH1...

..Eyes were evaluated by flash electroretinography (ERG) and histologically at 3, 21, and 60 days after lasering...

..These data demonstrate the functional significance of melatonin and MT1 receptors in the mammalian retina and create the basis for future studies on the therapeutic use of melatonin in retinal degeneration...

..To characterize the phenotype of members of a Swedish family with Best macular dystrophy and two distinct mutations in VMD2...

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..The case below (enhanced S cone syndrome) is a good example of this, and demonstrates the diagnostic importance of clinical electrophysiology...

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..GFP. The discordance of functional and structural results, especially in the rat models, points to the need for a greater understanding of the mechanism of action of CNTF before human application can be considered...

..of short wavelength automated perimetry (SWAP), frequency doubling technology perimetry (FDT), and pattern electroretinography (PERG) in predicting progressive glaucomatous visual field defects on standard automated perimetry (SAP).

..To examine the clinical phenotype of three Swedish families with Best's vitelliform macular dystrophy (BMD) and three different mutations in the recently identified bestrophin gene...

..These results suggest that continuous administration of CNTF could potentially be useful for the treatment of some forms of retinal degeneration...

..To describe morphological and functional changes in a single patient with multifocal Best vitelliform macular dystrophy (BVMD) and to perform a genotype/phenotype correlation...

..Both groups of mice had, on average, comparable subnormal ERG amplitudes. CONCLUSIONS: D-cis-Diltiazem had no detectable effect on preservation of photoreceptor structure and function in rd mice...

..Caution needs to be exerted in extrapolation to the comparable human forms of RP...

..Therefore, both of these variables must be considered in prognostic counseling and subject recruitment for future therapeutic trials...

..To study the slow and fast rod signals of the scotopic 15-Hz flicker ERG in patients carrying mutations in the NYX gene, which has been recently identified as the cause of the complete form of congenital stationary night blindness, CSNB1...

..To describe the phenotypes of 5 patients with NR2E3 mutations...

..Here, a number of uses for the technique are demonstrated, with examples from human electroretinography. A simple relationship between the binary kernels extracted from a single experiment permits us to ..

..These findings may help avoid limitations, risks, and damage associated with subretinal injections currently necessary for clinical gene therapy...

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..To identify the genetic basis of a large consanguineous Spanish pedigree affected with autosomal recessive retinitis pigmentosa (arRP) with premature macular atrophy and myopia...

..The authors studied the function of local retinal areas in the posterior pole of patients with OMD using multifocal electroretinograms (ERGs)...

..To determine the clinical importance of the photopic negative response (PhNR) of the focal electoretinogram (fERG) for diagnosing glaucoma...

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..To describe clinical, electrophysiologic, and immunologic features of a unique paraneoplastic retinopathy with characteristics of cancer-associated and melanoma-associated retinopathy...

..The light-exposure pathology in this transgenic mouse model indicates an essential role for SOD I in the protection of photoreceptors from light-damage...

..No off-target expression was observed. Rs1-KO mice treated with this vector showed a decrease in the schisis cavities and had improved retinal signaling evaluated by recording the electroretinogram 11-15 weeks after the application...

..The impact of ocular ischemia on luminance and frequency-modulated contrast vision was compared with the function of outer retinal cells and the number of intact retinal ganglion cells (RGCs)...

..To investigate the relationship between the components of the first-order multifocal electroretinogram (M-ERG) and glaucomatous visual field loss...

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..5:1, 1.7:1, or 2.5:1 and were studied via light and electron microscopy, immunocytochemistry, electroretinography (ERG), and spectrophotometry. Retinas with transgenic opsin levels equivalent to one endogenous allele (G0...

..To investigate the effects of laser photocoagulation (LP)-induced ocular hypertension (OHT) on the survival and retrograde axonal transport of retinal ganglion cells (RGC), as well as on the function of retinal layers...

..To describe and characterize a novel, non-invasive method to induce retinal ischemia in the rat...

..To elucidate the phenotypic and biochemical characteristics of a novel mutation associated with autosomal dominant cone-rod dystrophy (adCORD)...

..To report the clinical and electrophysiological findings in a three-generation pedigree with autosomal dominant vitreoretinochoroidopathy...

..To better understand the variable expressivity of autosomal dominant cone degeneration, we studied a single, large family...

..To perform a detailed morphologic and functional evaluation of Best macular dystrophy (BMD) associated with mutations in the VMD2 gene...

..To study prospectively retinal function, color discrimination, and ocular toxicity in women treated with standard-dosage tamoxifen for breast cancer...

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..Therefore, we suggest that a more appropriate name for this syndrome is hypotrichosis with cone-rod dystrophy...

..To evaluate the retinal phenotype of 7- and 14-month-old apoB100,LDLR-/- mice, a relevant animal model of lipid metabolism dysfunction...

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..Safety of the treatment will be established on the basis of retinal imaging, electroretinography and histopathology...

..structural testing for Aim 1, RGC function will also be assessed in both eyes using three proven forms of electroretinography (Aim 3)...

..Retinal function will be determined by electroretinography. We currently have large colonies of both the tubby and the Trx-tubby mice and will be able to complete the ..

..Third, we will turn to functional assays using computerized pupillometry and electroretinography (ERG) to determine the ability of transplanted MSCs and locally released neurotrophic factors to restore ..

..these mice demonstrate a slow retinal degeneration characterized by a gradual loss of scotopic and photopic electroretinography (ERGs) that become very apparent at 4 months of age (-30% loss)...

..Retinal function will be determined by electroretinography. We currently have large colonies of both the tubby and the Trx-tubby mice and will be able to complete the ..

..We will monitor survival of photoreceptors using electroretinography and morphometry and will measure the activation of the ER stress and apoptosis using specific antibodies ..

..Paired-flash" analyses of cone-derived electroretinography (ERG) will be used to assess the effect of RGS9-1/GBeta5-L over-expression on cone phototransduction...

..structural testing for Aim 1, RGC function will also be assessed in both eyes using three proven forms of electroretinography (Aim 3)...

..These questions will be explored using intraocular injections of AAV vectors followed by functional (electroretinography and multifocal electroretinography) and structural (quantitative histology) analysis of the retina...

..therapeutic approaches more effective than single approaches; is functional rescue, which has been seen by electroretinography, a preservation of function or a recovery of function; what is the relationship between rod and cone ..

..these mice demonstrate a slow retinal degeneration characterized by a gradual loss of scotopic and photopic electroretinography (ERGs) that become very apparent at 4 months of age (-30% loss)...

..Mice with known non-retinal X-linked mutations will be studied with electroretinography to seek models of human sex-linked retinal disease...

..can prevent the rapid degeneration of cone photoreceptor cells as judged by fluorescence microscopy and electroretinography. The ability to preserve the viability of cone photoreceptor celis in those afflicted with LCA will be a ..

..Assays for activity include morphological analysis of retinal degeneration, quantitative mRNA studies, and electroretinography. We are testing the specific hypothesis that viral mediated retinal delivery of ribozymes to reduce ..

..restore function to and promoter survival of photoreceptors will be evaluated using visual behavior assays, electroretinography, fluorescent microscopic analyses of retinal whole mounts, and molecular and protein analyses techniques...

..restore function to and promoter survival of photoreceptors will be evaluated using visual behavior assays, electroretinography, fluorescent microscopic analyses of retinal whole mounts, and molecular and protein analyses techniques...

..Cav-1 null mice display reduced retinal function in Cav-1 null mice as indicated by electroretinography (ERG) that suggested at a photoreceptor defect...

..genetic manipulation of the retina, 4) retinas can be conveniently collected for molecular studies, and 5) electroretinography provides a rapid and informative test of retinal function in living animals...

..Using fundal photography, electroretinography, and light and electron microscopy, we will test the hypothesis that mutations of VLGR1 lead to visual ..

..genetic manipulation of the retina, 4) retinas can be conveniently collected for molecular studies, and 5) electroretinography provides a rapid and informative test of retinal function in living animals...

..genetic manipulation of the retina, 4) retinas can be conveniently collected for molecular studies, and 5) electroretinography provides a rapid and informative test of retinal function in living animals...

..will be evaluated histologically by measuring the thickness of the outer nuclear layer and functionally by electroretinography. Proteomics and DNA-microarrays will be used to identify genes and proteins differentially expressed ..

..contrast coding through the use of innovative and sophisticated testing methods, including psychophysics, electroretinography (ERG), and the visual evoked potential (VEP), that are intended to target specific visual subsystems that ..