Abstract：
PURPOSE:To correlate retinal histopathology with functional changes caused by the rhodopsin Q64ter mutation. METHODS:A 50-year-old female heterozygote was evaluated clinically and with psychophysical and electroretinographic measurements of rod and cone function. The retinas obtained after death were examined microscopically, including immunolabeling with antibodies against the C- and N-termini of rhodopsin. RESULTS:On clinical examination 4 months before death, patient's acuity was 20/60, and she had midperipheral scotomas with retained function centrally and in the far periphery. The rod electroretinogram (ERG) was undetectable, and the cone ERG was reduced in amplitude with abnormal receptoral and postreceptoral responses. A previous study of the phenotype of mildly affected family members of the donor suggested that the rod outer segments (ROS) were shortened and that only wild-type rhodopsin was functional. The retinas contained only scattered cones in the midperiphery; the maculas and far peripheral regions contained reduced numbers of rods and cones with short to absent outer segments. The ROS appeared to contain wild-type, but not mutant, rhodopsin, and many peripheral rods had sprouted long rhodopsin-positive neurites that projected into the inner retina. Many cone synapses were abnormal, and the axons of some peripheral cones reached the inner plexiform layer. CONCLUSIONS:Microscopic changes in the donor retinas correlated well with the abnormalities in visual function in the patient donor and other family members. Postreceptoral ERG defects may relate to the abnormal photoreceptor processes found in the inner retina.
journal_name
Invest Ophthalmol Vis Scijournal_title
Investigative ophthalmology & visual scienceauthors
Milam AH,Li ZY,Cideciyan AV,Jacobson SGsubject
Has Abstractpub_date
1996-04-01 00:00:00pages
753-65issue
5eissn
0146-0404issn
1552-5783journal_volume
37pub_type
杂志文章abstract：:The retinal pigment epithelium (RPE) and photoreceptors of teleosts exhibit dramatic examples of cell motility (called retinomotor movements) in response to diurnal changes in lighting conditions. In darkness the pigment granules of the RPE migrate to the scleral base of the RPE cell and cone photoreceptors elongate. ...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1983-01-01 00:00:00
abstract：:Cone electroretinograms were elicited with a full-field red flash after-45 min of dark adaptation from 16 patients with retinitis pigmentosa and from 17 normal subjects. The average latency and implicit time of the cone a-waves recorded from the patients were each significantly delayed (p less than 0.005) compared wit...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1981-11-01 00:00:00
abstract：Purpose:We determine the feasibility of human conjunctival epithelial cells (hCjECs) on poly(lactic-co-glycolic) acid (PLGA) membranes for corneal epithelium regeneration. hCjECs on PLGA or polyester (PET) membranes with or without coculture of human Tenon's fibroblasts (hTFs) were compared in vitro, and to determine w...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.17-22719
更新日期：2018-03-01 00:00:00
abstract：PURPOSE:To present a new method of performing scanning laser ophthalmoscope perimetry that compensates for eye movements so that the correct retinal location is tested even if fixation changes. This allows for accurate testing of patients with central scotomas and for repeating testing longitudinally at the same retina...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1995-08-01 00:00:00
abstract：:Dog corneas were studied by indirect immunofluorescence with antibodies specific for pemphigus and pemphigoid antigens, laminin, and type IV collagen. The distribution of these antigens was similar to that observed in other squamous epithelia and skin. This study provides further evidence for close immunochemical simi...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1981-12-01 00:00:00
abstract：Purpose:Evidence from animals and blind humans suggests that early visual experience influences the developmental calibration of auditory localization. Hypothesizing that unilateral amblyopia may involve cross-modal deficits in spatial hearing, we measured the precision and accuracy of sound localization in humans with...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.18-24908
更新日期：2019-03-01 00:00:00
abstract：PURPOSE:The accessory lacrimal glands are assumed to contribute to the production of tear fluid, but little is known about their function. The goal of this study was to conduct an analysis of gene expression by glands of Wolfring that would provide a more complete picture of the function of these glands. METHODS:Gland...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.12-10750
更新日期：2012-10-01 00:00:00
abstract：PURPOSE:This study evaluated the role for poly(ADP-ribose) polymerase (PARP) in diabetes-induced cataractogenesis and early retinal changes. METHODS:Control and streptozotocin (STZ)-diabetic rats were treated with or without the PARP inhibitors 1,5-isoquinolinediol (ISO; 3 mg kg(-1) d(-1) intraperitoneally) and 10-(4-...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.08-2191
更新日期：2009-04-01 00:00:00
abstract：PURPOSE:Activation of complement has been implicated as one of the major causes of age-related macular degeneration (AMD). Evidence is accumulating for a role of complement in other retinal diseases, such as diabetic retinopathy and proliferative vitreoretinopathy. Because of the paucity of animal models that directly ...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.10-6002
更新日期：2011-05-18 00:00:00
abstract：PURPOSE:The purpose of the present study was to examine the roles of signaling pathways potentially activated by TGFbeta (i.e., Smad and p38 mitogen-activated kinase [MAPK]) in regulation of cell migration and proliferation of healing mouse corneal epithelium. METHODS:Activation of Smads or p38MAPK was evaluated by im...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.03-0700
更新日期：2004-01-01 00:00:00
abstract：:A method to quantify different glaucomatous visual field defects is presented. Three visual field indices are calculated: the short-term fluctuation, the mean defect, and the corrected loss variation. The method was applied to visual fields tested with program JO on the Octopus automated perimeter. The indices of 130 ...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1985-02-01 00:00:00
abstract：:The mitotic rate and goblet cell content of conjunctival epithelium following total or central corneal epithelial removal using n-heptanol was measured to determine how the conjunctival epithelium responds to injury and whether conjuctiva responds to central corneal epithelial loss. One day following a wound that remo...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1987-09-01 00:00:00
abstract：PURPOSE:Human tear film stability decreases with increasing age. In this study, the changes in meibum composition were measured in search of markers of tear film instability. METHODS:(1)H NMR nuclear magnetic resonance (NMR) spectra of 43 normal donors aged 1 to 88 years were acquired. RESULTS:Compared with meibum fr...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.11-8341
更新日期：2012-01-31 00:00:00
abstract：PURPOSE:To determine the interaction of M3 muscarinic receptors (M3AChR) and P2X(7) receptors to increase intracellular [Ca2+] ([Ca2+]i) and stimulate protein secretion in rat lacrimal gland cells. METHODS:Exorbital lacrimal glands from male Sprague-Dawley rats were divided into pieces or digested with collagenase to ...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.11-7210
更新日期：2011-05-01 00:00:00
abstract：Purpose:Global motion and global form perception have been found to be abnormal in the presence of amblyopia. How such deficits manifest in visual function reliant on the interactions between these two visual processing mechanisms has not been adequately explored. In the current study, we use dynamic Glass patterns (dG...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.61.5.58
更新日期：2020-05-11 00:00:00
abstract：Purpose:To investigate the chromatic characteristics and intensity-response function of light-induced reflex lacrimation and its correlation with the melanopsin-driven postillumination pupil response (PIPR). Methods:Eleven visually normal participants completed the experiment. Lacrimation was measured in one eye by pl...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.16-21285
更新日期：2017-03-01 00:00:00
abstract：PURPOSE:To investigate the discrepancy between grating and optotype visual acuity in children with visual impairment due to brain and/or ocular abnormalities. METHODS:Better eye acuity at 114 cm was studied in 81 patients (ages, 5-24 years) attending special schools for the visually handicapped. Fourteen patients had ...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.03-0822
更新日期：2004-12-01 00:00:00
abstract：:Treatment of human skin and corneal fibroblasts with prednisolone-21-phosphate did not increase the capacity of these cells to replicate type I herpes simplex virus (HSV). The steroid however was found to (1) inhibit human lymphocytes from mediating antibody-dependent cell-mediated cytotoxicity (ADCC) against HSV-infe...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1978-04-01 00:00:00
abstract：PURPOSE:Ibuprofen and indomethacin are nonselective prostaglandin synthetase inhibitors that have been shown to improve oxygen-induced retinopathy in mice. Vascular endothelial growth factor (VEGF), insulin-like growth factor (IGF)-I, and growth hormone (GH) are potent growth factors involved in retinal development. Th...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.06-0057
更新日期：2006-07-01 00:00:00
abstract：PURPOSE:To compare the mydriatic effect and the short-term corneal endothelial safety of intracamerally injected N-methyl-3,4-dihydroxyphenylamine (epinine) to phenylephrine in a porcine eye model. METHODS:One hundred twelve eyes from newly slaughtered pigs were used in this study. After pretreatment with 20 mg intrac...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.09-3651
更新日期：2009-11-01 00:00:00
abstract：:A monoclonal antibody has been produced that binds to the apical squames (flattened cells) of the rat ocular surface epithelium and to the goblet cells of the conjunctiva. Immunoelectron microscopic localization of the antigen indicates that in apical cells it is present along the apical-microplical membrane in the re...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1992-01-01 00:00:00
abstract：PURPOSE:Oxidative stress has been proposed as a major pathogenic factor in age-related macular degeneration (AMD), the leading cause of vision loss among elderly people of western European ancestry. Lutein (LUT) and zeaxanthin (ZEA), major components in macular pigment, are among the retinal antioxidants. Though xantho...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.07-0037
更新日期：2007-11-01 00:00:00
abstract：PURPOSE:Folic acid is essential for DNA, RNA, and protein synthesis, and deficiencies in folate can lead to nutritional amblyopia and optic neuropathy. The transport of folate from the choroidal blood supply to the retina is only now beginning to be understood. The reduced-folate transporter was reported recently to be...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1999-04-01 00:00:00
abstract：PURPOSE:To evaluate the feasibility of near-infrared (NIR) imaging acquisition in a large sample of consecutive pediatric patients with neurofibromatosis type 1 (NF1), to evaluate the diagnostic performance of NF1-related choroidal abnormalities as a diagnostic criterion of the disease, and to compare this criterion wi...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.14-16053
更新日期：2015-09-01 00:00:00
abstract：PURPOSE:To investigate the interobserver agreement for the detection of optical coherence tomography (OCT) features of disease activity in patients with neovascular age-related macular degeneration (nAMD). METHODS:This was a cross-sectional agreement study in which grading of OCT line scans from patients with nAMD was...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.09-3505
更新日期：2009-11-01 00:00:00
abstract：PURPOSE:Extracellular microRNAs (miRNAs) in aqueous humor were suggested to have a role in transcellular signaling and may serve as disease biomarkers. The authors adopted next-generation sequencing (NGS) techniques to further characterize the miRNA profile in single samples of 60 to 80 μL human aqueous humor. METHODS...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.15-17828
更新日期：2016-04-01 00:00:00
abstract：PURPOSE:To investigate the pharmacologic effect of tetramethylpyrazine (TMP) on human Tenon's fibroblasts (HTFs), the cells implicated in scarring after filtration surgery. METHODS:Transforming growth factor-β2 (TGF-β2) was used to stimulate a fibrotic phenotype in primary HTFs, and the influence of TMP on the fibroti...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.16-19529
更新日期：2016-09-01 00:00:00
abstract：PURPOSE:To explore the correlation between optical coherence tomography (OCT) and stereoscopic fundus photographs (FP) for the assessment of retinal thickening (RT) in diabetic macular edema (DME) within a clinical trial. METHODS:OCT, FP, and best corrected visual acuity (VA) measurements were obtained in both eyes of...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章,多中心研究,随机对照试验
doi：10.1167/iovs.07-1257
更新日期：2008-05-01 00:00:00
abstract：PURPOSE:The aim of this study was to evaluate a DNA dot hybridization assay (DHA) for assessing bacterial bioburden in orthokeratology lens (OK) storage cases. METHODS:Forty-one OK wearers participated in this study. The dot hybridization assay was used to assess the bacterial bioburden of OK after removal and 6-hour ...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.14-15920
更新日期：2014-12-23 00:00:00
abstract：PURPOSE:Fibrillins are the major constituent of tissue microfibrils, which form the ocular zonule. In Marfan syndrome (MFS), FBN1 mutations lead to ectopia lentis. The goal of this work was to investigate zonule composition and formation in fibrillin-deficient and wild-type mice. METHODS:Immunofluorescence staining of...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.13-13121
更新日期：2013-12-23 00:00:00
