Abstract:
:The sequential electrophysiologic and pathologic changes in the retina in ceroid-lipofuscinosis were recorded in a time-course study in the ovine model. Over a relatively short period in the course of the disease, a severe reduction in both rod and cone b-wave amplitudes developed with rod b-wave changes preceeding those of cones. These changes paralleled a similar loss of rod and cone photoreceptor cells. In affected retinas, outer segments appeared shorter than normal. By 84 weeks of age, the outer nuclear layer was reduced to the width of a single nucleus. In addition to these changes, electronmicroscopy showed the formation of abnormal dystrophic rod and cone outer segments in photoreceptor cells. Most cells in the retina showed the accumulation of a fluorescent lipopigment, this being most prominent in ganglion cells. Ultrastructural studies showed them to be made of electron dense granular material and a variety of membranous and tubular arrays.
journal_name
Invest Ophthalmol Vis Scijournal_title
Investigative ophthalmology & visual scienceauthors
Graydon RJ,Jolly RDsubject
Has Abstractpub_date
1984-03-01 00:00:00pages
294-301issue
3eissn
0146-0404issn
1552-5783journal_volume
25pub_type
杂志文章abstract:PURPOSE:Trabecular meshwork (TM) cells appear to sense changes in intraocular pressure (IOP) as mechanical stretching. In response, they make homeostatic corrections in the aqueous humor outflow resistance, partially by increasing extracellular matrix (ECM) turnover initiated by the matrix metalloproteinases. To unders...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.05-0075
更新日期:2005-08-01 00:00:00
abstract:PURPOSE:To investigate in vitro the cell toxicity and antioxidant effects of two major tear substitutes, hyaluronic acid and a widely used carbomer, with and without preservative. METHODS:Chang conjunctival cells were treated with different concentrations of unpreserved or preserved carbomer 934P (0.03% and 0.3%), unp...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:2002-11-01 00:00:00
abstract::Although aqueous outflow is most likely a passive, nonenergy-dependent process, alterations in cellular function in the trabecular meshwork presumably are involved in the development of some types of glaucoma. Accordingly, it seems important to define both the normal and abnormal biochemistry of this tissue. The autho...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1984-11-01 00:00:00
abstract:Purpose:Our goal was to describe the clinical and molecular genetic findings in Thai patients with Leber congenital amaurosis (LCA). Methods:Whole exome sequencing (WES) was performed in eight unrelated patients. All genes responsible for inherited retinal diseases (IRDs) based on RetNet were selected for analysis. Po...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.16-21322
更新日期:2017-04-01 00:00:00
abstract:PURPOSE:To determine whether anthocyanin-enriched bilberry extracts modulate pre- or posttranslational levels of oxidative stress defense enzymes heme-oxygenase (HO)-1 and glutathione S-transferase-pi (GST-pi) in cultured human retinal pigment epithelial (RPE) cells. METHODS:Confluent ARPE-19 cells were preincubated w...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.06-0452
更新日期:2007-05-01 00:00:00
abstract:PURPOSE:To evaluate interictal visual dysfunction in persons with migraine in terms of spatiotemporal selectivity and location within the visual pathways. METHODS:The vision of a group of 15 persons who had experienced migraine with aura was compared with that of 15 normal age-matched control subjects. A range of thre...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:2001-03-01 00:00:00
abstract:PURPOSE:In the studies of spatial integration an increase in the spatial extent of the stimulus usually results in a decrease in the spatial frequency bandwidth of the stimulus. The authors investigated separately the effects of these two factors on contrast detectability. METHODS:Efficiencies were measured for a circ...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1994-06-01 00:00:00
abstract::Liposomes 0.5 to 5 micron in diameter and of various phospholipid compositions were used as a simplified model of rod outer segment membranes to examine the phagocytic mechanism of cultured explants of retinal dystrophic Royal College of Surgeons (RCS) and normal Long-Evans (LE) rat pigment epithelium. RCS pigment epi...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1981-10-01 00:00:00
abstract:PURPOSE:Using transient ischemia followed by reperfusion (IR) to model ischemic retinal disease, this study compares the effects of ischemic preconditioning (IPC) and therapies targeting vascular endothelial growth factor (VEGF) and tumor necrosis factor (TNF)-α on retinal apoptosis, vascular permeability, and mRNA exp...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.10-5264
更新日期:2010-11-01 00:00:00
abstract:PURPOSE:This was a prospective case-control study conducted to evaluate the effectiveness of the Tear Stability Analysis System (TSAS) for the assessment of patients with dry eye. METHODS:The TSAS can take 10 consecutive corneal topograms at one per second for 10 seconds. Examinations using the TSAS were conducted in ...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.03-0712
更新日期:2004-05-01 00:00:00
abstract:PURPOSE:To quantitate in vivo retinal ganglion cell (RGC) survival after transient periods of pressure-induced ischemia of the rat retina and after different survival intervals. METHODS:In adult rats, RGCs were labeled with fluorogold applied to their main targets in the brain. Seven days later, in several groups of r...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1996-09-01 00:00:00
abstract:PURPOSE:To estimate gender- and age-specific prevalence of myopia and identify risk indicators for myopia in a population-based sample of Latino adults aged 40 years and older in La Puente, California. METHODS:Noncycloplegic automated refraction with supplemental standardized subjective refraction was performed for pr...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.05-1153
更新日期:2006-05-01 00:00:00
abstract:PURPOSE:Keratoconus is characterized by thinning and scarring of the central region of the cornea. Previous research showed that, in corneas obtained from patients with keratoconus, lysosomal enzyme activities are elevated, whereas levels of protease inhibitors such as alpha1-proteinase inhibitor are reduced. This stud...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1998-06-01 00:00:00
abstract:PURPOSE:Connexin50 (Cx50) is absolutely essential for normal postnatal lens growth. Deletion of Cx50 or replacement with Cx46 by knockin resulted in smaller lenses containing fewer cells. To determine why Cx50-deficient lenses fail to grow normally, cell proliferation was assayed during the period of growth failure. M...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.04-0194
更新日期:2004-09-01 00:00:00
abstract:PURPOSE:Reports of lymphatics in the posterior human uvea are contradictory. We systematically analyzed the choroid by combining various lymphatic markers, following recently established guidelines for the immunohistochemical detection of ocular lymphatics. METHODS:Human choroids were prepared for flat mount serial cr...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.15-17883
更新日期:2015-11-01 00:00:00
abstract::The transport rates of radiolabeled ascorbic acid and dehydroascorbic acid, as well as, labeled 3-O-methyl-D-glucose and L-glucose from a central plasma compartment into aqueous and vitreous humors and cerebrospinal fluid were studied in vivo. Normal, male albino Sprague-Dawley rats and English Short Haired guinea pig...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1989-11-01 00:00:00
abstract:PURPOSE:Keratoconus is a progressive disorder of the cornea that can lead to severe visual impairment or blindness. Although several genomic regions have been linked to rare familial forms of keratoconus, no genes have yet been definitively identified for common forms of the disease. METHODS:Two genome-wide associatio...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.11-8261
更新日期:2011-10-31 00:00:00
abstract:PURPOSE:Allopregnanolone is a neurosteroid and powerful modulator of neuronal excitability. The neuroprotective effects of allopregnanolone involve potentiation of γ-aminobutyric acid (GABA) inhibitory responses. Although glutamate excitotoxicity contributes to ganglion cell death in glaucoma, the role of GABA in glauc...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.14-15624
更新日期:2014-11-18 00:00:00
abstract:PURPOSE:Bevacizumab eyedrops inhibit corneal neovascularization. The purpose of this study was to analyze the safety profile of VEGF-A neutralization at the ocular surface. METHODS:Bevacizumab eyedrops (5 mg/mL) and an antimurine VEGF-A antibody (250 microg/mL) were applied to normal murine corneas five times a day fo...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.07-1129
更新日期:2009-05-01 00:00:00
abstract:PURPOSE:To assess the impact of vitamin A supplementation at 4-month intervals on the prevalence and incidence of xerophthalmia among preschool-age children. METHODS:A stratified, random sample of 40 wards with 4766 children in Sarlahi district of Nepal was selected to participate in a randomized, controlled, communit...
journal_title:Investigative ophthalmology & visual science
pub_type: 临床试验,杂志文章,随机对照试验
doi:
更新日期:1995-12-01 00:00:00
abstract:PURPOSE:To present a detailed phenotypic and molecular study of a series of 18 patients from 11 families with retinal dystrophies consequent on mutations in the cone-rod homeobox (CRX) gene and to report a novel phenotype. METHODS:Families were ascertained from a tertiary clinic in the United Kingdom and enrolled into...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.14-14715
更新日期:2014-09-30 00:00:00
abstract:PURPOSE:To perform a mutation screening of TACSTD2 in 13 Iranian Gelatinous Drop-like Corneal Dystrophy (GDLD) pedigrees. To assess genotype-phenotype correlations. To determine intragenic SNP haplotypes associated with the mutations, so as to gain information on their origin. METHODS:The coding region of TACSTD2 was ...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.07-0264
更新日期:2007-10-01 00:00:00
abstract:PURPOSE:To study the aggregation of bovine gamma-crystallins in aqueous solutions and the effect of gamma(s)-crystallin on the aggregation of other gamma-crystallins. METHODS:Aggregation in aqueous solutions of gamma(s)-, gammaII-, and gammaIVa-crystallin was monitored by quasielastic light scattering. Aggregation in ...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1998-08-01 00:00:00
abstract:PURPOSE:To investigate the effect of amino acids on ornithine cytotoxicity in ornithine-delta-aminotransferase (OAT)-deficient human retinal pigment epithelial (RPE) cells as an in vitro model of gyrate atrophy (GA) of the choroid and retina. METHODS:RPE cells were treated with 0.5 mM 5-fluoromethylornithine (5-FMOrn)...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.03-0403
更新日期:2003-11-01 00:00:00
abstract:PURPOSE:To demonstrate the conjunctival alterations in patients with Sjögren's (SSDE) and non-Sjögren's syndrome dry eye (NSSDE) using a new generation confocal microscope (HRTII/ RCM; Heidelberg Engineering, Heidelberg, Germany), in a prospective controlled study. METHODS:Twenty-eight right eyes of 28 patients with S...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.08-2722
更新日期:2010-01-01 00:00:00
abstract::Neodymium: yttrium aluminum garnet (Nd:YAG) laser transscleral cyclophotocoagulation has been shown to be an effective method of lowering intraocular pressure (IOP). Transmission and absorption features of diode laser radiation (810 nm) make these new laser sources suitable for production of transscleral thermal lesio...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1991-04-01 00:00:00
abstract:PURPOSE:To investigate the retinal-protective effects of the oral iron chelator deferiprone (DFP) in mice lacking the iron regulatory hormone hepcidin (Hepc). These Hepc knockout (KO) mice have age-dependent systemic and retinal iron accumulation leading to retinal degeneration. METHODS:Hepc KO mice were given DFP in ...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.14-14568
更新日期:2014-06-26 00:00:00
abstract:PURPOSE:To characterize age-related changes of retinal ganglion cell (RGC) function, IOP, and anatomical markers of axon/glia integrity in a transgenic mouse expressing Tyr437His mutant of human myocilin protein. METHODS:Retinal ganglion cell electrical responsiveness was tested with pattern electroretinogram (PERG) i...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.14-14793
更新日期:2014-08-14 00:00:00
abstract:PURPOSE:To study the diameter response of retinal arterioles as a function of the arteriolar diameter. METHODS:Ten healthy young volunteers aged 22 to 33 years were subjected to diameter measurement of four successive segments of a retinal arteriole with a retinal vessel analyzer (RVA). At each of the segments, the di...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.06-0360
更新日期:2007-01-01 00:00:00
abstract:PURPOSE:The efficacy of the peptide Ac-EEED on reducing cell adhesion and proliferation in vitro and choroidal neovascularization (CNV) in vivo was examined. METHODS:The peptide chimera containing the Ac-EEED sequence was chemically linked to the N terminus of the XMTM delivery peptide from the E(rns) viral surface pr...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.10-6351
更新日期:2011-04-16 00:00:00