Abstract:
:Visual thresholds and rhodopsin levels were determined in nine subjects with autosomal dominant retinitis pigmentosa. The subjects fell into two groups, corresponding to two subtypes of the disease revealed by two-color, dark-adapted static perimetry. In the first of these subtypes, rod-mediated function was variably reduced and was accompanied by a corresponding reduction in cone function in the same retinal region. Dark-adapted threshold elevations varied in a way consistent with decreased quantal absorption by the rods as a result of reduced rhodopsin levels. In the second subtype, rod function was greatly reduced or absent throughout the retina, while cone function was much less severely affected. Although the levels of rhodopsin were only about half of normal, they were much too great to account for the visual threshold elevations on the basis of decreased probabilities of absorption by the visual pigment. Rhodopsin regeneration appeared to follow normal kinetics in patients from both groups. The results indicate that the examples of the two psychophysical subtypes of AD RP investigated here have very different disease manifestations.
journal_name
Invest Ophthalmol Vis Scijournal_title
Investigative ophthalmology & visual scienceauthors
Kemp CM,Jacobson SG,Faulkner DJsubject
Has Abstractpub_date
1988-08-01 00:00:00pages
1235-41issue
8eissn
0146-0404issn
1552-5783journal_volume
29pub_type
杂志文章abstract::Retinal cone photoreceptors are polarized cells with discrete functional compartments. Little is known of the membrane-skeleton components in cones responsible for the maintenance of cellular morphology and compartmentalization of integral membrane proteins. The authors describe a high density of the membrane-skeleton...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1991-01-01 00:00:00
abstract:PURPOSE:Angiogenesis, the formation of new capillary blood vessels, is an essential biological process under physiological conditions, including embryonic development, reproduction, and wound repair. Under pathologic conditions, this process plays a critical role in a variety of diseases such as cancer, rheumatoid arth...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.07-1527
更新日期:2008-09-01 00:00:00
abstract::Using a freezing point depression method, requiring volumes of only 200 nanoliters, the osmolalities of 324 tear samples collected at consecutive 10-minute intervals throughout 8.5-hour periods from each of six healthy young adults were measured. The population mean was found to be 318 mOsm/kg, with a population media...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1983-12-01 00:00:00
abstract:PURPOSE:Retinal dopamine deficiency is a potential cause of myopia and visual deficits in retinopathy of prematurity (ROP). We investigated the cellular mechanisms responsible for lowered levels of retinal dopamine in an oxygen-induced retinopathy (OIR) mouse model of ROP. METHODS:Retinopathy was induced by exposing m...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.16-19346
更新日期:2016-06-01 00:00:00
abstract:PURPOSE:7-Ketocholesterol is a highly toxic oxysterol found in abundance in atherosclerotic plaques and is believed to play a critical role in atherosclerosis. The purpose of this study was to identify and localize 7-ketocholesterol (7kCh) in the primate retina and to examine the potential consequences of its presence ...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.08-2373
更新日期:2009-02-01 00:00:00
abstract:PURPOSE:Glaucoma is an optic neuropathy commonly associated with elevated intraocular pressure (IOP), leading to optic nerve head (ONH) cupping, axon loss, and apoptosis of retinal ganglion cells (RGCs), which could ultimately result in blindness. Brn3b is a class-4 POU domain transcription factor that plays a key role...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.14-15008
更新日期:2015-01-13 00:00:00
abstract:PURPOSE:Recent studies have suggested the involvement of nerve growth factor (NGF) in the conjunctival inflammatory process and in corneal epithelium proliferation and differentiation. To verify the hypothesis that NGF could locally modulate the inflammatory and reparative processes, the authors evaluated the expressio...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1998-06-01 00:00:00
abstract::Epidermal growth factor (EGF), which has been shown to stimulate epidermal proliferation and keratinization and to induce regeneration of rabbit corneal epithelium, was studied for its immunogenic potential in rabbits. Mouse-derived EGF was administered topically, subconjunctivally, intrastromally, and systemically. S...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1981-01-01 00:00:00
abstract:PURPOSE:The sense of vision in humans is robust, and visual flickering is rarely experienced. To investigate this mechanism, electrophysiological and molecular biological techniques were used on human cone and rod photoreceptors. METHODS:Voltage-gated currents were recorded using the patch-clamp technique on isolated ...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.04-0724
更新日期:2005-01-01 00:00:00
abstract::Bovine corneal endothelial cells in culture synthesize predominantly type III collagen, unlike rabbit corneal endothelial cultures which synthesize type IV collagen. In an attempt to document whether this type III collagen synthesis by bovine cells is a tissue culture-specific phenomenon, collagens synthesized by orga...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1988-02-01 00:00:00
abstract::Exposure of adult male and female albino rats to high-intensity illumination for 17.5 hr results in a marked, transient exophthalmos, which persists for approximately 48 hr after the light exposure. Upon examination of the Harderian glands in these rats, a significant glandular weight increase was observed up to 2 day...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1979-12-01 00:00:00
abstract:PURPOSE:To determine the involvement of calpain in ovine cataractogenesis by measuring calcium, calpain activity, proteolysis, and the effect of calpain inhibition. METHODS:Sheep with genetic cataracts were examined for cataract severity. Calcium in normal and cataract lenses was measured. The presence of calpain was ...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.04-1291
更新日期:2005-12-01 00:00:00
abstract:PURPOSE:Linkage intervals for erosive vitreoretinopathy (ERVR) and Wagner disease previously were found to overlap at 5q14.3. In a Japanese family with Wagner disease, a CSPG2/Versican splice site mutation (c.4004-2A-->G) was recently reported that resulted in a 39-nucleotide exon 8 in-frame deletion. We investigated w...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.06-0141
更新日期:2006-08-01 00:00:00
abstract::Two distinct patterns of delayed-onset, cell-mediated hypersensitivity have been induced in the guinea pig eye with the same soluble protein antigen and two different methods of immunization. The predominant histologic feature of these reactions was the large number of eosinophils which infiltrated the uveal tract and...
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:Children with idiopathic infantile nystagmus (IIN) exhibit visual acuity deficits that have been modeled in terms of foveation characteristics of the nystagmus waveform. Here we present evidence for an additional component of acuity loss associated with the deprivation experienced during the sensitive period of...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.13-11992
更新日期:2013-05-01 00:00:00
abstract:PURPOSE:To compare the magnification properties of four different indirect double aspheric fundus examination lenses for clinical disc biometry. METHODS:Experimental study in a model eye. The relationship between the true size of a fundus object and its image was calculated for each fundus lens for an ametropic range ...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:2001-01-01 00:00:00
abstract:PURPOSE:To assess the postural stability and gait characteristics of adults with age-related maculopathy (ARM) and to identify the visual factors associated with postural stability and gait in this clinical population. METHODS:Participants included 80 individuals with a range of severity of ARM (mean age, 77.2 years)....
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.08-1942
更新日期:2009-01-01 00:00:00
abstract:PURPOSE:To determine the effects of overexpression of mutated BIGH3 in HeLa and human corneal epithelial (HCE) cells. METHODS:Six mutations known to be responsible for autosomal dominant corneal dystrophies linked to chromosome 5 were generated in a BIGH3 expression vector and transfected in HeLa and HCE cells. The ex...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.02-0661
更新日期:2003-07-01 00:00:00
abstract:PURPOSE:Lysophosphatidic acid (LPA) is a phospholipid growth factor that stimulates proliferation, chemotaxis, cation currents, and K(+) currents in retinal pigment epithelial (RPE) cells. LPA receptor transduction was analyzed in human and rat RPE cells. METHODS:Cells were cultured with standard methods, and signalin...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:2002-07-01 00:00:00
abstract:PURPOSE:FOXC1 mutations result in Axenfeld-Rieger syndrome, a disorder characterized by a broad spectrum of malformations of the anterior segment of the eye and an elevated risk for glaucoma. A novel FOXC1 W152G mutation was identified in a patient with aniridia. Molecular analysis was conducted to determine the functi...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.08-3032
更新日期:2009-08-01 00:00:00
abstract:PURPOSE:To investigate the potential use of amniotic membrane transplantation (AMT) in the construction of glaucoma filtering blebs. METHODS:Twenty-four albino rabbits underwent glaucoma filtration surgery in one eye. In alternate cases, the conjunctival flap was replaced with AMT. Postoperative examination data were ...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:2001-07-01 00:00:00
abstract:PURPOSE:Ion channels in the ciliary epithelium play critical roles in the formation of aqueous humor in the eye. The present study identified a novel, swelling-activated K(+) current in freshly dissociated porcine pigmented ciliary epithelial cells. METHODS:Ciliary epithelial cells were freshly dissociated from porcin...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.11-7173
更新日期:2011-07-29 00:00:00
abstract:PURPOSE:Methionine-sulfoxide reductases are unique, in that their ability to repair oxidized proteins and MsrA, which reduces S-methionine sulfoxide, can protect lens cells against oxidative stress damage. To date, the roles of MsrB1, -B2 and -B3 which reduce R-methionine sulfoxide have not been established for any mam...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.05-0018
更新日期:2005-06-01 00:00:00
abstract:PURPOSE:The objective of the study was to investigate the effect of glial cell line-derived neurotrophic factor (GDNF) on the multifocal electroretinogram (mfERG) following an induced branch retinal vein occlusion (BRVO) in pigs. METHODS:Electrophysiological examination of the retina was performed in 20 pigs with stan...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.12-10110
更新日期:2012-09-14 00:00:00
abstract:PURPOSE:Evidence suggests that capillary degeneration in early diabetic retinopathy results from chronic inflammation, and leukotrienes have been implicated in this process. The authors investigated the cellular sources of leukotriene biosynthesis in diabetic retinas and the effects of hyperglycemia on leukotriene prod...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.09-3557
更新日期:2010-03-01 00:00:00
abstract:PURPOSE:To investigate modifications with aging of the presence, distribution and morphologic features of conjunctiva-associated lymphoid tissue (CALT) in healthy human subjects using laser scanning in vivo confocal microscopy (IVCM). METHODS:A total of 108 (age range, 17-75 years) subjects were enrolled. In vivo conf...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.14-14365
更新日期:2014-07-29 00:00:00
abstract::Reaching a consensus in terms of interchangeability and utility (i.e., disease detection/monitoring) of a medical device is the eventual aim of repeatability and agreement studies. The aim of the tolerance and relative utility indices described in this report is to provide a methodology to compare change in clinical m...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.14-16091
更新日期:2015-08-01 00:00:00
abstract:PURPOSE:The purpose of this study was to evaluate the performance of optical coherence tomography angiography (OCTA) in visualizing laser-induced choroidal neovascularization (CNV) in the rodent retina. METHODS:Choroidal neovascularization was induced via laser photocoagulation in 2 male Brown Norway rats and 2 male C...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.15-18946
更新日期:2016-07-01 00:00:00
abstract::PURPOSE. The purpose of the study was to investigate the effects of hyperglycemia on EGFR (epidermal growth factor receptor)-mediated wound response and signal transduction in the corneal epithelium of rats with type I diabetes mellitus (DM). METHODS. Corneal epithelia were removed from streptozotocin (STZ)- and weigh...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.10-5670
更新日期:2011-05-17 00:00:00
abstract:PURPOSE:Activity in regions of the visual cortex corresponding to central scotomas in subjects with macular degeneration (MD) is considered evidence for functional reorganization in the brain. Three unresolved issues related to cortical activity in subjects with MD were addressed: Is the cortical response to stimuli pr...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.09-4926
更新日期:2010-12-01 00:00:00