Abstract：
PURPOSE:To describe the dark-adaptation (DA) functions in subjects with molecularly proven achromatopsia (ACHM) using refined testing conditions with a view to guiding assessment in forthcoming gene therapy trials. METHODS:The DA functions of nine subjects with ACHM were measured and compared with those of normal observers. The size and retinal location of the stimuli used to measure DA sensitivities were varied in four distinct testing condition sets, and the effect of altering these parameters assessed. RESULTS:In three of the four testing condition sets, achromats had significantly higher mean final thresholds than normal observers, whereas in the fourth condition set they did not. A larger, more central stimulus revealed the greatest difference between the final DA thresholds of achromat and normal subjects, and also demonstrated the slowest rate of recovery among the achromat group. CONCLUSIONS:In this, the largest study of DA functions in molecularly proven ACHM to date, we have identified optimal testing conditions that accentuate the relative difference between achromats and normal observers. These findings can help optimize DA testing in future trials, as well as help resolve the dichotomy in the literature regarding the normality or otherwise of DA functions in ACHM. Furthermore, the shorter testing time and less intense adaptation light used in these experiments may prove advantageous for more readily and reliably probing scotopic function in retinal disease, and be particularly valuable in the frequent post therapeutic assessments required in the context of the marked photophobia in ACHM.
journal_name
Invest Ophthalmol Vis Scijournal_title
Investigative ophthalmology & visual scienceauthors
Aboshiha J,Luong V,Cowing J,Dubis AM,Bainbridge JW,Ali RR,Webster AR,Moore AT,Fitzke FW,Michaelides Mdoi
10.1167/iovs.14-14910subject
Has Abstractpub_date
2014-08-28 00:00:00pages
6340-9issue
10eissn
0146-0404issn
1552-5783pii
iovs.14-14910journal_volume
55pub_type
杂志文章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:We evaluated whether UVA-riboflavin collagen cross-linking (CXL) increases transverse stromal shear moduli ex vivo, whether the shear moduli are greater in the anterior compared to the posterior stroma, and whether the shear moduli are affected by CXL. METHODS:The resistance to unidirectional transverse shear ...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.12-10710
更新日期：2013-07-26 00:00:00
abstract：PURPOSE:To demonstrate corneal endothelial (CE) integrity enhanced during eye banking by a brief treatment of human donor corneoscleral explant (explant) with CE autocrine trophic factor vasoactive intestinal peptide (VIP). METHODS:Paired explants were used as control versus VIP (10 nM)-treated before storage in corne...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.10-5983
更新日期：2011-07-29 00:00:00
abstract：PURPOSE:To conduct clinical and genetic studies in a European family with autosomal dominant Stargardt-like macular dystrophy (adSTGD-like MD) and to investigate the functional consequences of a novel ELOVL4 mutation. METHODS:Ophthalmic examination and mutation screening by direct sequencing of the ELOVL4 gene was per...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.04-0078
更新日期：2004-12-01 00:00:00
abstract：:Photoreceptor-mediated mechanisms were studied in patients with a recently identified retinopathy typified by night blindness, cystoid maculopathy, and similar scotopic and photopic electroretinograms (ERGs). Dark-adapted spectral sensitivity functions were only partly explained as composites of rod and cone curves sh...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1990-05-01 00:00:00
abstract：PURPOSE:To determine the metrics of vertical saccades in elderly subjects for comparison in neurologic disease. METHODS:Sixteen elderly subjects, ten middle-aged subjects, and thirteen young subjects were examined using a magnetic search coil technique. Saccades were measured to predictable vertical target steps and m...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1993-07-01 00:00:00
abstract：PURPOSE:Recovery of photoreceptor cells after light damage is thought to involve the physiologic process of disk renewal. However, only indirect evidence is available to support this hypothesis. The present study sought to examine more directly the mechanisms of photoreceptor cell recovery by quantitatively assessing t...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1994-08-01 00:00:00
abstract：:The visual field of normal infants was assessed using a perimeter with LED stimuli and a forced-choice observation procedure. Central fixation was elicited by four central, pulsing LEDs and maintained with the aid of auditory stimuli. Field extent was derived from the four-alternative, forced-choice judgments of an ad...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1988-03-01 00:00:00
abstract：Purpose:Extremely preterm infants are at increased risk for retinopathy of prematurity (ROP). We previously identified several inflammatory proteins that were expressed early in life and are associated with an increased risk of ROP and several angiogenic and neurotrophic growth factors in the neonatal systemic circulat...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章,多中心研究
doi：10.1167/iovs.18-26466
更新日期：2019-07-01 00:00:00
abstract：PURPOSE:To assess whether saccadic eye movements show distinct changes in patients with early active Graves' ophthalmopathy (GO), which could serve as a diagnostic tool for early detection and treatment. METHODS:Each of two prospective studies included 10 patients with early acute GO and 10 age- and sex-matched contro...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：2000-06-01 00:00:00
abstract：:Neuropeptide Y (NPY) is a recently discovered, amidated 36 amino acid residue neuropeptide present in many but not all sympathetic noradrenergic neurons. In the guinea-pig eye, NPY immunoreactive fibers were found to have the same distribution as noradrenergic fibers except that there were fewer at the iris dilator, i...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1984-10-01 00:00:00
abstract：PURPOSE:To assess corneal epithelial cell survival after keratoplasty. METHODS:Corneal impression cytology (CIC) was performed on sex-mismatched corneal transplants. Fluorescent in situ hybridization (FISH) with sex chromosome-specific probes was performed to identify epithelial cell mosaicism and therefore allocate t...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.10-5394
更新日期：2011-02-22 00:00:00
abstract：PURPOSE:To characterize the molecular properties, cellular distribution, and subcellular distribution of bovine rom-1 and its interaction with peripherin/rds in photoreceptor cells as an important step toward elucidating the role of rom-1 in photoreceptor outer segment structure-function relationships and in inherited ...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1996-02-01 00:00:00
abstract：PURPOSE:To use a theoretical model of the observer on the Farnsworth-Munsell 100-hue test to estimate the magnitude of random variation in 100-Hue test error scores. METHODS:The model was based upon classical signal detection theory. Results from the model were obtained by computer simulation. RESULTS:There is a fair...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1993-03-01 00:00:00
abstract：PURPOSE:To characterize retinal functional consequences of elevated intraocular pressure (IOP) in a rat model of experimental glaucoma. METHODS:Unilateral elevation of IOP was produced by hypertonic saline injection into an episcleral vein in 20 adult male Brown-Norway rats. IOP was measured in both eyes of awake anim...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.03-1411
更新日期：2004-06-01 00:00:00
abstract：:Tendon and cornea of 17-day-old chick embryos primarily synthesize type I collagen as the major collagenous component nd type V collagen as a minor collagenous component. Matrix-free cells isolated from cornea and tendon synthesize and secrete mainly type I procollagen. A short lag period for the secretion of collagen...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1982-12-01 00:00:00
abstract：PURPOSE:One of the methods used in macular translocation (MT) surgery for subfoveal neovascularization is to create a temporary total retinal detachment followed by a 360 degrees retinotomy. The whole retina is then shifted from the original surface of the retinal pigment epithelium (RPE), resulting in an unusual retin...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：2002-02-01 00:00:00
abstract：PURPOSE:There are two human 15-lipoxygenases (LOX), 15-LOX-1 and -2, which convert arachidonic acid to 15S-hydroxyeicosatetraenoic acid (15S-HETE). The presence of both 15-LOXs in the human cornea prompted this study to delineate their roles in the human corneal epithelium. METHODS:Human corneal epithelia from donor c...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.04-1166
更新日期：2005-03-01 00:00:00
abstract：PURPOSE:To examine the involvement of p27(KIP1) in the regulation of the proliferation of the developing corneal endothelium. METHODS:Central and peripheral corneas in C57Bl6 mice at postnatal day (P)1, P11, and 12 weeks after birth were analyzed by immunocytochemistry with anti-p27(KIP1), -p57(KIP2), and -proliferati...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.03-1238
更新日期：2004-07-01 00:00:00
abstract：PURPOSE:To develop a novel in vitro model to study the formation of Staphylococcus epidermidis biofilm on intraocular lenses (IOLs) from the primary-attachment phase to the biofilm-accumulation phase. The model was designed to replicate intraocular conditions especially by taking into account intraocular hydrodynamics....
journal_title：Investigative ophthalmology & visual science
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doi：10.1167/iovs.05-1070
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abstract：Purpose:Mutations in the retinitis pigmentosa-1-like-1 (RP1L1) gene are the major cause of autosomal dominant occult macular dystrophy (OCMD), while recessive mutations have been linked to autosomal recessive retinitis pigmentosa (arRP). We present the clinical phenotype of a large German OCMD cohort, as well as four R...
journal_title：Investigative ophthalmology & visual science
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doi：10.1167/iovs.18-24033
更新日期：2018-06-01 00:00:00
abstract：PURPOSE:The endothelium produces nitric oxide and endothelin (ET). This study was designed to investigate the endothelium-dependent regulation of the porcine ophthalmic microcirculation. METHODS:Isolated porcine eyes were perfused with a modified Langendorff setup (Hugo Sachs Elektronik KG, Freiburg, Germany) at a per...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1993-12-01 00:00:00
abstract：:In the tree shrew retina individual rod and cone photoreceptors can be readily identified and quantified because their perikarya are arranged in a single layer. This retina is therefore an ideal system for testing the specificity of photoreceptor-directed antibodies. Here we describe the staining properties of polyclo...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1989-03-01 00:00:00
abstract：:Fibroblasts in the retroocular connective tissue appear to play a central role in the pathogenesis of Graves' ophthalmopathy (GO). We hypothesize that specific attributes, differentiating normal from GO retroocular fibroblasts, may render the latter more vulnerable to the ongoing immunopathological process in GO. We i...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1992-02-01 00:00:00
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：Purpose:To examine the coaggregation and cohesion between the commonly isolated bacteria from contact lens cases. Methods:Four or five strains each of commonly isolated bacteria from contact lens cases, Staphylococcus aureus, Staphylococcus epidermidis, Pseudomonas aeruginosa, and Serratia marcescens, were grown, wash...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.16-20593
更新日期：2017-01-01 00:00:00
abstract：PURPOSE:To investigate the patterns of retinal nerve fiber layer (RNFL) defects in mean deviation-matched early glaucomatous eyes with either superior or inferior visual hemifield loss. METHODS:Seventy-five open-angle glaucoma patients with isolated parafoveal scotoma (PFS) within a central 10° of fixation, and 62 pat...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.13-12344
更新日期：2013-10-23 00:00:00
abstract：:Heightened interest in meibomian glands dysfunction prompted the authors to examine the ultrastructure of the glandular epithelium in specimens of surgical origin, by thin section and freeze-fracture electron microscopies. In meibomian glands, the morphology and ultrastructure of acinar cells varies considerably accor...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1992-06-01 00:00:00
abstract：PURPOSE:To assess the effects of diabetes mellitus on corneal structure and function. METHODS:The authors measured endothelial permeability to fluorescein and corneal deswelling for 7.5 hours after 2 hours of hypoxic contact lens wear in 20 patients with diabetes who had nonproliferative retinopathy and 21 age-matched...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1995-03-01 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
