Abstract:
Purpose:To ascertain cellular constituents within islands of preserved retina in choroideremia (CHM) by multimodal imaging. Methods:CHM probands (16) and female carriers (9) of CHM were studied. Near-infrared autofluorescence (NIR-AF; 787-nm excitation; emission, >830 nm), short-wavelength autofluorescence (SW-AF; 488-nm excitation, 500- to 680-nm emission), and spectral-domain optical coherence tomography (SD-OCT) images were acquired with a confocal scanning laser ophthalmoscope. SW-AF intensities were measured by quantitative fundus autofluorescence (qAF), and NIR-AF intensity profiles were analyzed. Retinal thicknesses and visual acuity were measured. Results:In 19 of 31 eyes of affected males, islands of preserved NIR-AF signal were also visible as fluorescence signal in SW-AF images. Notable in 12 eyes were areas of speckled SW-AF that was hypoautofluorescent in the NIR-AF image. Islands of preserved NIR-AF and SW-AF signal were often associated with the presence of visible but thinned outer nuclear layer and discontinuous interdigitation zone, ellipsoid zone, and external limiting membrane. NIR-AF profiles revealed that even in areas of preserved retina, the NIR-AF signal from retinal pigment epithelium (RPE) melanin is greatly reduced. qAF was reduced overall. The fundus of carriers was characterized by a mosaicism in which patches of reduced NIR-AF colocalized with reduced SW-AF. Conclusions:In CHM-affected males, the presence of RPE was indicated by an NIR-AF signal and the absence of hypertransmission of OCT signal into the choroid. RPE preservation was associated with better visual acuity. In carriers, patches of reduced SW-AF colocalized with decreased NIR-AF and qAF was severely reduced.
journal_name
Invest Ophthalmol Vis Scijournal_title
Investigative ophthalmology & visual scienceauthors
Paavo M,Carvalho JRL Jr,Lee W,Sengillo JD,Tsang SH,Sparrow JRdoi
10.1167/iovs.19-27366subject
Has Abstractpub_date
2019-09-03 00:00:00pages
3752-3761issue
12eissn
0146-0404issn
1552-5783pii
2749691journal_volume
60pub_type
杂志文章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:One of the critical difficulties in design of a high-resolution retinal implant is the proximity of stimulating electrodes to the target cells. This is a report of a phenomenon of retinal cellular migration into a perforated membrane that may help to address this problem. METHODS:Mylar membranes with an array ...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.03-1327
更新日期:2004-09-01 00:00:00
abstract::Chinese hamster ovarian (CHO) cells in culture were used to evaluate the relative antiproliferative potential of drugs. These agents have been used to improve the clinical response after glaucoma filtering surgery. The following drugs were evaluated: 5-fluorouracil (5-FU) as the benchmark, 5-fluorouridine (FUR), 5-flu...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1990-12-01 00:00:00
abstract::The amount of unscheduled DNA synthesis (UDS) produced in the cells adjacent to excimer laser ablations in the cornea was compared for 193-nm and 248-nm laser wavelengths. UDS is interpreted to indicate the process of excision repair of pyrimidine dimers formed in DNA. 193-nm laser ablation did not produce a statistic...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1987-02-01 00:00:00
abstract:PURPOSE:To explore the effect of dominance and laterality on refractive error and axial length. METHODS:Ocular dominance was assessed with the hole-in-the-card test in 543 children during their 2006 follow-up visits for the Singapore Cohort study Of the Risk factors for Myopia (SCORM). Data were compared to cycloplegi...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.06-1489
更新日期:2007-08-01 00:00:00
abstract:Purpose:To clarify the distinct molecular pathogenesis of central serous chorioretinopathy (CSC) and pachychoroid neovasculopathy (PNV). Methods:Aqueous humor (AH) was collected from 18 acute CSC, 20 chronic CSC, and 20 PNV patients. Concentrations of 30 cytokines in the AH were analyzed using a multiplex bead immunoa...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.18-25517
更新日期:2018-12-03 00:00:00
abstract:PURPOSE:To relate the oxygen environment of the retina to photoreceptor stability, protection, and function in the P23H rat. METHODS:Heterozygote P23H-3 (Line 3) rats were studied. Photoreceptor death rates were assessed with the TUNEL technique for detection of fragmenting DNA, in a developmental series from postnata...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.03-0845
更新日期:2004-06-01 00:00:00
abstract:PURPOSE:To investigate the expression of humanin (HN) in human retinal pigment epithelial (hRPE) cells and its effect on oxidative stress-induced cell death, mitochondrial bioenergetics, and senescence. METHODS:Humanin localization in RPE cells and polarized RPE monolayers was assessed by confocal microscopy. Human RP...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.15-17053
更新日期:2016-03-01 00:00:00
abstract:PURPOSE:The aim of this study was to show the usefulness of three methods for measuring IOP: Goldmann applanation tonometry, rebound tonometry, and Ultra-High-Speed Scheimpflug technology. METHODS:The examined group consisted of 96 patients (192 eyes), including 63 women and 33 men with a mean age of 59.3 ± 19.9 years...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.13-13172
更新日期:2014-02-04 00:00:00
abstract:PURPOSE:Oxidative stress plays an important role in health and aging. We have shown that oxidative stress impairs mitochondrial function and promotes RPE cell death in an age-dependent manner. This study investigates the role of pigment epithelium-derived factor (PEDF) in limiting oxidative stress-induced damage to RPE...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.14-14696
更新日期:2014-09-11 00:00:00
abstract:PURPOSE:To investigate whether the detection of apoptosing retinal cells (DARC) could detect cells undergoing apoptosis in a laser model of retinal damage. METHODS:Laser lesions were placed, with the use of a frequency-doubled Nd:YAG laser, on the retina in 34 eyes of anesthetized Dark Agouti rats. Lesion size and las...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.07-1335
更新日期:2008-06-01 00:00:00
abstract::Prostaglandins and other eicosanoids in the trabecular meshwork may play important physiological and pharmacological roles in the aqueous outflow pathway. In the present studies, we employed [14C]-arachidonic acid to explore potentially important pathways for the production of eicosanoids in cultured human trabecular ...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1988-11-01 00:00:00
abstract::Smooth pursuit and other eye movements were quantitatively studied in patients with chronic schizophrenia to characterize the pattern and severity of eye movement abnormalities in this disorder. Twenty-one patients with schizophrenia, 13 patients with other psychiatric disorders (manic-depressive psychosis, schizoaffe...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1987-02-01 00:00:00
abstract:Purpose:Conjunctival squamous cell carcinoma (SCC), a type of ocular surface neoplasia, is primarily treated by surgical resection and topical immuno- or chemotherapy. Metastatic disease may be treated with systemic chemo- or immunotherapy, albeit with variable response. The purpose of this study was to determine wheth...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.19-26894
更新日期:2019-05-01 00:00:00
abstract:PURPOSE:In several retinal complications, such as age-dependent macular degeneration (AMD), oxidative stress is increased and cytokine level is elevated. These are shown to alter the activation and expression of matrix metalloproteinase (MMP) both in human primary and immortalized retinal pigment epithelial (RPE) cells...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.15-17333
更新日期:2015-10-01 00:00:00
abstract:PURPOSE:To determine the sensitivity to change and specificity achieved when published test-retest variability (TRV) data are used to determine whether measured changes in ETDRS logarithm of the minimum angle of resolution (logMAR) visual acuity reflect true clinical change or are attributable to measurement error alon...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.02-1100
更新日期:2003-08-01 00:00:00
abstract::Ocular malformations and visual deficits are pathognomic of fetal alcohol syndrome (FAS). However, there are no reports on retinal visual function. To determine if prenatal (human second-trimester equivalent) or prenatal plus 10 days of postnatal (human third-trimester equivalent) ethanol exposure produced scotopic an...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1991-10-01 00:00:00
abstract:Purpose:To quantify and evaluate macular superficial capillaries and large vessels separately using an optical coherence tomographic angiography (OCTA)-based automatic segmentation algorithm. Methods:In this cross-sectional study, all eyes were scanned using an OCTA device with 3 × 3 mm cube centered on the fovea. Ret...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.17-23676
更新日期:2018-04-01 00:00:00
abstract:PURPOSE:Local first-order multifocal electroretinogram (mfERG) implicit time (K1-IT) delays have proved to be important and predictive indicators of retinal function in diabetes. To better understand the nature of these delays, the authors examined the spatial association between K1-IT and second-order amplitudes (K2-S...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.07-0180
更新日期:2007-11-01 00:00:00
abstract:PURPOSE:We studied morphologic changes of the retinal vasculature in eyes with central retinal vein occlusion (CRVO) through the use of optical coherence tomography (OCT). METHODS:Major retinal vessels in 35 eyes from 35 consecutive patients with acute CRVO were examined prospectively and longitudinally with sequentia...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.13-13201
更新日期:2014-01-07 00:00:00
abstract:PURPOSE:Trefoil factor family (TFF) peptides, and in particular TFF3, are characteristic secretory products of mucous epithelia that promote antiapoptosis, epithelial migration, restitution, and wound healing. For a long time, a receptor for TFF3 had not yet been identified. However, the chemokine receptor CXCR4 has be...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.15-18129
更新日期:2016-01-01 00:00:00
abstract::Overall corneal hydration control expressed as the percent recovery per hour (PRPH) can be assessed with an exponential model that uses data derived from two kinds of corneal thickness measurements; one from monitoring recovery after inducing corneal swelling, and the other from measurements made after the eye has bee...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1990-07-01 00:00:00
abstract:PURPOSE:To determine the roles of glial fibrillary acidic protein (GFAP) and vimentin in Müller cell reactivity. METHODS:Retinal detachments were created in mice deficient for GFAP and vimentin (GFAP(-/-)vim(-/-)) and age-matched wild-type (wt) mice. The reactivity of the retina was studied by immunofluorescence and e...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.07-1474
更新日期:2008-08-01 00:00:00
abstract:PURPOSE:Methylenetetrahydrofolate reductase (Mthfr) is a key enzyme in homocysteine-methionine metabolism. We investigated Mthfr expression in retina and asked whether mild hyperhomocysteinemia, due to Mthfr deficiency, alters retinal neurovascular structure and function. METHODS:Expression of Mthfr was investigated a...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.14-16190
更新日期:2015-04-01 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 authors investigated how individual differences in macular pigment (MP) density are related to loss of visual sensitivity with age. METHODS:Macular pigment and visual sensitivity of 27 healthy older subjects (aged 60-84 years) were compared with data from 10 younger subjects (aged 24-36 years). Macular pig...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1998-02-01 00:00:00
abstract:PURPOSE:Migration of Müller glial cells is enhanced in proliferative retinopathies, but the mechanisms involved are ill defined. Sphingosine-1-phosphate (S1P) is a bioactive sphingolipid synthesized by sphingosine kinase (SphK), which promotes proliferation, migration, and inflammation, acting as an intracellular media...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.14-16195
更新日期:2015-09-01 00:00:00
abstract:PURPOSE:To characterize the potential for neuropeptide Y (NPY) signaling in the retinal pigment epithelium (RPE) by identifying the NPY receptor subtypes present, determining the effect of NPY on second-messenger production and membrane conductance, and establishing the neural retina as a site of NPY gene expression. ...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1998-09-01 00:00:00
abstract:PURPOSE:Dysregulation of the extracellular matrix (ECM) plays an important role in the pathogenesis of age-related macular degeneration (AMD). Elastin is a fibrous protein constituent of the ECM, degradation of which may be detected by the presence of serum elastin-derived peptides (S-EDPs) in circulation. This study w...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.04-1277
更新日期:2005-09-01 00:00:00
abstract:PURPOSE:To characterize the response of cultured human retinal pigment epithelial (RPE) cells to lowered environmental oxygen. METHODS:The response of cultured RPE cells to lowered oxygen environments was compared to that of cell types of presumed high (Madin-Darby canine kidney [MDCK] cells, an epithelial cell line) ...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1994-05-01 00:00:00