Abstract：
PURPOSE:Leber congenital amaurosis (LCA) is a group of childhood-onset retinal diseases characterized by severe visual impairment or blindness. One form is caused by mutations in the RPE65 gene, which encodes the retinal pigment epithelium (RPE) isomerase. In this study, the retinal structure and expression of molecular markers for different retinal cell types were characterized, and differences between control and RPE65 mutant dogs during the temporal evolution of the disease were analyzed. METHODS:Retinas from normal and mutant dogs of different ages were examined by immunofluorescence with a panel of 16 different antibodies. RESULTS:Cones and rods were preserved in the mutant retinas, and the number of cones was normal. However, there was altered expression of cone arrestin and delocalization of rod opsin. The ON bipolar cells showed sprouting of the dendritic arbors toward the outer nuclear layer (ONL) and retraction of their axons in the inner nuclear layer (INL). A decreased expression of GABA, and an increased expression of intermediate filament glial markers was also found in the mutant retinas. These changes were more evident in the adult than the young mutant retinas. CONCLUSIONS:The structure of the retina is well preserved in the mutant retina, but several molecular changes take place in photoreceptors and in bipolar and amacrine cells. Some of these changes are structural, whereas others reflect a change in localization of the examined proteins. This study provides new information that can be applied to the interpretation of outcomes of retinal gene therapy in animal models and humans.
journal_name
Invest Ophthalmol Vis Scijournal_title
Investigative ophthalmology & visual scienceauthors
Hernández M,Pearce-Kelling SE,Rodriguez FD,Aguirre GD,Vecino Edoi
10.1167/iovs.10-5213subject
Has Abstractpub_date
2010-12-01 00:00:00pages
6793-802issue
12eissn
0146-0404issn
1552-5783pii
iovs.10-5213journal_volume
51pub_type
杂志文章abstract：Purpose:The three-dimensional configurations of rod and cone bipolar cell (BC) dendrites and horizontal cell (HC) processes outside rod and cone synaptic terminals have not been fully elucidated. We reveal how these neurites are mutually arranged to coordinate formation and maintenance of the postsynaptic complex of ri...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.62.1.31
更新日期：2021-01-04 00:00:00
abstract：PURPOSE:We evaluated the clinical use of segmented macular layer thickness measurement in terms of glaucoma diagnosis and the ability to detect progression, and to compare such outcomes to those by circumpapillary retinal nerve fiber layer (cRNFLT) and total macular thickness (TMT) measurements. METHODS:The study incl...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.11-9369
更新日期：2012-06-20 00:00:00
abstract：:Gross correlation coefficient between light-scattering intensity and the optical parameters obtained for 48 sections of eight lenses with nuclear cataracts were evaluated. On the basis of these and other data in the literature, the structural elements, within the lens fiber are identified. These give rise to the optic...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1981-03-01 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:Histone lysine methylation (HKM) is an important epigenetic mechanism that establishes cell-specific gene expression and functions in development. However, epigenetic control of retinal development is poorly understood. To study the roles of HKM in retinogenesis, the authors examined the dynamic changes of thre...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.09-4730
更新日期：2010-12-01 00:00:00
abstract：PURPOSE:To determine whether glaucoma alters intrinsically photosensitive retinal ganglion cell (ipRGC) function. METHODS:Forty-one patients (25 with glaucoma and 16 healthy age-matched control participants) were tested. Intrinsically photosensitive retinal ganglion cell function was directly measured by the sustained...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.10-7069
更新日期：2011-06-21 00:00:00
abstract：:Vitrectomy has been shown to halt diabetic retinal neovascularization, but the mechanism of this process is unknown. We propose that vitrectomy improves the oxygen supply to ischemic inner retina by way of fluid currents in the vitreous cavity. In order to test this hypothesis, we induced branch retinal vein occlusion...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1990-02-01 00:00:00
abstract：PURPOSE:To prepare binary transgenic mouse lines that overexpress reporter genes in a corneal-epithelium-specific manner when induced by doxycycline. METHODS:A gene-targeting construct containing an internal ribosomal entry site-reverse tetracycline transcription activator (IRES-rtTA) cassette was inserted into the Kr...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.04-1464
更新日期：2005-06-01 00:00:00
abstract：PURPOSE:The purpose of this study was to assess qualitatively the expression of adhesion molecules by human retinal pigment epithelium (RPE) and to study their regulation by inflammatory cytokines. These molecular events and the role played by inflammatory cytokines are important for selective lymphocyte trafficking in...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1995-10-01 00:00:00
abstract：Purpose:We investigated the cross-sectional associations between macular pigment optical density (MPOD), plasma lutein (L), and zeaxanthin (Z) concentrations and cognitive function in 184 older adults of the 3-City-Bordeaux cohort. Methods:MPOD was measured using the two-wavelength autofluorescence method with a modif...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.17-22656
更新日期：2018-04-01 00:00:00
abstract：PURPOSE:To elucidate the anatomy of the trabecular meshwork (TM) and its connection to ciliary muscle (CM) tendons with two photon excitation microscopic (TPEM) imaging. METHODS:The human aqueous outflow pathway was imaged in an unfixed and nonembeded state by using an inverted TPEM. Laser (Ti:Sapphire) was tuned at 8...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.15-17091
更新日期：2016-03-01 00:00:00
abstract：:Evoked potentials (EP's) in response to phase-alternating gratings were recorded from normal and stereoblind or stereodefective subjects (adults and children) under monocular and binocular viewing conditions. The amplitude of the binocular EP was found to exceed the larger monocular EP in normal but not in stereodefec...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1978-09-01 00:00:00
abstract：PURPOSE:To investigate the characteristics of retinal nerve fiber layer (RNFL) defects associated with type II diabetes. METHODS:Forty nonglaucomatous eyes with type II diabetes and 54 eyes with early open angle glaucoma that exhibited a localized RNFL defect and 42 eyes from age- and sex-matched nondiabetic, nonglauc...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.16-19525
更新日期：2016-08-01 00:00:00
abstract：PURPOSE:Glaucoma is the second most frequent cause of visual impairment worldwide. Elevated intraocular pressure (IOP) causes glaucomatous optic nerve damage, especially in the primary open-angle glaucoma (POAG) subtype. As most previous studies on IOP genetics were analyses of glaucomatous families, a study of general...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.09-3979
更新日期：2010-03-01 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：PURPOSE:The influences of diurnal rhythms involving a variety of ocular parameters are implicated in the development of myopia. The purpose of this study was to determine the expression of the melatonin receptor subtype proteins in chick ocular tissues and to examine the role of the circadian signaling molecule melaton...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.05-0195
更新日期：2006-01-01 00:00:00
abstract：PURPOSE:Rabbits are used commonly for the evaluation of drugs and surgery to lower intraocular pressure (IOP). The authors compared the accuracy and variability of three tonometers for measuring IOP in rabbits. METHODS:The anterior chamber of adult rabbits was cannulated with a 25-gauge needle connected to an elevated...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1996-04-01 00:00:00
abstract：:The existence of wide-band, phase-specific channels in the human visual system has been suggested in recent investigations. We used a number of adapting gratings to test for phase-specific adaptation effects. Observers were asked to discriminate between a simple 3 cpd sine-wave grating, (3), and a complex grating comp...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1978-05-01 00:00:00
abstract：PURPOSE:To develop a new test of activities of daily living (ADLs) appropriate for the low-vision population: the Melbourne Low-Vision ADL Index (MLVAI). METHODS:The MLVAI was designed as a desk-based clinical assessment, comprising 18 observed items on complex ADLs in part (a) and 9 questions on broad self-care ADLs ...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：2001-05-01 00:00:00
abstract：:Paired human donor corneas (age, 73 +/- 12 yr), preserved in organ culture medium, were used to evaluate the effect of human epidermal growth factor (hEGF) on endothelial wound closure rate (WCR), on morphometric parameters (cell size, shape, and density), and on cell division in the wound area. The endothelium of the...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1992-05-01 00:00:00
abstract：PURPOSE:To assess alterations in allergic ocular responses to nonparasite antigens in an experimental system in which mice were skewed toward a Th2 cytokine profile by helminth infection. METHODS:Mice were inoculated with Ascaris suum (A. suum) eggs concurrent with ragweed (RW) sensitization (RW/acute) or by repeated ...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.04-0899
更新日期：2005-08-01 00:00:00
abstract：PURPOSE:Sarcoidosis is a heterogeneous and multisystem granulomatous disorder. The etiology still is uncertain, but the disease currently is thought to be triggered by various genetic as well as environmental factors. Recently, an association between sarcoidosis and the butyrophilin-like 2 (BTNL2) gene located in close...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.12-10491
更新日期：2012-10-11 00:00:00
abstract：Purpose:To examine the association between intraocular pressure (IOP) and the prevalence of glaucomatous optic neuropathy (GON) in high myopia. Methods:The hospital-based observational study consisted of patients treated in the Tokyo High Myopia Clinics and for whom fundus photographs and IOP readings were available. ...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.17-21942
更新日期：2017-11-01 00:00:00
abstract：PURPOSE:To assess the impact of zinc finger E-box binding homeobox 1 (ZEB1) gene mutations associated with posterior polymorphous corneal dystrophy 3 (PPCD3) and Fuchs' endothelial corneal dystrophy (FECD). METHODS:Thirteen of the 27 previously reported ZEB1 truncating mutations associated with PPCD3 and the six previ...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.14-15247
更新日期：2014-09-04 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
abstract：:Hypothesis-driven science is expected to result in a continuum of studies and findings along a discrete path. By comparison, serendipity can lead to new directions that branch into different paths. Herein, I describe a diverse series of findings that were motivated by hypotheses, but driven by serendipity. I summarize...
journal_title：Investigative ophthalmology & visual science
pub_type：
doi：10.1167/iovs.11-8330
更新日期：2011-09-29 00:00:00
abstract：PURPOSE:To examine the expression and localization of the neuroplastins (np), two synapse-enriched members of the immunoglobulin (Ig) superfamily of cell-adhesion molecules, in the developing and adult retina and optic nerve. METHODS:Expressions of the two isoforms np55 and np65 and carboxyl-terminal splice variants w...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：2001-07-01 00:00:00
abstract：:The hypotensive effect of ascorbate on intraocular pressure has been reported following topical application, oral administration, or anterior chamber infusion in animals. The present report describes the correlation of aqueous humor ascorbate concentration with intraocular pressure as well as outflow facility in vivo....
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1978-08-01 00:00:00
abstract：PURPOSE:The authors conducted a morphology analysis of filtering blebs with anterior segment optical coherence tomography (AS-OCT) in patients shortly after trabeculectomy to find further criteria for success or failure of a filtering bleb. METHODS:Thirty-seven patients (43 eyes) who had recently (<2 years) undergone ...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.10-5520
更新日期：2011-04-14 00:00:00
abstract：PURPOSE:The purpose of this study was to evaluate the Tono-Pen 2 tonometer for measuring intraocular pressure (IOP) in the living rat eye. METHODS:One eye from each of 20 adult, anesthetized brown Norway rats (group 1) was cannulated and simultaneously connected to a syringe and a pressure transducer with a chart reco...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1993-02-01 00:00:00
