Abstract：
PURPOSE:To identify genes and molecular mechanisms associated with photoreceptor degeneration in a canine model of XLRP caused by an RPGR exon ORF15 microdeletion. Methods. Expression profiles of mutant and normal retinas were compared by using canine retinal custom cDNA microarrays. qRT-PCR, Western blot analysis, and immunohistochemistry (IHC) were applied to selected genes, to confirm and expand the microarray results. RESULTS:At 7 and 16 weeks, respectively, 56 and 18 transcripts were downregulated in the mutant retinas, but none were differentially expressed (DE) at both ages, suggesting the involvement of temporally distinct pathways. Downregulated genes included the known retina-relevant genes PAX6, CHML, and RDH11 at 7 weeks and CRX and SAG at 16 weeks. Genes directly or indirectly active in apoptotic processes were altered at 7 weeks (CAMK2G, NTRK2, PRKCB, RALA, RBBP6, RNF41, SMYD3, SPP1, and TUBB2C) and 16 weeks (SLC25A5 and NKAP). Furthermore, the DE genes at 7 weeks (ELOVL6, GLOD4, NDUFS4, and REEP1) and 16 weeks (SLC25A5 and TARS2) are related to mitochondrial functions. qRT-PCR of 18 genes confirmed the microarray results and showed DE of additional genes not on the array. Only GFAP was DE at 3 weeks of age. Western blot and IHC analyses also confirmed the high reliability of the transcriptomic data. CONCLUSIONS:Several DE genes were identified in mutant retinas. At 7 weeks, a combination of nonclassic anti- and proapoptosis genes appear to be involved in photoreceptor degeneration, whereas at both 7 and 16 weeks, the expression of mitochondria-related genes indicates that they may play a relevant role in the disease process.
journal_name
Invest Ophthalmol Vis Scijournal_title
Investigative ophthalmology & visual scienceauthors
Genini S,Zangerl B,Slavik J,Acland GM,Beltran WA,Aguirre GDdoi
10.1167/iovs.10-5443subject
Has Abstractpub_date
2010-11-01 00:00:00pages
6038-50issue
11eissn
0146-0404issn
1552-5783pii
iovs.10-5443journal_volume
51pub_type
杂志文章abstract：PURPOSE:To evaluate the effects of unilateral, stereotactic, posteroventral pallidotomy on saccadic eye movements in patients with Parkinson disease (PD). METHODS:Eye movements of 31 patients with moderate to advanced PD were recorded with an infrared system 1 month before and 3 months after pallidotomy. Two kinds of ...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：2000-07-01 00:00:00
abstract：PURPOSE:To assess the prevalence, distribution, and demographic associations of refractive error in an urban population in southern India. METHODS:Two thousand five hundred twenty-two subjects of all ages, representative of the Hyderabad population, were examined in the population-based Andhra Pradesh Eye Disease Stud...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1999-11-01 00:00:00
abstract：PURPOSE:The anterior surface of the eye is covered by several physically contiguous but histologically distinguishable epithelial overlying the cornea, limbus, bulbar conjunctiva, fornix conjunctiva, and palpebral conjunctiva. It is important to determine whether the different phenotypes of these epithelia are the resu...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1993-04-01 00:00:00
abstract：PURPOSE:To identify the underlying mechanisms by which lipid mediator lysophosphatidic acid (LPA) acts as a growth factor in stimulating extracellular signal-regulated kinase (ERK) and phosphatidylinositol 3'-kinase (PI3K) during corneal epithelial wound healing. METHODS:Epithelial debridement wounds in cultured porci...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.06-0203
更新日期：2007-02-01 00:00:00
abstract：:Chloroquine was administered intramuscularly 5 days a week to rhesus monkeys for as long as 4 1/2 years. No clinical, fluorescein angiographic, or electrophysiological evidence of retinal damage was observed. Yet chloroquine/chloroquine byproduct analysis of the ocular tissues revealed an enormous binding capacity of ...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1978-12-01 00:00:00
abstract：PURPOSE:Visual sensations in patients with blindness and retinal degenerations may be restored by electrical stimulation of retinal neurons with implantable microelectrode arrays. A prospective trial was initiated to evaluate the safety and efficacy of a wireless intraocular retinal implant (EPIRET3) in six volunteers ...
journal_title：Investigative ophthalmology & visual science
pub_type： 临床试验,杂志文章
doi：10.1167/iovs.08-2752
更新日期：2009-06-01 00:00:00
abstract：Purpose:Treatment success in clinical trials for AMD would ideally be aligned to measurable performance in visual tasks rather than imperceptible changes on clinical charts. We test the hypothesis that patients with dry AMD perform worse than visually healthy peers on computer-based surrogates of "real-world" visual se...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.16-21122
更新日期：2017-03-01 00:00:00
abstract：PURPOSE:Visibility of low-spatial frequency stimuli improves when their contrast is modulated at 5 to 10 Hz compared with stationary stimuli. Therefore, temporal modulations of visual objects could enhance the performance of low vision patients who primarily perceive images of low-spatial frequency content. We investig...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.11-9157
更新日期：2012-09-06 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：PURPOSE:In young Swiss (HSD:ICR) outbred mice, corneal clarity is restored after Pseudomonas aeruginosa ocular infection, whereas disease in aged outbred mice progresses to corneal perforation. This study was conducted to elucidate further the mechanism responsible for this age-related disparity in disease response. M...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1995-05-01 00:00:00
abstract：PURPOSE:To study the damaging effect of UV-A irradiation on the chaperone-like properties of alpha-crystallin and the subsequent recovery process of young and old bovine lenses. METHODS:Young and old bovine lenses were kept in organ culture. After 24 hours of incubation they were irradiated with UV-A at 365 nm, and op...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：2000-01-01 00:00:00
abstract：PURPOSE:To visualize and quantify the three-dimensional (3D) spatial relationships of the structures of the aqueous outflow system (AOS) within intact enucleated mouse eyes using spectral two-photon microscopy (TPM) techniques. METHODS:Spectral TPM, including two-photon autofluorescence (TPAF) and second-harmonic gene...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.15-18491
更新日期：2016-06-01 00:00:00
abstract：PURPOSE:To study the effects of nipradilol, a nonselective beta-blocker with alpha 1-blocking activity and nitroglycerin-like activity, on aqueous humor dynamics and optic nerve head (ONH) circulation in albino rabbits. METHODS:Experiments were carried out during the dark phase, in conscious rabbits conditioned to a s...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1998-04-01 00:00:00
abstract：PURPOSE:To investigate whether visual deficits in children with infantile spasm (IS) are the result of seizure activity or of treatment with the anticonvulsant drug vigabatrin (VGB). METHODS:Vision function was determined in three experiments by determining peak contrast sensitivity (CS) and grating acuity (GA) with t...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.06-0755
更新日期：2007-08-01 00:00:00
abstract：PURPOSE:To characterize in SV40-immortalized human corneal epithelial cells (tHCEC) osmosensitive taurine transporter gene and protein expression as well as its functional activity. To evaluate whether medium supplementation with taurine improves cell viability during a hypertonic challenge. METHODS:tHCEC were preincu...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：2002-09-01 00:00:00
abstract：PURPOSE:To study retinal structure in choroideremia patients and carriers using high-resolution imaging techniques. METHODS:Subjects from four families (six female carriers and five affected males) with choroideremia (CHM) were characterized with best-corrected visual acuity (BCVA), kinetic and static perimetry, full-...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.12-10707
更新日期：2013-02-01 00:00:00
abstract：:To assess whether prostaglandins contribute to the control of basal retinal and choroidal hemodynamics, retinal (RBF) and choroidal blood flow (ChBF) were measured by a microsphere technique in 1- to 4-day-old pigs before and after (at 20 and 60 min) administration of indomethacin (0.3 mg/kg, n = 6 or 10 mg/kg, n = 5)...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1992-11-01 00:00:00
abstract：PURPOSE:To examine the visual evoked potentials (VEP) and electroretinograms (ERG) generated during electrical stimulation of the human optic nerve using the optic nerve visual prosthesis. METHODS:Two volunteers blind from retinitis pigmentosa (RP) and with no light perception each received a chronically implanted opt...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.09-4346
更新日期：2010-10-01 00:00:00
abstract：Purpose:Candida remains the leading cause of fungal endophthalmitis. However, the pathobiology and innate immune responses in this disease are not well characterized. Here, we developed two murine models of candida endophthalmitis and evaluated their disease susceptibility and differential immune response. Methods:End...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.61.11.31
更新日期：2020-09-01 00:00:00
abstract：:Stiles-Crawford functions were obtained from the maculas of 22 patients with different genetic types of retinitis pigmentosa and visual acuity of 20/40 or better. Reduced cone directional sensitivity was seen in the fovea with both focal cone electroretinographic testing and psychophysical testing. Functions from the ...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1982-02-01 00:00:00
abstract：PURPOSE:High concentrations of unconjugated bilirubin are neurotoxic and cause brain damage in newborn infants. However, the exact level of bilirubin that may be neurotoxic in a given infant is unknown. The aim of this study was to use a quantitative measure of neural activity, the swept parameter visual evoked potenti...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.14-14261
更新日期：2014-09-02 00:00:00
abstract：:Electroretinographic studies of myctalopic Appaloosa horses demonstrated photopic and scotopic abnormalities similar to those in humans with congenital stationary night blindness (CSNB) of the Schubert-Bornschein type. The phototopic abnormalities consisted of reduced b-wave amplitudes and slower than normal b-wave im...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1978-08-01 00:00:00
abstract：:The visual effects of carbon disulfide exposure were studied in macaque monkeys with measurements of visual thresholds, fluorescein angiography and fundus photography. Five monkeys were exposed by inhalation for 6 hr a day, 5 days a week to 256 ppm carbon disulfide (CS2). The motor dysfunction observed in these monkey...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1988-04-01 00:00:00
abstract：PURPOSE:Rabbits are commonly used for the evaluation of drugs and surgery to lower intraocular pressure (IOP). The accuracy of intraocular pressure measurement is therefore critical in the analysis of data and subsequent extrapolation to humans. The purpose of this study was to establish the most reliable technique, fr...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.04-0610
更新日期：2005-07-01 00:00:00
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journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.07-1050
更新日期：2008-03-01 00:00:00
abstract：PURPOSE:To evaluate the longitudinal variability of optic disc and retinal nerve fiber layer (RNFL) measurements obtained from optical coherence tomography (OCT), scanning laser polarimetry (SLP), and confocal scanning laser ophthalmoscopy (CSLO). METHODS:Forty-five normal and 43 glaucomatous eyes of 88 subjects were ...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.07-1187
更新日期：2008-11-01 00:00:00
abstract：PURPOSE:Vitreous degeneration contributes to several age-related eye diseases, including retinal detachment, macular hole, macular traction syndrome, and nuclear cataracts. Remarkably little is understood about the molecular interactions responsible for maintaining vitreous structure. The purpose of this study was to m...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：10.1167/iovs.13-13026
更新日期：2014-01-03 00:00:00
abstract：:We evaluated the dose relationship between the antiproliferative and toxic effects of 5-fluorouracil (5-FU) on the ocular surface epithelium following experimental corneal epithelial wounding in rabbits. Central corneal epithelial defects 8 mm in diameter were made using n-heptanol. 5-FU (0.05 mg, 0.5 mg, or 5.0 mg pe...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1987-10-01 00:00:00
abstract：:Biometry performed in patients with unilateral dense congenital cataracts and unilateral complete blepharoptosis did not show a consistent elongation of the involved eye. The antero-posterior axis of the visually deprived eye was longer than that in the normal fellow eye in some but shorter or unchanged in other patie...
journal_title：Investigative ophthalmology & visual science
pub_type： 杂志文章
doi：
更新日期：1987-04-01 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
