Abstract:
PURPOSE:To determine the morphologic changes in the retina in the macula and around the optic disc in patients with autosomal dominant optic atrophy (ADOA) associated with a mutation in the OPA1 gene. METHODS:Cross-sectional images of the macular area of the retina were obtained by optical coherence tomography (OCT) in patients with ADOA who had a heterozygous mutation in the OPA1 gene. There were 15 eyes of eight patients from five families: four men and four women. The average age of the patients was 48.1 years. In the OCT images, the cross sections of the sensory retina were divided manually into four areas. The thickness of the overall sensory retina and the divided areas were measured at 1 and 2 mm on the temporal, nasal, superior, and inferior sides of the fovea as well as at the fovea. The thickness of the retinal nerve fiber layer (RNFL) around the optic discs was measured by taking circular scans (3.4 mm in diameter) centered on the optic disc. The results in the patients with ADOA were compared with those from 11 normal control subjects. RESULTS:The overall thickness of the sensory retina in the macular area was significantly thinner in the patients with ADOA than in the control subjects at all points except the fovea (P < 0.0001). The RNFL in the macular area in the patients with ADOA was significantly thinner than that in control subjects at all points (P < 0.0001), especially at 1 mm from the fovea. The circumpapillary RNFL was significantly thinner at the temporal, superior, and inferior areas in patients with ADOA but not in the nasal area. The total cross-sectional area of the circumpapillary RNFL was significantly correlated with visual acuity. The thickness of the combined ganglion cell layer, inner plexiform layer, inner nuclear layer, and outer plexiform layer in the macular area was significantly thinner in the patients (P < 0.0056). The thickness of the outer nuclear layer and the photoreceptor inner segments and the thickness of the photoreceptor outer segments were not significantly different between the patients with ADOA and normal control subjects. CONCLUSIONS:The RNFL and the layer including the ganglion cell layer are significantly thinner in patients with ADOA associated with an OPA1 gene mutation, whereas the photoreceptor layers are not affected morphologically. The inner retina is the main area of the retina altered in ADOA.
journal_name
Invest Ophthalmol Vis Scijournal_title
Investigative ophthalmology & visual scienceauthors
Ito Y,Nakamura M,Yamakoshi T,Lin J,Yatsuya H,Terasaki Hdoi
10.1167/iovs.07-0024subject
Has Abstractpub_date
2007-09-01 00:00:00pages
4079-86issue
9eissn
0146-0404issn
1552-5783pii
48/9/4079journal_volume
48pub_type
杂志文章abstract:PURPOSE:To understand the genetic regulatory pathways underlying the retinoic acid (RA) induction of cone arrestin, gene array technology and other molecular tools were used to profile global gene expression changes in human retinoblastoma cells. METHODS:Weri-Rb-1 retinoblastoma cells were cultured in the absence or p...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.02-0434
更新日期:2003-03-01 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:PURPOSE:Peroxynitrite generated during the early phase of experimental autoimmune uveoretinitis (EAU) causes peroxidation of docosahexaenoic acid (22:6), a principal unsaturated fatty acid of the photoreceptor membrane, to its hydroperoxide (22:6HP). During this phase, microglia migrate to the site of photoreceptors. T...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.06-0221
更新日期:2006-08-01 00:00:00
abstract:PURPOSE:To evaluate the structure-function relationship between ganglion cell-inner plexiform layer (GCIPL) thickness at the macula and 10-2 standard automated perimetry (SAP) in glaucoma and to evaluate the relationship using a recently proposed linear model. METHODS:In a cross-sectional analysis, structure-function ...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.15-16943
更新日期:2015-06-01 00:00:00
abstract:PURPOSE:To make the first quantitative assessment of the rate of oxygen consumption in high oxygen-consuming layers of both the outer and inner retina of the rat in vivo. METHODS:Oxygen-sensitive microelectrodes were used to measure the oxygen tension as a function of depth through the retina in anesthetized rats. Ind...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:2002-06-01 00:00:00
abstract:PURPOSE:Mutations in the frizzled-4 gene (FZD4) have recently been associated with autosomal dominant familial exudative vitreoretinopathy (FEVR) in families linking to the EVR1 locus on the long arm of chromosome 11. The purpose of this study was to screen FZD4 in a panel of 40 patients with FEVR to identify the types...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.03-1044
更新日期:2004-07-01 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
abstract::Using aqueous and vitreous fluorophotometry, the authors examined the blood-aqueous and blood-retinal barrier functions in three groups of pigmented rabbits. Epinephrine (1.25%) was applied topically five times daily and indomethacin (0.5% sesame oil suspension) was applied topically three times daily to one eye of ea...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1987-03-01 00:00:00
abstract:PURPOSE:Currently, there are no definitive immunomarkers for epithelial stem cells (corneal and conjunctival) or their poorly understood niche microenvironment. The H2B-GFP/K5tTA mouse enables visualization of label-retaining cells (LRCs), which exhibit the functional marker of stem cell quiescence. We used immunofluor...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.15-18038
更新日期:2015-11-01 00:00:00
abstract:PURPOSE:To assess the reproducibility of the ocular response analyzer (ORA) in nonoperated eyes and the impact of corneal biomechanical properties on intraocular pressure (IOP) measurements in normal and glaucomatous eyes. METHODS:In the reliability study, two independent examiners obtained repeated ORA measurements i...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.07-0280
更新日期:2008-03-01 00:00:00
abstract::A single injection of an aqueous suspension of group A streptococcal peptidoglycan-polysaccharide complexes (PG-PS) when injected intraperitoneally into Lewis rats induced a self-limiting bilateral uveitis with associated perpetuating polyarthritis. The uveitis was characterized clinically during the first 72 h by iri...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1986-06-01 00:00:00
abstract:Purpose:Patients express their discomfort by subjective complaints, which may not clearly express the extent of their discomfort. This study noninvasively and objectively quantified ocular discomfort, a form of feeling, from the prefrontal cortex by functional near-infrared ray spectroscopy topography. Methods:This ca...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.18-24840
更新日期:2018-09-04 00:00:00
abstract:PURPOSE:Recently, RAP1 (Telomeric Repeat Binding Factor 2, Interacting Protein [TERF2IP]) was discovered as a modulator that selectively regulates nuclear factor light chain kappa enhancer of activated B cells (NFκB) signaling. The roles of RAP1 in regulation of inflammation and angiogenesis for corneal recovery follow...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.14-15268
更新日期:2015-01-08 00:00:00
abstract::Three monoclonal antibodies against lens crystallin have been used to study the accumulation of specific polypeptides during development of the human lens. One antibody which recognizes an antigen common to three polypeptides with molecular weights close to 31,000 reacted equally well to the human lens cortex and nucl...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1985-07-01 00:00:00
abstract:PURPOSE:To assess the range of macular pigment optical density (MPOD) in a healthy group of young adults of South Asian origin; to investigate whether any dietary factors or personal characteristics were related to intersubject variations in MPOD; and to compare the mean MPOD of the South Asian group with the mean MPOD...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.12-10957
更新日期:2013-04-17 00:00:00
abstract::The level of oxygen at the anterior corneal surface beneath the closed eyelid is shown to be equivalent to an atmosphere of 7.7% oxygen. This finding is in good agreement with assumptions which have been based on the oxygen level at the palpebral conjunctiva. However, in some instances a significant amount of oxygen i...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1979-01-01 00:00:00
abstract:PURPOSE:Mutations in PITX2 are associated with Axenfeld-Rieger syndrome (ARS), which involves ocular, dental, and umbilical abnormalities. Identification of cis-regulatory elements of PITX2 is important to better understand the mechanisms of disease. METHODS:Conserved noncoding elements surrounding PITX2/pitx2 were id...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.10-6060
更新日期:2011-03-18 00:00:00
abstract:PURPOSE:This study sought to determine the earliest time-point at which evidence of choroidal neovascularization (CNV) could be detected with visible-light optical coherence tomography angiography (vis-OCTA) in a mouse model of laser-induced CNV. METHODS:Visible light-OCTA was used to study laser-induced CNV at differ...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.15-18891
更新日期:2016-07-01 00:00:00
abstract:PURPOSE:Because several polypeptide growth factors are known to influence capillary endothelial cell mitogenesis, the authors investigated the presence of some of these molecules in choroidal neovascular membranes (CNVMs) removed surgically from human subjects with age-related macular degeneration (ARMD). METHODS:The ...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1994-07-01 00:00:00
abstract:PURPOSE:It is well documented that grafting of cells in the subretinal space of Royal College of Surgeons (RCS) rats limits deterioration of vision and loss of photoreceptors if performed early in postnatal life. What is unclear is whether cells introduced later, when photoreceptor degeneration is already advanced, can...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.07-0992
更新日期:2008-01-01 00:00:00
abstract:Purpose:To evaluate the association between endothelial cell density (ECD) after Descemet's stripping automated endothelial keratoplasty (DSAEK) and preoperative cytokine levels in the aqueous humor (AqH). Methods:This prospective consecutive case series included 97 consecutive patients who underwent DSAEK (64 eyes) o...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.17-23049
更新日期:2018-02-01 00:00:00
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:We compare the choroidal vascular area between Bietti crystalline dystrophy (BCD) patients with CYP4V2 mutations, retinitis pigmentosa (RP) patients with EYS mutations, and normal controls, and investigate the correlation between choroidal vascular area and associated parameters. Methods:This prospective case-...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.17-21515
更新日期:2017-08-01 00:00:00
abstract:PURPOSE:Late infantile neuronal ceroid lipofuscinosis (NCL) is an inherited disorder characterized by progressive vision loss. The disease results from mutations in the TPP1 (CLN2) gene. Studies were undertaken to characterize the effects of a TPP1 frameshift mutation on the retina in Dachshunds. METHODS:A litter of f...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.08-1712
更新日期:2008-06-01 00:00:00
abstract:PURPOSE:Trabecular meshwork (TM) cells appear to sense changes in intraocular pressure (IOP) as mechanical stretching. In response, they make homeostatic corrections in the aqueous humor outflow resistance, partially by increasing extracellular matrix (ECM) turnover initiated by the matrix metalloproteinases. To unders...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.05-0075
更新日期:2005-08-01 00:00:00
abstract:PURPOSE:Floppy eyelid syndrome (FES) is an acquired hyperelasticity disorder affecting the upper eyelid. The tarsal plate becomes hyperelastic with a loss of intrinsic rigidity. As a result, the eyelid is subjected to cyclic mechanical stress. This condition was used as a model to investigate changes in dynamic fibrobl...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.09-4724
更新日期:2010-08-01 00:00:00
abstract::Ultrastructural morphometric techniques were used to quantify pericyte degeneration in retinal and uveal capillaries of streptozotocin-diabetic rats in order to assess the suitability of this small rodent model of diabetes for studies of the pathogenesis of microvascular eye disease in diabetic humans. Male, Sprague-D...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1986-05-01 00:00:00
abstract:PURPOSE:Hormone diseases induce changes in the lacrimal gland (LG) and ocular surface (OS). Thyroid hormone (TH) induces cell proliferation and lipid metabolism through the activation of TH receptors. The aim of the present study was to evaluate the location and comparative expression of TH receptor beta-1 (Thrb) in LG...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.06-1309
更新日期:2007-07-01 00:00:00
abstract:PURPOSE:Adaptive optics scanning laser ophthalmoscopy (AOSLO) under optimized wavefront correction allows for routine imaging of foveal cone photoreceptors. The intersubject variability of foveal cone density was measured and its relation to eye length evaluated. METHODS:AOSLO was used to image 18 healthy eyes with ax...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.10-5499
更新日期:2010-12-01 00:00:00
abstract:PURPOSE:To compare the distributional parameters for a series of ocular biometric traits between twins and their singleton siblings, to evaluate the generalizability of twin data, as used in heritability analyses to the general population. METHODS:A series of birth, anthropometric, and 13 ocular biometric traits were ...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.11-7258
更新日期:2011-07-29 00:00:00