Abstract:
Purpose:The present study investigated retinal glia and choroidal vessels in flatmounts and sections from individuals with clinically diagnosed Stargardt disease (STGD). Methods:Eyes from three donors clinically diagnosed with STGD were obtained through the Foundation Fighting Blindness (FFB). Genetic testing was performed to determine the disease-causing mutations. Eyes were enucleated and fixed in 4% paraformaldehyde and 0.5% glutaraldehyde. After imaging, retinas were dissected and immunostained for glial fibrillary acidic protein, vimentin, and peanut agglutin. Following RPE removal, the choroid was immunostained with Ulex europaeus agglutinin lectin. For each choroid, the area of affected vasculature, percent vascular area, and choriocapillaris luminal diameters were measured. The retina from one donor was hemisected and cryopreserved or embedded in JB-4 for cross-section analysis. Results:Genetic testing confirmed the STGD diagnosis in donor 1, whereas a mutation in peripherin 2 was identified in donor 3. Genetic testing was not successful on donor 2. Therefore, only donor 1 can definitively be classified as having STGD. All donors had areas of RPE atrophy within the macular region, which correlated with underlying choriocapillaris loss. In addition, Müller cells formed pre- and subretinal membranes. Subretinal gliotic membranes correlated almost identically with RPE and choriocapillaris loss. Conclusions:Despite bearing different genetic mutations, all donors demonstrated choriocapillaris loss and Müller cell membranes correlating with RPE loss. Müller cell remodeling was most extensive in the donor with the peripherin mutation, whereas choriocapillaris loss was greatest in the confirmed STGD donor. This study emphasizes the importance of genetic testing when diagnosing macular disease.
journal_name
Invest Ophthalmol Vis Scijournal_title
Investigative ophthalmology & visual scienceauthors
Edwards MM,Bonilha VL,Bhutto IA,Bell BA,McLeod DS,Hollyfield JG,Lutty GAdoi
10.1167/iovs.61.8.27subject
Has Abstractpub_date
2020-07-01 00:00:00pages
27issue
8eissn
0146-0404issn
1552-5783pii
2770344journal_volume
61pub_type
杂志文章abstract:Purposes:Trabeculectomy is a surgical procedure for lowering intraocular pressure in glaucoma patients, in which excessive scarring leading to failure of the filtering bleb adversely affects the surgical outcome. Heightened Tenon's capsule fibroblast (TCF) proliferation and extracellular matrix (ECM) deposition are imp...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.16-19480
更新日期:2016-10-01 00:00:00
abstract:PURPOSE:A subset of patients with anterior uveitis express the marker, perinuclear anti-neutrophil cytoplasmic antibody (pANCA). In this study, recombinantly isolated pANCA monoclonal antibodies were used to search for ocular cells expressing the pANCA antigen. METHODS:Paraffin sections of human ocular tissues obtaine...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1999-05-01 00:00:00
abstract:PURPOSE:Imaging studies suggest that the choroidal vasculature may be altered in central serous chorioretinopathy. Little is known, however, about the regulation of ocular blood flow in patients with central serous chorioretinopathy (CSC). The hypothesis for the present study was that choroidal blood flow changes durin...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.05-0268
更新日期:2005-12-01 00:00:00
abstract:PURPOSE:To describe a method for assessment and individualized compensation of anterior segment birefringence with scanning laser polarimetry. METHODS:A scanning laser polarimeter (GDx Nerve Fiber Analyzer; Laser Diagnostic Technologies, Inc., San Diego, CA) was modified to accommodate a variable compensator. The magn...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:2002-07-01 00:00:00
abstract::The morphologic effects on rabbit corneal endothelium of several common ophthalmic vehicle constituents were examined following subconjunctival administration. Profound dose-dependent changes consisting of intercellular vacuolization and thickening of the endothelial layer were noted within 1 day following administrat...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1986-04-01 00:00:00
abstract:PURPOSE:To describe and interpret "choroidal caverns," an unreported optical coherence tomography (OCT) finding in the choroid of patients with geographic atrophy (GA) secondary to atrophic AMD. METHODS:Retrospective analysis of patients with GA. Main outcomes measures included estimation of the prevalence of choroida...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.16-19083
更新日期:2016-05-01 00:00:00
abstract::The Early Treatment Diabetic Retinopathy Study (ETDRS) and other standardized classification schemes have laid a foundation for tremendous advances in the understanding and management of diabetic retinopathy (DR). However, technological advances in optics and image analysis, especially optical coherence tomography (OC...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.17-21873
更新日期:2018-01-01 00:00:00
abstract:PURPOSE:To compare local functional measures, the multifocal electroretinogram (mfERG) and visual field sensitivity, with a local structural measure, spectral domain (SD) optical coherence tomography (OCT), of receptor damage in patients with retinitis pigmentosa (RP). METHODS:MfERGs, visual fields, and SD-OCT scans w...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.11-8410
更新日期:2012-02-21 00:00:00
abstract:PURPOSE:To investigate the molecular basis of hereditary lattice corneal dystrophy (LCD) type IIIA associated with corneal amyloid deposits afflicting several members of a four-generation family. METHODS:Histologic, immunohistochemical and biochemical studies were performed on corneal tissue samples obtained after per...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.04-1319
更新日期:2005-04-01 00:00:00
abstract::Failure of corneal grafts is thought to involve the development and activation of specifically sensitized T-cells. One method which might be used to circumvent the development of such cells is the phenomenon of anterior chamber-associated immune deviation (ACAID). Injection of antigen into the anterior chamber of the ...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1990-10-01 00:00:00
abstract:PURPOSE:To investigate the protective effects of cobalt protoporphyrin (CoPP), a potent heme oxygenase (HO)-1 inducer, in a rat model of ischemia-reperfusion injury and to document the possible antiapoptotic and anti-inflammatory mechanisms underlying the protection. METHODS:Rats pretreated with intraperitoneal inject...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.09-4086
更新日期:2010-09-01 00:00:00
abstract::Demeclocycline, tetracycline and other tetracycline derivatives lowered intraocular pressure (IOP) in rabbits following intravitreal injection, but the onset of this effect was not evident until 1 or more days after drug administration. Of the drugs tested, demeclocycline was the most active ocular hypotensive agent. ...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1989-07-01 00:00:00
abstract:PURPOSE:To compare the Swedish interactive thresholding algorithm (SITA) with the full-threshold (FT) strategy for short-wavelength automated perimetry (SWAP). METHODS:One eye of 286 patients with glaucomatous optic neuropathy (GON) and 289 age-matched participants without GON from the Diagnostic Innovations in Glauco...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.08-2718
更新日期:2009-04-01 00:00:00
abstract::The phosphatic metabolite content of specific anatomic regions within the crystalline lens was determined by phosphorus-31 nuclear magnetic resonance analyses performed on tissue perchloric acid (PCA) extracts. Anatomically distinct zones each were dissected from five sets of 10 porcine lenses, isolated, and frozen in...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1985-04-01 00:00:00
abstract:Purpose:Age-related macular degeneration (AMD) is a progressive disease with multifactorial etiology. There is a need to identify clinical features that are harbingers of advanced disease. We evaluated morphologic features of the retina and choroid on optical coherence tomography (OCT) to determine if they predict prog...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.17-21696
更新日期:2017-07-01 00:00:00
abstract:PURPOSE:Dietary supplementation with vitamin A is sometimes prescribed as a treatment for retinitis pigmentosa, a group of inherited retinal degenerations that cause progressive blindness. Loss-of-function mutations in the ABCA4 gene are responsible for a subset of recessive retinitis pigmentosa. Other mutant alleles o...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.07-1470
更新日期:2008-09-01 00:00:00
abstract:PURPOSE:To investigate the alterations in microRNA (miRNA) expression during replicative senescence (RS) in human trabecular meshwork (HTM) cells. METHODS:Two HTM cell lines were serially passaged until they reached RS. Changes in expression of 30 miRNAs were assessed by real-time quantitative (q)-PCR. The effects of ...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.09-4874
更新日期:2010-06-01 00:00:00
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 obse...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.14-14910
更新日期:2014-08-28 00:00:00
abstract:PURPOSE:To determine the impact of substratum compliance and latrunculin-B (Lat-B), both alone and together, on fundamental human trabecular meshwork (HTM) cell behavior. Lat-B is a reversible actin cytoskeleton disruptor that decreases resistance to aqueous humor outflow and decreases intraocular pressure. METHODS:HT...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.11-7857
更新日期:2011-12-02 00:00:00
abstract::In an anisotonic medium the tendency of the lens to swell or shrink in response to the osmotic gradient is countered by a change in the content of lens solute, so that the change in the volume of lens water is minimal. This phenomenon is known as volume regulation. The rapid phase of volume regulation occurs in less t...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1981-01-01 00:00:00
abstract:PURPOSE:To investigate the pharmacologic effect of tetramethylpyrazine (TMP) on human Tenon's fibroblasts (HTFs), the cells implicated in scarring after filtration surgery. METHODS:Transforming growth factor-β2 (TGF-β2) was used to stimulate a fibrotic phenotype in primary HTFs, and the influence of TMP on the fibroti...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.16-19529
更新日期:2016-09-01 00:00:00
abstract:PURPOSE:CD4(+)CD25(+)FoxP3(+) naturally occurring regulatory T cells (Tregs) maintain self-tolerance and function to suppress overly exuberant immune responses. However, it is unclear whether innate immune cells modulate Treg function. Here the authors examined the role of innate immunity in lymphomyeloid homeostasis. ...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.10-6536
更新日期:2011-04-08 00:00:00
abstract:PURPOSE:To assess the repeatability and reproducibility of central corneal thickness (CCT) measurements obtained by combined scanning-slit/Placido-disc topography (Orbscan II; Bausch & Lomb, Rochester, NY) after laser in situ keratomileusis (LASIK) and to compare the results with another noncontact method, specular mic...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.09-3408
更新日期:2009-09-01 00:00:00
abstract:PURPOSE:To evaluate the intraocular safety and pharmacokinetics of hexadecyloxypropyl-cidofovir (HDP-CDV), the hydrolysis product of HDP-cyclic-CDV, a long-lasting intravitreal cidofovir prodrug for cytomegalovirus (CMV) retinitis. METHODS:HDP-cyclic-CDV was suspended in phosphate-buffered saline (PBS) at 37°C and for...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.11-8293
更新日期:2011-12-09 00:00:00
abstract:PURPOSE:To study the relationships between the refractive power of the crystalline lens, overall refractive error of the eye, and degree of nuclear cataract. METHODS:All phakic participants of the population-based Central India Eye and Medical Study with an age of 50+ years were included. Calculation of the refractive...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章,多中心研究
doi:10.1167/iovs.11-8523
更新日期:2012-02-01 00:00:00
abstract:Purpose:The purpose of this paper was to discuss manifestations of primary mitochondrial dysfunctions and whether the retinal pigment epithelium or the photoreceptors are preferentially affected. Methods:A retrospective analysis was performed of patients with clinically and laboratory confirmed diagnoses of maternally...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.61.12.12
更新日期:2020-10-01 00:00:00
abstract:PURPOSE:To test the effect of valacyclovir alone and with aspirin on the asymptomatic shedding of HSV-1 DNA in tears and saliva of healthy individuals. METHOD. The subjects (n = 45) were randomized into three groups without regard to age, sex, or race. Group 1 (n = 14) received the placebo, group 2 (n = 15) received a ...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章,随机对照试验
doi:10.1167/iovs.09-3729
更新日期:2009-12-01 00:00:00
abstract:Purpose:To define the nature and extent of temporal frequency abnormalities in diabetics who have mild or no nonproliferative diabetic retinopathy (NPDR) by using the flicker electroretinogram (ERG). Methods:Light-adapted flicker ERGs were recorded from 20 diabetics who have no clinically apparent retinopathy, 20 diab...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.18-25199
更新日期:2018-10-01 00:00:00
abstract:PURPOSE:Leber congenital amaurosis (LCA) is an inherited retinal disease that causes early-onset severe visual impairment. To evaluate the mutation spectrum in the Chinese population, we performed a mutation screen in 145 Chinese LCA families. METHODS:First, we performed direct Sanger sequencing of 7 LCA disease genes...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.14-15972
更新日期:2015-06-01 00:00:00
abstract:PURPOSE:The outcome of glaucoma filtration surgery is affected by subconjunctival wound healing. The effects of the antiglaucoma drug latanoprost on the contractility of human Tenon fibroblasts (HTFs) cultured in a three-dimensional collagen gel were investigated. METHODS:HTFs were cultured in a type I collagen gel wi...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.07-0451
更新日期:2008-04-01 00:00:00