Ocular abnormalities in mice lacking the Ski proto-oncogene.

Abstract:

PURPOSE:Persistent hyperplastic primary vitreous (PHPV) is a developmental ocular malformation often associated with additional ocular abnormalities. This study involved a novel mouse model of PHPV, generated by a null mutation of the Ski proto-oncogene, that displays other anterior segment and retinal malformations often found in human cases of PHPV. METHODS:Morphologic and histologic analyses of Ski-/- mice were used to document ocular abnormalities in comparison to those of normal littermates. Immunohistochemical studies were used to examine the expression of relevant markers of ocular and vascular development including Pax6, beta-III tubulin, and Flk1. RESULTS:PHPV and microphthalmia were found in 100% of Ski-/- fetuses. Other abnormalities included anterior segment and lens dysgenesis, retinal folds, chorioretinal coloboma, and Peters anomaly. The severity was variable, even in a highly homogeneous genetic background. PHPV was characterized by the presence of retrolental fibrous and vascular tissue that did not express the neuronal marker beta-III tubulin, but was positive for Flk1 expression and contained no obviously pigmented cells. CONCLUSIONS:The results show that normal ocular development requires the function of the Ski proto-oncogene, and mice lacking Ski have many features associated with PHPV, and some similarities with Peters anomaly in humans. Defects in Ski-/- mice closely resemble those described in animals lacking several of the retinoic acid receptor genes, or in animals exposed to excess retinoic acid during gestation. Ski has been shown to repress transcription induced by retinoic acid signaling, and may thus affect ocular development by regulating RA signaling.

authors

McGannon P,Miyazaki Y,Gupta PC,Traboulsi EI,Colmenares C

doi

10.1167/iovs.05-1543

subject

Has Abstract

pub_date

2006-10-01 00:00:00

pages

4231-7

issue

10

eissn

0146-0404

issn

1552-5783

pii

47/10/4231

journal_volume

47

pub_type

杂志文章
  • Confocal microscopy reveals zones of membrane remodeling in the outer cortex of the human lens.

    abstract:PURPOSE:To optimize fixation, sectioning, and immunolabeling protocols to map the morphology of the human lens with confocal microscopy. METHODS:Transparent human lenses were fixed in 0.75% paraformaldehyde for 24 hours, cut in half, and fixed for another 24 hours. Lenses were cryoprotected, sectioned, and labeled wit...

    journal_title:Investigative ophthalmology & visual science

    pub_type: 杂志文章

    doi:10.1167/iovs.09-3435

    authors: Lim JC,Walker KL,Sherwin T,Schey KL,Donaldson PJ

    更新日期:2009-09-01 00:00:00

  • Neuroprotectin D1 synthesis and corneal nerve regeneration after experimental surgery and treatment with PEDF plus DHA.

    abstract:PURPOSE:This study was conducted to define whether pigment epithelial-derived growth factor (PEDF), together with docosahexaenoic acid (DHA), enhances the synthesis of neuroprotectin D1 (NPD1) and the regeneration of corneal nerves damaged after surgery. METHODS:Corneal stromal dissection was performed in the left eye...

    journal_title:Investigative ophthalmology & visual science

    pub_type: 杂志文章

    doi:10.1167/iovs.09-3641

    authors: Cortina MS,He J,Li N,Bazan NG,Bazan HE

    更新日期:2010-02-01 00:00:00

  • 3D collagen orientation study of the human cornea using X-ray diffraction and femtosecond laser technology.

    abstract:PURPOSE:To study the distribution and predominant orientations of fibrillar collagen at different depths throughout the entire thickness of the human cornea. This information will form the basis of a full three-dimensional reconstruction of the preferred orientations of corneal lamellae. METHODS:Femtosecond laser tech...

    journal_title:Investigative ophthalmology & visual science

    pub_type: 杂志文章

    doi:10.1167/iovs.09-3669

    authors: Abahussin M,Hayes S,Knox Cartwright NE,Kamma-Lorger CS,Khan Y,Marshall J,Meek KM

    更新日期:2009-11-01 00:00:00

  • Blockade of insulin receptor substrate-1 inhibits corneal lymphangiogenesis.

    abstract:PURPOSE:To analyze whether insulin receptor substrate (IRS-1) is involved in lymphatic vessel development and whether IRS-1 blockade can inhibit lymphangiogenesis in vivo. METHODS:The impact of IRS-1 blockade by GS-101 (Aganirsen), an antisense oligonucleotide against IRS-1, on lymphatic endothelial cell (LEC) prolife...

    journal_title:Investigative ophthalmology & visual science

    pub_type: 杂志文章

    doi:10.1167/iovs.10-6816

    authors: Hos D,Regenfuss B,Bock F,Onderka J,Cursiefen C

    更新日期:2011-07-29 00:00:00

  • A comprehensive survey of mutations in the OPA1 gene in patients with autosomal dominant optic atrophy.

    abstract:PURPOSE:To characterize the spectrum of mutations in the OPA1 gene in a large international panel of patients with autosomal dominant optic atrophy (adOA), to improve understanding of the range of functional deficits attributable to sequence variants in this gene, and to assess any genotype-phenotype correlations. MET...

    journal_title:Investigative ophthalmology & visual science

    pub_type: 杂志文章,多中心研究

    doi:

    authors: Thiselton DL,Alexander C,Taanman JW,Brooks S,Rosenberg T,Eiberg H,Andreasson S,Van Regemorter N,Munier FL,Moore AT,Bhattacharya SS,Votruba M

    更新日期:2002-06-01 00:00:00

  • Linkage of a mild late-onset phenotype of Fuchs corneal dystrophy to a novel locus at 5q33.1-q35.2.

    abstract:PURPOSE:To identify the disease locus associated with autosomal dominant Fuchs corneal dystrophy (FCD) in a large family and to compare the progression of severity in families mapped to the FCD1 and FCD2 loci. METHODS:Seventeen individuals in a large family were examined by slit lamp biomicroscopy. Blood samples were ...

    journal_title:Investigative ophthalmology & visual science

    pub_type: 杂志文章

    doi:10.1167/iovs.09-3764

    authors: Riazuddin SA,Eghrari AO,Al-Saif A,Davey L,Meadows DN,Katsanis N,Gottsch JD

    更新日期:2009-12-01 00:00:00

  • Tear meniscus volume in dry eye after punctal occlusion.

    abstract::Purpose. To use optical coherence tomography (OCT) to evaluate the effect of punctal occlusion on tear meniscus volume in dry eye patients. Methods. Occlusion of both upper and lower puncta with collagen plugs was performed on one eye each in 20 dry eye patients and 20 normal subjects. The upper and lower tear menisci...

    journal_title:Investigative ophthalmology & visual science

    pub_type: 杂志文章

    doi:10.1167/iovs.09-4349

    authors: Chen F,Shen M,Chen W,Wang J,Li M,Yuan Y,Lu F

    更新日期:2010-04-01 00:00:00

  • Definition of ocular antigens in ciliary body and retinal ganglion cells by the marker antibody pANCA.

    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:

    authors: Gordon LK,Eggena M,Targan SR,Braun J

    更新日期:1999-05-01 00:00:00

  • An elevated hematogenous photosensitizer in the preterm neonate.

    abstract:PURPOSE:Human blood contains low levels of protoporphyrin IX (PP IX), a photoactive compound that produces reactive oxygen species when exposed to light. It has been proposed that photoactivation of PP IX and subsequent generation of potentially tissue-damaging reactive oxygen may be a mechanism of retinal injury in re...

    journal_title:Investigative ophthalmology & visual science

    pub_type: 杂志文章

    doi:

    authors: Bynoe LA,Gottsch JD,Sadda SR,Panton RW,Haller EM,Gleason CA

    更新日期:1993-09-01 00:00:00

  • Cone differentiation with no photopigment coexpression.

    abstract:PURPOSE:To decide whether the transitory coexpression of cone visual pigments described in the developing rat and gerbil retina is a universal feature of dichromatic mammalian species. METHODS:The rabbit, a species widely used in eye research, was selected for the study and a search made for the presence of cones that...

    journal_title:Investigative ophthalmology & visual science

    pub_type: 杂志文章

    doi:

    authors: Szepessy Z,Lukáts A,Fekete T,Barsi A,Röhlich P,Szél A

    更新日期:2000-09-01 00:00:00

  • Characteristics of Hyaluronan Synthesis Inhibition by 4-Methylumbelliferone in Orbital Fibroblasts.

    abstract:Purpose:Hyaluronan (HA) overproduction by orbital fibroblasts (OFs) is a major factor in the pathogenesis of Graves' orbitopathy (GO). 4-methylumbelliferone (4-MU) is an inhibitor of HA synthesis in different cell types in vitro and has beneficial effects in animal models of autoimmune diseases. Methods:HA production ...

    journal_title:Investigative ophthalmology & visual science

    pub_type: 杂志文章

    doi:10.1167/iovs.61.2.27

    authors: Galgoczi E,Jeney F,Katko M,Erdei A,Gazdag A,Sira L,Bodor M,Berta E,Ujhelyi B,Steiber Z,Gyory F,Nagy EV

    更新日期:2020-02-07 00:00:00

  • Corneal endothelial cell survival in organ cultures under acute oxidative stress: effect of VIP.

    abstract:PURPOSE:Human corneal endothelium, a neural crest-derived tissue, has a very limited regenerative capacity and may depend on trophic factors for its survival throughout life, as well as after injury and during storage before transplantation. The purpose of this study was to determine whether vasoactive intestinal pepti...

    journal_title:Investigative ophthalmology & visual science

    pub_type: 杂志文章

    doi:

    authors: Koh SW,Waschek JA

    更新日期:2000-12-01 00:00:00

  • Lacrimal gland involvement in graft-versus-host disease: a murine model.

    abstract:PURPOSE:To describe lacrimal gland involvement in a murine model of acute graft-versus-host disease (GVHD). METHODS:Histopathologic examination was performed on lacrimal glands of mice affected by GVHD at 1, 2, 4, and 6 weeks after allogeneic bone marrow transplantation (BMT). Histopathologic scoring, based on charact...

    journal_title:Investigative ophthalmology & visual science

    pub_type: 杂志文章

    doi:10.1167/iovs.05-0040

    authors: Hassan AS,Clouthier SG,Ferrara JL,Stepan A,Mian SI,Ahmad AZ,Elner VM

    更新日期:2005-08-01 00:00:00

  • Cataract and the acceleration of calpain-induced beta-crystallin insolubilization occurring during normal maturation of rat lens.

    abstract:PURPOSE:To determine if limited proteolysis of beta-crystallins is associated with insolubilization of proteins in rats lens during maturation and to test if the protease, calpain II, is involved. METHODS:Soluble and insoluble lens proteins from 4-day-old to 4-month-old rat lens cortexes and nuclei were separated by t...

    journal_title:Investigative ophthalmology & visual science

    pub_type: 杂志文章

    doi:

    authors: David LL,Azuma M,Shearer TR

    更新日期:1994-03-01 00:00:00

  • Effects of Narrowband Light on Choroidal Thickness and the Pupil.

    abstract:Purpose:To determine the effects of narrowband light exposure on choroidal thickness and the pupil response in humans. Methods:Twenty subjects, ages 21 to 43 years, underwent 1 hour of exposure to broadband, short wavelength "blue," or long wavelength "red" light, or darkness. Choroidal thickness, imaged with spectral...

    journal_title:Investigative ophthalmology & visual science

    pub_type: 杂志文章

    doi:10.1167/iovs.61.10.40

    authors: Lou L,Ostrin LA

    更新日期:2020-08-03 00:00:00

  • Localization of alkaline phosphatase on basolateral plasma membrane of normal and regenerating retinal pigment epithelium. A cytochemical study in rabbits.

    abstract::Ultrastructural cytochemistry was used to analyze the polarized distribution of alkaline phosphatase (AP) on the plasma membranes of normal and regenerating retinal pigment epithelium (RPE) and the ciliary body epithelium in rabbits. The AP activity was concentrated on the basolateral plasma membrane in normal RPE. In...

    journal_title:Investigative ophthalmology & visual science

    pub_type: 杂志文章

    doi:

    authors: Korte GE,Rappa E,Andracchi S

    更新日期:1991-12-01 00:00:00

  • Delayed regeneration of foveal cone photopigments in Vogt-Koyanagi-Harada disease at the convalescent stage.

    abstract:PURPOSE:To evaluate the physiological characteristics of the macula in patients with Vogt-Koyanagi-Harada disease during the convalescent stage with specific reference to the kinetics of foveal cone photopigment regeneration. METHODS:Six eyes of three patients at the convalescent stage of Vogt-Koyanagi-Harada disease ...

    journal_title:Investigative ophthalmology & visual science

    pub_type: 杂志文章

    doi:10.1167/iovs.03-0381

    authors: Okamoto Y,Miyake Y,Horio N,Takakuwa H,Yamamoto E,Terasaki H

    更新日期:2004-01-01 00:00:00

  • Localization of corneal epithelial stem cells in the developing rat.

    abstract::A monoclonal antibody, 4G10.3, was developed that preferentially binds limbal basal cells in adult rat, rabbit, and human corneas. These cells were hypothesized to be the stem cells for the corneal epithelium. The antibody 4G10.3 was localized by immunofluorescence microscopy in rats 1 d and 1, 1.5, 2, 3, 4, and 6 wk ...

    journal_title:Investigative ophthalmology & visual science

    pub_type: 杂志文章

    doi:

    authors: Chung EH,Bukusoglu G,Zieske JD

    更新日期:1992-06-01 00:00:00

  • Hyaluronan Derived From the Limbus is a Key Regulator of Corneal Lymphangiogenesis.

    abstract:Purpose:We recently reported that the glycosaminoglycan hyaluronan (HA), which promotes inflammatory angiogenesis in other vascular beds, is an abundant component of the limbal extracellular matrix. Consequently, we have explored the possibility that HA contributes to lymphangiogenesis in the inflamed cornea. Methods:...

    journal_title:Investigative ophthalmology & visual science

    pub_type: 杂志文章

    doi:10.1167/iovs.18-25920

    authors: Sun M,Puri S,Mutoji KN,Coulson-Thomas YM,Hascall VC,Jackson DG,Gesteira TF,Coulson-Thomas VJ

    更新日期:2019-03-01 00:00:00

  • Immunohistologic evidence for retinal glial cell changes in human glaucoma.

    abstract:PURPOSE:Glial cells are structurally and functionally linked to neuronal tissues. Pathologically, the cells may be activated and characterized by increased size and number and altered cellular properties. In glaucoma, pathologic mechanisms within the anterior optic nerve may include glial activation. This study examine...

    journal_title:Investigative ophthalmology & visual science

    pub_type: 杂志文章

    doi:

    authors: Wang L,Cioffi GA,Cull G,Dong J,Fortune B

    更新日期:2002-04-01 00:00:00

  • IFT81 as a Candidate Gene for Nonsyndromic Retinal Degeneration.

    abstract:Purpose:IFT81, a core component of the IFT-B complex, involved in the bidirectional transport of ciliary proteins, has been recently implicated in syndromic ciliopathies. However, none of the IFT-B core complex proteins have been associated with nonsyndromic retinal dystrophies. Given the importance of ciliary transpor...

    journal_title:Investigative ophthalmology & visual science

    pub_type: 杂志文章

    doi:10.1167/iovs.16-19133

    authors: Dharmat R,Liu W,Ge Z,Sun Z,Yang L,Li Y,Wang K,Thomas K,Sui R,Chen R

    更新日期:2017-05-01 00:00:00

  • Degradation of Visual Performance With Increasing Levels of Retinal Stray Light.

    abstract:Purpose:To quantify the effect of induced stray light on halo size, luminance threshold, and contrast sensitivity. Methods:Retinal stray light was induced in five healthy subjects using different photographic filters. The stray light induced ranged from levels observed in intraocular lenses (IOLs) with glistenings (lo...

    journal_title:Investigative ophthalmology & visual science

    pub_type: 杂志文章

    doi:10.1167/iovs.15-18514

    authors: van der Mooren M,Rosén R,Franssen L,Lundström L,Piers P

    更新日期:2016-10-01 00:00:00

  • Effect of erythropoietin axotomy-induced apoptosis in rat retinal ganglion cells.

    abstract:PURPOSE:Erythropoietin (EPO) modulates erythropoiesis by inhibiting apoptosis in erythrocyte progenitors. Recently, EPO has been shown to be protective in experimental models of mechanical trauma, neuroinflammation, cerebral and retinal ischemia, and even in a human stroke trial. However, little is known about EPO sign...

    journal_title:Investigative ophthalmology & visual science

    pub_type: 杂志文章

    doi:10.1167/iovs.03-1039

    authors: Weishaupt JH,Rohde G,Pölking E,Siren AL,Ehrenreich H,Bähr M

    更新日期:2004-05-01 00:00:00

  • Detection of cell adhesion molecules in lens epithelial cells of human cataracts.

    abstract:PURPOSE:To detect the expression of cell adhesion molecules (CAMs), including beta integrins, intercellular adhesion molecule-1 (ICAM-1), vascular cell adhesion molecule-1, leukocyte-endothelial cell adhesion molecule-2, E-cadherin, and CD44, in cultured lens epithelial cells (LECs) of human cataracts. To show that LEC...

    journal_title:Investigative ophthalmology & visual science

    pub_type: 杂志文章

    doi:

    authors: Nishi O,Nishi K,Akaishi T,Shirasawa E

    更新日期:1997-03-01 00:00:00

  • Change in the synthesis rates of ocular retinoic acid and scleral glycosaminoglycan during experimentally altered eye growth in marmosets.

    abstract:PURPOSE:The purpose of this study was to examine the possibility that all-trans-retinoic acid (RA) in the eye is a signal related to changes in scleral extracellular matrix in a primate model of postnatal eye growth. METHODS:Juvenile marmosets (Callithrix jacchus) were divided into two experimental groups based on the...

    journal_title:Investigative ophthalmology & visual science

    pub_type: 杂志文章

    doi:10.1167/iovs.05-0298

    authors: Troilo D,Nickla DL,Mertz JR,Summers Rada JA

    更新日期:2006-05-01 00:00:00

  • In vitro growth of Chlamydia trachomatis in conjunctival and corneal epithelium.

    abstract::This study used primary cultures of conjunctival and corneal epithelial cells from rabbit, monkey and humans to investigate the infection process and host interactions of chlamydia. The epithelial cells were isolated from bulbar and palpebral conjunctivae and the cornea following incubation with EDTA or dispase and mi...

    journal_title:Investigative ophthalmology & visual science

    pub_type: 杂志文章

    doi:

    authors: Patton DL,Chan KY,Kuo CC,Cosgrove YT,Langley L

    更新日期:1988-07-01 00:00:00

  • Mislocalization and degradation of human P23H-rhodopsin-GFP in a knockin mouse model of retinitis pigmentosa.

    abstract:PURPOSE:To engineer a knockin mouse model that can be used to monitor the effects of treatments on degradation and mislocalization of proline-to-histidine change at codon 23 (P23H) rhodopsin, a common cause of autosomal dominant retinitis pigmentosa (ADRP). The goal was to introduce a gene that expressed rhodopsin at l...

    journal_title:Investigative ophthalmology & visual science

    pub_type: 杂志文章

    doi:10.1167/iovs.11-8654

    authors: Price BA,Sandoval IM,Chan F,Simons DL,Wu SM,Wensel TG,Wilson JH

    更新日期:2011-12-28 00:00:00

  • Alpha2-Adrenergic-Agonist Brimonidine Stimulates Negative Feedback and Attenuates Injury-Induced Phospho-ERK and Dedifferentiation of Chicken Müller Cells.

    abstract:PURPOSE:Retinal injury induces Müller cell dedifferentiation by activating extracellular signal-regulated kinase (ERK) signaling. Stimulation of α2-adrenergic receptors protects against injury but also activates ERK in Müller cells. The purpose of this work was to study the effect of α2-adrenergic signaling on injury-i...

    journal_title:Investigative ophthalmology & visual science

    pub_type: 杂志文章

    doi:10.1167/iovs.15-16816

    authors: Harun-Or-Rashid M,Díaz-DelCastillo M,Galindo-Romero C,Hallböök F

    更新日期:2015-09-01 00:00:00

  • Interphotoreceptor Retinoid-Binding Protein Mitigates Cellular Oxidative Stress and Mitochondrial Dysfunction Induced by All-trans-Retinal.

    abstract:PURPOSE:Point and null mutations in interphotoreceptor retinoid-binding protein (IRBP) cause retinal dystrophy in affected patients and IRBP-deficient mice with unknown mechanism. This study investigated whether IRBP protects cells from damages induced by all-trans-retinal (atRAL), which was increased in the Irbp(-/-) ...

    journal_title:Investigative ophthalmology & visual science

    pub_type: 杂志文章

    doi:10.1167/iovs.15-18551

    authors: Lee M,Li S,Sato K,Jin M

    更新日期:2016-04-01 00:00:00

  • Malnutrition and retinal vascular caliber in the elderly: the POLA study.

    abstract:PURPOSE:The pathway linking late-life malnutrition to greater risk of cardiovascular disease is unclear. Microcirulatory changes assessed by retinal vascular caliber have been linked with increased risk of stroke and coronary heart disease. The purpose of this study was to examine whether retinal vascular calibers are ...

    journal_title:Investigative ophthalmology & visual science

    pub_type: 杂志文章

    doi:10.1167/iovs.13-13721

    authors: Daien V,Carriere I,Kawasaki R,Cristol JP,Villain M,Fesler P,Ritchie K,Delcourt C,POLA Study Group.

    更新日期:2014-05-27 00:00:00