Abstract:
Purpose:To investigate the underlying mechanisms for how the mouse Cx50-R205G point mutation, a homologue of the human Cx50-R198W mutation that is linked to cataract-microcornea syndrome, affects proper lens growth and fiber cell differentiation to lead to severe lens phenotypes. Methods:EdU labeling, immunostaining, confocal imaging analysis, and primary lens epithelial cell culture were performed to characterize the lens epithelial cell (LEC) proliferation and fiber cell differentiation in wild-type and Cx50-R205G mutant lenses in vivo and in vitro. Results:The Cx50-R205G mutation severely disrupts the lens size and transparency. Heterozygous and homozygous Cx50-R205G mutant and Cx50 knockout lenses all show decreased central epithelium proliferation while only the homozygous Cx50-R205G mutant lenses display obviously decreased proliferating LECs in the germinative zone of neonatal lenses. Cultured Cx50-R205G lens epithelial cells reveal predominantly reduced Cx50 gap junction staining but no change of the endoplasmic reticulum stress marker BiP. The heterozygous Cx50-R205G lens fibers show moderately disrupted Cx50 and Cx46 gap junctions while the homozygous Cx50-R205G lens fibers have drastically reduced Cx50 and Cx46 gap junctions with severely altered fiber cell shape in vivo. Conclusions:The Cx50-R205G mutation inhibits both central and equatorial lens epithelial cell proliferation to cause small lenses. This mutation also disrupts the assembly and functions of both Cx50 and Cx46 gap junctions in lens fibers to alter fiber cell differentiation and shape to lead to severe lens phenotypes.
journal_name
Invest Ophthalmol Vis Scijournal_title
Investigative ophthalmology & visual scienceauthors
Tjahjono N,Xia CH,Li R,Chu S,Wang J,Gong Xdoi
10.1167/iovs.61.3.25subject
Has Abstractpub_date
2020-03-09 00:00:00pages
25issue
3eissn
0146-0404issn
1552-5783pii
2763319journal_volume
61pub_type
杂志文章abstract:PURPOSE:This study describes the effects of the cyclic adenosine monophosphate (cAMP) pathway on the tight junctional barrier of the corneal endothelium, which plays a critical role in maintaining the corneal stroma in an underhydrated, transparent state. METHODS:Subcultured bovine corneal endothelial cells grown on f...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1995-11-01 00:00:00
abstract::In order to investigate the effect of traction of the vasculature on induction or acceleration of neovascularization, the chorioallantoic membranes (CAMs) of the developing chick embryo were placed under tension with 10-0 nylon sutures and subsequently examined histologically. In the initial phase of the study, CAMs f...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1989-04-01 00:00:00
abstract:Purpose:To compare static rod function obtained with and without photobleach in control and intermediate age-related macular degeneration (iAMD) participants with and without subretinal drusenoid deposits (SDD). Methods:In this cross-sectional study, retinal sensitivities within the central 24° retina were obtained tw...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.18-23970
更新日期:2018-08-01 00:00:00
abstract:PURPOSE:Cataract is the most common cause of blindness in the world today, and yet there is no generally accepted treatment other than surgical intervention. Studies in rodent models designed to increase understanding of the molecular basis of cataract have shown that transforming growth factor (TGF)-beta induces morph...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:2000-10-01 00:00:00
abstract::The free epsilon-amino groups and 5-hydroxymethylfurfural (5-HMF) contents were determined in soluble and insoluble proteins of clear human lenses and diabetic and nondiabetic senile cataractous lenses. The free epsilon-amino group content of soluble proteins in diabetic cataracts was decreased by 37% (P less than 0.0...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1986-01-01 00:00:00
abstract:PURPOSE:To investigate the responses of retinal ganglion cells (RGCs) to electrical stimulation, using electrodes comparable in size to those used in human studies investigating the feasibility of an electronic retinal prosthesis. METHODS:Rabbit retinas were stimulated in vitro with current pulses applied to the inner...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.04-1018
更新日期:2005-04-01 00:00:00
abstract:PURPOSE:We assessed whether partial occlusion of the nonamblyopic eye with Bangerter filters can immediately reduce suppression and promote binocular summation of contrast in observers with amblyopia. METHODS:In Experiment 1, suppression was measured for 22 observers (mean age, 20 years; range, 14-32 years; 10 females...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.14-15224
更新日期:2014-10-28 00:00:00
abstract:PURPOSE:To determine the fate of donor epithelial, stromal, and endothelial cells after corneal transplantation in humans. METHODS:Fifty-two transplanted corneal buttons were explanted over a 2-year period from patients who required regrafting and had received corneas from donors of opposite sex. Fluorescence in situ ...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.08-2923
更新日期:2009-06-01 00:00:00
abstract:PURPOSE:Reading a text requires vergence angle adjustments, so that the images in the two eyes fall on corresponding retinal areas. Vergence adjustments bring the two retinal images into Panum's fusional area and therefore, small remaining errors or regulations do not lead to double vision. The present study evaluated ...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章,随机对照试验
doi:10.1167/iovs.11-8237
更新日期:2011-12-09 00:00:00
abstract:Purpose:Diabetes causes dysfunction in the retinal mitochondria and increases base mismatches in their DNA (mtDNA). The enzyme responsible for repairing the base mismatches, MutL homolog 1 (Mlh1), is compromised. Diabetes also favors many epigenetic modifications and activates DNA methylation machinery, and Mlh1 has a ...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.19-27602
更新日期:2019-09-03 00:00:00
abstract:PURPOSE:To examine the relationship between short-wavelength-sensitive (SWS) resolution acuity and epidemiologically defined stages of early age-related maculopathy (ARM). METHODS:Subjects consisted of 88 adults aged 51 to 87 years. Psychophysical testing was undertaken in only one eye of each subject (the study eye)....
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.05-1227
更新日期:2006-10-01 00:00:00
abstract:PURPOSE:To assess the effect of cataract on the evaluation of macular pigment optical density (MPOD) in aged patients. METHODS:MPOD was prospectively measured using autofluorescence spectrometry before and after cataract surgery. The Lens Opacities Classification System III was used to grade the cataracts at baseline....
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.10-5664
更新日期:2011-02-16 00:00:00
abstract::The amount of unscheduled DNA synthesis (UDS) produced in the cells adjacent to excimer laser ablations in the cornea was compared for 193-nm and 248-nm laser wavelengths. UDS is interpreted to indicate the process of excision repair of pyrimidine dimers formed in DNA. 193-nm laser ablation did not produce a statistic...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1987-02-01 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:Adeno-associated viral (AAV) vectors have been used to express several different proteins in the eye. The purpose of this study was to determine whether AAV-mediated intraocular gene transfer of pigment epithelium-derived factor (PEDF) inhibits the development of choroidal neovascularization (CNV) in a murine m...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:2002-06-01 00:00:00
abstract::Transmembrane electrical measurements were performed on the isolated rabbit iris-ciliary body (I-CB) to study the direct effects of halogenated inhalation anesthetic agents on the ciliary epithelium. Addition of either halothane, enflurane, or isoflurane to the control 95% O2:5% CO2 gas mixture resulted in a dose-depe...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1991-05-01 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:The global or gross response index of visual performance measured from the eye does not necessarily translate to global responses measured from the brain. A better understanding of this relationship would facilitate the monitoring of disease models that affect the visual pathway. We consider whether rod- and co...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.13-13003
更新日期:2014-02-20 00:00:00
abstract::Patients with choroidal melanomas and simulating choroidal tumors were tested in a masked manner in a leukocyte-migration inhibition (LMI) assay with a 3M KCl antigen derived from a metastatic choroidal melanoma. Good discrimination petween patient groups was observed with this antigen. The sensitivity and specificity...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1977-02-01 00:00:00
abstract:Purpose:Our goal was to generate and characterize a new mouse model in which only angiogenesis- and glaucoma-relevant tissues would be naturally fluorescent. The Matrix Gla (MGP) gene is highly expressed in vascular smooth muscle cells (VSMC) and trabecular meshwork (TM). We sought to direct our Mgp-Cre.KI mouse recomb...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.17-22992
更新日期:2018-02-01 00:00:00
abstract::The corneas of 12 eye donors with maturity-onset diabetes were obtained, and the corneal epithelial basement membranes were studied by transmission electron microscopy. Similar tissue was obtained from 12 nondiabetic eye donors who were matched for age (within 2 years) and race. The mean thickness of the corneal epith...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1981-04-01 00:00:00
abstract:PURPOSE:There are two human 15-lipoxygenases (LOX), 15-LOX-1 and -2, which convert arachidonic acid to 15S-hydroxyeicosatetraenoic acid (15S-HETE). The presence of both 15-LOXs in the human cornea prompted this study to delineate their roles in the human corneal epithelium. METHODS:Human corneal epithelia from donor c...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.04-1166
更新日期:2005-03-01 00:00:00
abstract:Purpose:The purpose of this study was to determine the relative roles of the sympathetic (SNS) and parasympathetic nervous system (PNS) in pupillary hippus. Methods:We used a paired-eye control study design with three cohorts receiving either 1.0% tropicamide (PNS antagonist) in light (TL), 1.0% tropicamide in dark (T...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.16-20785
更新日期:2017-01-01 00:00:00
abstract:PURPOSE:To assess the diagnostic validity of a fully automated image analysis algorithm of in vivo confocal microscopy images in quantifying corneal subbasal nerves to diagnose diabetic neuropathy. METHODS:One hundred eighty-six patients with type 1 and type 2 diabetes mellitus (T1/T2DM) and 55 age-matched controls un...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.13-13787
更新日期:2014-04-03 00:00:00
abstract:PURPOSE:Adeno-associated virus serotype 2 (AAV2) has been shown to be effective in transducing inner retinal neurons after intravitreal injection in several species. However, results in nonprimates may not be predictive of transduction in the human inner retina, because of differences in eye size and the specialized mo...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.10-6250
更新日期:2011-04-25 00:00:00
abstract:PURPOSE:Transforming growth factor beta-induced protein (TGFBIp; 68 kDa) is a secreted extracellular matrix (ECM) protein that has been demonstrated to regulate cell attachment in a variety of cell types. The sclera synthesizes and secretes TGFBIp, which may function to facilitate scleral ECM remodeling events associat...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.09-3460
更新日期:2009-08-01 00:00:00
abstract:PURPOSE:To determine new referents, or cutoff levels for tear film hyperosmolarity in the diagnosis of keratoconjunctivitis sicca (KCS) and to assess their effectiveness in independent patient groups. METHOD:A meta-analysis was performed on published data for tear osmolarity in samples of normal eyes and various subty...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章,meta分析
doi:10.1167/iovs.05-1504
更新日期:2006-10-01 00:00:00
abstract:PURPOSE:We characterized fluorescent bone marrow cells (YFP(+) BMCs) in the thy1-YFP mouse and determine if they promote trigeminal ganglion (TG) cell neurite growth. METHODS:Excimer laser annular keratectomy was performed in thy1-YFP mice, and corneas were imaged. BMCs were harvested from femur and tibia, and the exp...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:10.1167/iovs.13-12237
更新日期:2013-09-03 00:00:00
abstract::Total outflow facility was determined by two-level constant pressure perfusion of the anterior chamber in surgically virgin, aniridic, and ciliary muscle disinserted cynomolgus monkey eyes 6 to 13 times at 1- to 2-month intervals over periods of 8 to 24 months. Facility decreased approximately 15 to 20% between consec...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1983-02-01 00:00:00
abstract::Sixteen patients with osteogenesis imperfecta (OI) have undergone a thorough eye examination. These patients had statistically significantly lower ocular rigidity measurements than a group of normal volunteers matched on age, sex, and refractive error. In addition, the corneal diameter and length of the eyeball was sm...
journal_title:Investigative ophthalmology & visual science
pub_type: 杂志文章
doi:
更新日期:1981-06-01 00:00:00