Abstract：
:Autophagy is a lysosomal degradative process that is essential for cellular homeostasis and metabolic stress adaptation. Defective autophagy is involved in the pathogenesis of many diseases including granular corneal dystrophy type 2 (GCD2). GCD2 is an autosomal dominant disorder caused by substitution of histidine for arginine at codon 124 (R124H) in the transforming growth factor β-induced gene (TGFBI) on chromosome 5q31. Transforming growth factor β-induced protein (TGFBIp) is degraded by autophagy, but mutant-TGFBIp accumulates in autophagosomes and/or lysosomes, despite significant activation of basal autophagy, in GCD2 corneal fibroblasts. Furthermore, inhibition of autophagy induces cell death of GCD2 corneal fibroblasts through active caspase-3. As there is currently no pharmacological treatment for GCD2, development of novel therapies is required. A potential strategy for preventing cytoplasmic accumulation of mutant-TGFBIp in GCD2 corneal fibroblasts is to enhance mutant-TGFBIp degradation. This could be achieved by activation of the autophagic pathway. Here, we will consider the role and the potential therapeutic benefits of autophagy in GCD2, with focus on TGFBIp degradation, in light of the recently established role of autophagy in protein degradation.
journal_name
Exp Eye Resjournal_title
Experimental eye researchauthors
Choi SI,Kim EKdoi
10.1016/j.exer.2015.09.008subject
Has Abstractpub_date
2016-03-01 00:00:00pages
14-21eissn
0014-4835issn
1096-0007pii
S0014-4835(15)30029-4journal_volume
144pub_type
杂志文章,评审abstract：:The effects of temperature on rod outer segment (ROS) length and membrane shedding were studied in a cyprinodont fish, Fundulus zebrinus. After 30 days in 14L/10D cyclic light and 17 degrees C, ROS length averaged 41.2 microns. Fish were then exposed to 7, 17 or 27 degrees C for 10 and 25 days before being sampled 5 h...
journal_title：Experimental eye research
pub_type： 杂志文章
doi：10.1016/s0014-4835(05)80036-3
更新日期：1995-08-01 00:00:00
abstract：:Keratoconus is a debilitating corneal thinning disease that principally develops in the second and third decades of life. Our group previously developed a novel approach to studying keratoconus, based on the observation that there is a gradient of damage across the keratoconic cone. We identified a number of cellular ...
journal_title：Experimental eye research
pub_type： 杂志文章
doi：10.1016/s0014-4835(03)00148-9
更新日期：2003-10-01 00:00:00
abstract：:Certain mutations in optineurin (gene OPTN) are associated with primary open angle glaucoma. Optineurin is ubiquitously expressed but it shows high level of expression in certain cells and tissues including retinal ganglion cells. It interacts with many proteins, often acting as an adaptor to link two or more proteins...
journal_title：Experimental eye research
pub_type： 杂志文章,评审
doi：10.1016/j.exer.2015.08.020
更新日期：2016-03-01 00:00:00
abstract：:In the present study we have investigated retinal pigment epithelium-photoreceptor cell interactions in vitro, and their contributions to photoreceptor cell survival and differentiation. Preparations enriched for intact photoreceptor cells from neonatal rat retina were grown in either serum-free medium supplemented wi...
journal_title：Experimental eye research
pub_type： 杂志文章
doi：10.1016/0014-4835(92)90020-s
更新日期：1992-05-01 00:00:00
abstract：:Rituximab is a monoclonal antibody directed against the CD20 B-cell antigen and is approved for the treatment of B-cell lymphoma. We investigated the pharmacokinetics of rituximab following intravitreal administration to assess the feasibility of treating primary intraocular lymphoma. Intravitreal injections of rituxi...
journal_title：Experimental eye research
pub_type： 杂志文章
doi：10.1016/j.exer.2005.09.018
更新日期：2006-05-01 00:00:00
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journal_title：Experimental eye research
pub_type： 杂志文章
doi：10.1016/j.exer.2006.11.008
更新日期：2007-03-01 00:00:00
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journal_title：Experimental eye research
pub_type： 杂志文章
doi：10.1006/exer.2002.2049
更新日期：2002-11-01 00:00:00
abstract：:The expression and intracellular localization of the estrogen receptor (ER) subtypes, ERalpha and ERbeta were examined in cultured human lens epithelial cells (HLE-B3). ERalpha and ERbeta mRNA expression was evaluated by coupled reverse transcription-polymerase chain reaction (RT-PCR) and Southern blot analysis. Subce...
journal_title：Experimental eye research
pub_type： 杂志文章
doi：10.1016/j.exer.2003.09.027
更新日期：2004-04-01 00:00:00
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journal_title：Experimental eye research
pub_type： 杂志文章
doi：10.1016/0014-4835(90)90088-c
更新日期：1990-11-01 00:00:00
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journal_title：Experimental eye research
pub_type： 杂志文章
doi：10.1006/exer.2000.0883
更新日期：2000-09-01 00:00:00
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journal_title：Experimental eye research
pub_type： 杂志文章
doi：10.1016/j.exer.2013.06.020
更新日期：2013-10-01 00:00:00
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journal_title：Experimental eye research
pub_type： 杂志文章
doi：10.1016/j.exer.2017.05.011
更新日期：2017-08-01 00:00:00
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journal_title：Experimental eye research
pub_type： 杂志文章
doi：10.1016/j.exer.2009.10.004
更新日期：2010-01-01 00:00:00
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journal_title：Experimental eye research
pub_type： 杂志文章
doi：10.1006/exer.1996.0062
更新日期：1996-05-01 00:00:00
abstract：:The zebrafish retina regenerates in response to acute retinal lesions, replacing damaged neurons with new neurons. In this study we test the hypothesis that chronic stress to inner retinal neurons also triggers a retinal regeneration response in the bugeye zebrafish. Mutations in the lrp2 gene in zebrafish are associa...
journal_title：Experimental eye research
pub_type： 杂志文章
doi：10.1016/j.exer.2011.06.001
更新日期：2011-10-01 00:00:00
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journal_title：Experimental eye research
pub_type： 杂志文章
doi：10.1016/j.exer.2019.107701
更新日期：2019-12-01 00:00:00
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journal_title：Experimental eye research
pub_type： 杂志文章
doi：10.1006/exer.1994.1006
更新日期：1994-02-01 00:00:00
abstract：:Thirty-one trabeculectomy specimens of patients suffering from primary open-angle glaucoma (POAG) who had received no, or only minor, medication prior to surgery were ultrastructurally and quantitatively analysed. Most of the specimens revealed thickened trabeculae, increased amounts of plaque-material deposited withi...
journal_title：Experimental eye research
pub_type： 杂志文章
doi：10.1006/exer.1993.1085
更新日期：1993-06-01 00:00:00
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journal_title：Experimental eye research
pub_type： 杂志文章
doi：10.1016/j.exer.2018.04.015
更新日期：2018-08-01 00:00:00
abstract：:This is a brief overview of multifaceted anatomical, experimental and clinical studies conducted by the author since 1955 on the optic nerve head circulation in health and disease. Conclusions, based on the accumulated information provided by these studies, are summarized. The studies on the pattern of blood supply of...
journal_title：Experimental eye research
pub_type： 杂志文章,评审
doi：10.1016/s0014-4835(05)80121-6
更新日期：1995-09-01 00:00:00
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journal_title：Experimental eye research
pub_type： 杂志文章
doi：10.1016/j.exer.2013.10.020
更新日期：2014-01-01 00:00:00
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journal_title：Experimental eye research
pub_type： 杂志文章
doi：10.1016/j.exer.2019.107869
更新日期：2020-01-01 00:00:00
abstract：:Lack of an optimal in vitro model of human corneal epithelial (HCE) cells is a major limitation in studying normal functions and gene regulations in HCE. Moreover, availability of a multi-layered HCE culture can reduce the usage of animals in the toxicity testing of consumer products. We have developed tetracycline-re...
journal_title：Experimental eye research
pub_type： 杂志文章
doi：10.1016/s0014-4835(03)00175-1
更新日期：2003-10-01 00:00:00
abstract：:The effect of acute exposure to various intensities of white light on visual behavior and retinal structure was evaluated in the T4R RHO dog, a naturally-occurring model of autosomal dominant retinitis pigmentosa due to a mutation in the Rhodopsin gene. A total of 14 dogs (ages: 4-5.5 months) were used in this study: ...
journal_title：Experimental eye research
pub_type： 杂志文章
doi：10.1016/j.exer.2016.04.006
更新日期：2016-05-01 00:00:00
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journal_title：Experimental eye research
pub_type： 杂志文章
doi：10.1016/j.exer.2019.04.025
更新日期：2019-07-01 00:00:00
abstract：:The overall importance of the peak or the mean serum concentrations as predictors of ocular drug penetration is unknown. To address this fundamental question with an agent which shows promise as adjunctive therapy in the treatment of endophthalmitis, we studied the penetration of ciprofloxacin into the aqueous and vit...
journal_title：Experimental eye research
pub_type： 杂志文章
doi：10.1006/exer.1996.0101
更新日期：1996-08-01 00:00:00
abstract：:Various concentrations of three dopamine antagonists (fluphenazine, haloperidol and sulpiride) with different affinities to the receptor subtypes were applied in order to test their effect on responses from outer (isolated PIII-component of the ERG), middle (b-wave) and inner (optic nerve response, ONR) retinal layers...
journal_title：Experimental eye research
pub_type： 杂志文章
doi：10.1016/0014-4835(91)90038-g
更新日期：1991-04-01 00:00:00
abstract：:A lens serine proteinase with trypsin-like specificity has been purified to homogeneity. This is one of two serine proteinases associated with the alpha-crystallin fraction from bovine lens. The purification was accomplished by a combination of isoelectric precipitation, activation to release the proteinase, gel-filtr...
journal_title：Experimental eye research
pub_type： 杂志文章
doi：10.1016/0014-4835(83)90135-5
更新日期：1983-12-01 00:00:00
abstract：:The corneal endothelial monolayer helps maintain corneal transparency through its barrier and ionic "pump" functions. This transparency function can become compromised, resulting in a critical loss in endothelial cell density (ECD), corneal edema, bullous keratopathy, and loss of visual acuity. Although penetrating ke...
journal_title：Experimental eye research
pub_type： 杂志文章,评审
doi：10.1016/j.exer.2011.08.014
更新日期：2012-02-01 00:00:00
abstract：:The purpose of these experiments was to develop a hypothesis to explain activation of m-calpain in cataractogenesis observed in rodents. The in vitro model used to study m-calpain activation was to correlate breakdown of the 'reporter' protein alpha-crystallin with the appearance of activated m-calpain using protein s...
journal_title：Experimental eye research
pub_type： 杂志文章
doi：10.1006/exer.1996.0234
更新日期：1997-04-01 00:00:00
