The Role of RPE65 in Inherited Retinal Diseases

Retina ◽  
2005 ◽  
Vol 25 (Supplement) ◽  
pp. S61-S62 ◽  
Author(s):  
DEAN BOK
Keyword(s):  
Author(s):  
Sonia Mehta ◽  
Joshua L. Dunaief
Keyword(s):  

2020 ◽  
Vol 21 (3) ◽  
pp. 1021 ◽  
Author(s):  
Carlota Suárez-Barrio ◽  
Susana del Olmo-Aguado ◽  
Eva García-Pérez ◽  
María de la Fuente ◽  
Francisco Muruzabal ◽  
...  

Oxidative stress has a strong impact on the development of retinal diseases such as age-related macular degeneration (AMD). Plasma rich in growth factors (PRGF) is a novel therapeutic approach in ophthalmological pathologies. The aim of this study was to analyze the antioxidant effect of PRGF in retinal epithelial cells (EPR) in in vitro and ex vivo retinal phototoxicity models. In vitro analyses were performed on ARPE19 human cell line. Viability and mitochondrial status were assessed in order to test the primary effects of PRGF. GSH level, and protein and gene expression of the main antioxidant pathway (Keap1, Nrf2, GCL, HO-1, and NQO1) were also studied. Ex vivo analyses were performed on rat RPE, and HO-1 and Nrf2 gene and protein expression were evaluated. The results show that PRGF reduces light insult by stimulating the cell response against oxidative damage and modulates the antioxidant pathway. We conclude that PRGF’s protective effect could prove useful as a new therapy for treating neurodegenerative disorders such as AMD.


Cells ◽  
2020 ◽  
Vol 9 (12) ◽  
pp. 2594
Author(s):  
Yue Ruan ◽  
Tobias Böhmer ◽  
Subao Jiang ◽  
Adrian Gericke

The retina is a part of the central nervous system, a thin multilayer with neuronal lamination, responsible for detecting, preprocessing, and sending visual information to the brain. Many retinal diseases are characterized by hemodynamic perturbations and neurodegeneration leading to vision loss and reduced quality of life. Since catecholamines and respective bindings sites have been characterized in the retina, we systematically reviewed the literature with regard to retinal expression, distribution and function of alpha1 (α1)-, alpha2 (α2)-, and beta (β)-adrenoceptors (ARs). Moreover, we discuss the role of the individual adrenoceptors as targets for the treatment of retinal diseases.


Cells ◽  
2020 ◽  
Vol 9 (3) ◽  
pp. 705 ◽  
Author(s):  
Emilie Picard ◽  
Alejandra Daruich ◽  
Jenny Youale ◽  
Yves Courtois ◽  
Francine Behar-Cohen

Iron is essential for cell survival and function. It is a transition metal, that could change its oxidation state from Fe2+ to Fe3+ involving an electron transfer, the key of vital functions but also organ dysfunctions. The goal of this review is to illustrate the primordial role of iron and local iron homeostasis in retinal physiology and vision, as well as the pathological consequences of iron excess in animal models of retinal degeneration and in human retinal diseases. We summarize evidence of the potential therapeutic effect of iron chelation in retinal diseases and especially the interest of transferrin, a ubiquitous endogenous iron-binding protein, having the ability to treat or delay degenerative retinal diseases.


2017 ◽  
Vol 8 ◽  
Author(s):  
Jiantong Du ◽  
Hongfang Jin ◽  
Liu Yang

2020 ◽  
Vol 201 ◽  
pp. 108255 ◽  
Author(s):  
Thomas CW. Chan ◽  
Jennifer L. Wilkinson Berka ◽  
Devy Deliyanti ◽  
Damien Hunter ◽  
Adrian Fung ◽  
...  

2016 ◽  
Vol 14 (5) ◽  
pp. 4811-4815
Author(s):  
Jiali Li ◽  
Xueshan Xiao ◽  
Shiqiang Li ◽  
Xiaoyun Jia ◽  
Xiangming Guo ◽  
...  
Keyword(s):  

2021 ◽  
Vol 22 (12) ◽  
pp. 6207
Author(s):  
Radoslaw Kaczmarek ◽  
Pawel Gajdzis ◽  
Malgorzata Gajdzis

Retinal diseases are the leading cause of irreversible blindness. They affect people of all ages, from newborns in retinopathy of prematurity, through age-independent diabetic retinopathy and complications of retinal detachment, to age-related macular degeneration (AMD), which occurs mainly in the elderly. Generally speaking, the causes of all problems are disturbances in blood supply, hypoxia, the formation of abnormal blood vessels, and fibrosis. Although the detailed mechanisms underlying them are varied, the common point is the involvement of Eph receptors and ephrins in their pathogenesis. In our study, we briefly discussed the pathophysiology of the most common retinal diseases (diabetic retinopathy, retinopathy of prematurity, proliferative vitreoretinopathy, and choroidal neovascularization) and collected available research results on the role of Eph and ephrins. We also discussed the safety aspect of the use of drugs acting on Eph and ephrin for ophthalmic indications.


Antioxidants ◽  
2021 ◽  
Vol 10 (10) ◽  
pp. 1595
Author(s):  
Larissa Ikelle ◽  
Muna I. Naash ◽  
Muayyad R. Al-Ubaidi

Retinal oxidative stress is a common secondary feature of many retinal diseases. Though it may not be the initial insult, it is a major contributor to the pathogenesis of highly prevalent retinal dystrophic diseases like macular degeneration, diabetic retinopathy, and retinitis pigmentosa. We explored the role of superoxide dismutase 3 (SOD3) in retinal homeostasis since SOD3 protects the extracellular matrix (ECM) from oxidative injury. We show that SOD3 is mainly extracellularly localized and is upregulated as a result of environmental and pathogenic stress. Ablation of SOD3 resulted in reduced functional electroretinographic responses and number of photoreceptors, which is exacerbated with age. By contrast, overexpression showed increased electroretinographic responses and increased number of photoreceptors at young ages, but appears deleterious as the animal ages, as determined from the associated functional decline. Our exploration shows that SOD3 is vital to retinal homeostasis but its levels are tightly regulated. This suggests that SOD3 augmentation to combat oxidative stress during retinal degenerative changes may only be effective in the short-term.


Author(s):  
M.A. Kovalevskaya ◽  
◽  
O.A. Pererva ◽  

Vitreomacular interface is an important part of the diagnosis from retinopathy of prematurity (ROP) to retinal diseases in adults. It is required to develop a method for macula localization to determine 3 stable points for photographic measurements of a 3D eye model. Purpose. To minimize the risk of mistakes in the diagnosis and choice of treatment for ROP and diabetic angioretinopathy (DARP) by improving the quality and analysis of modeled wide-field images on the Key to Diagnosis I and II platforms. Material and methods. 1278 RetCam and fundus images of 402 patients. 3 clinical groups: 1 – ROP 272 (544 eyes): 1A – I stage 152(304 eyes), 1B – II stage 45 (90 eyes), 1C – III stage 8 (12 eyes), 1D – posterior aggressive ROP 7 (14 eyes), 1E-retinal immaturity 60 (120 eyes); 2 – DARP 120 (240 eyes): 2A – non-proliferative 13 (17 eyes), 2B – preproliferative 47 (55 eyes), 2C – proliferative 27 (33 eyes), 2D – terminal 33 (37 eyes), 3 – control 10 (20 eyes). A standard clinical examination, analysis of modeled wide-field images, localization of the macula and traction index of the macular region (Tm) were performed. Results. Platform modules have been developed for automatic wide-field images modeling and macular localization, which is a reference point for assessing the extent of pathological changes. In ROP there was significant negative correlation of the Tm value with a stage: 1А – 0,8±0,03, 1B – 0,75±0,04, 1C – 0,74±0,13, 1D – 0,99±0,01, 1E – 0,91±0,09. For groups 2A – 2B, there was no correlation between Tm and the DARP severity. Conclusion. Tm reflects the severity of traction processes at the periphery of the retina and can be used as a marker in case of diagnostic defects in ROP. The localization of the macula determines the stable points for automatic morphometry. Key words: retinopathy of prematurity, pediatric retinal camera RetCam-Shuttle, telemedicine, diabetic angioretinopathy, vitreomacular interface.


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