scholarly journals HEREDITARY MACULAR DYSTROPHIES. PART 1. DYSTROPHIES ASSOCIATED WITH DYSFUNCTION OF RETINAL PIGMENT EPITHELIAL CELLS

2018 ◽  
Vol 13 (2) ◽  
pp. 103-108
Author(s):  
L. A Katargina ◽  
E. V Denisova ◽  
Natal’ja A. Osipova

Purpose - to acquaint the reader with a relatively rare heterogeneous group of diseases - hereditary macular dystrophies associated with damage to cells of retinal pigment epithelium and external segments of photoreceptors. А review of the literature based on publications from the Medline scientific medical articles database is presented. The review includes a description of the clinical picture, consideration of diagnosis and differential diagnosis of the main juvenile macular dystrophies, illustrated by own clinical examples.

2019 ◽  
Vol 9 (1) ◽  
Author(s):  
Laura Hellinen ◽  
Marja Hagström ◽  
Heidi Knuutila ◽  
Marika Ruponen ◽  
Arto Urtti ◽  
...  

Abstract Melanin pigment has a significant role in ocular pharmacokinetics, because many drugs bind at high extent to melanin in the retinal pigment epithelial cells. Most retinal pigment epithelial cell lines lack pigmentation and, therefore, we re-pigmented human ARPE-19 cells to generate a pigmented cell model. Melanosomes from porcine retinal pigment epithelium were isolated and co-incubated with ARPE-19 cells that spontaneously phagocytosed the melanosomes. Internalized melanosomes were functionally integrated to the cellular system as evidenced by correct translocation of cellular Rab27a protein to the melanosomal membranes. The pigmentation was retained during cell cultivation and the level of pigmentation can be controlled by altering the amount of administered melanosomes. We used these cells to study melanosomal uptake of six drugs. The uptake was negligible with low melanin-binders (methotrexate, diclofenac) whereas most of the high melanin-binders (propranolol, chloroquine) were extensively taken up by the melanosomes. This cell line can be used to model pigmentation of the retinal pigment epithelium, while maintaining the beneficial cell line characteristics, such as fast generation of cultures, low cost, long-term maintenance and good reproducibility. The model enables studies at normal and decreased levels of pigmentation to model different retinal conditions.


Acta Naturae ◽  
2018 ◽  
Vol 10 (3) ◽  
pp. 30-39 ◽  
Author(s):  
A. E. Kharitonov ◽  
A. V. Surdina ◽  
O. S. Lebedeva ◽  
A. N. Bogomazova ◽  
M. A. Lagarkova

The retinal pigment epithelium is a monolayer of pigmented, hexagonal cells connected by tight junctions. These cells compose part of the outer blood-retina barrier, protect the eye from excessive light, have important secretory functions, and support the function of photoreceptors, ensuring the coordination of a variety of regulatory mechanisms. It is the degeneration of the pigment epithelium that is the root cause of many retinal degenerative diseases. The search for reliable cell sources for the transplantation of retinal pigment epithelium is of extreme urgency. Pluripotent stem cells (embryonic stem or induced pluripotent) can be differentiated with high efficiency into the pigment epithelium of the retina, which opens up possibilities for cellular therapy in macular degeneration and can slow down the development of pathology and, perhaps, restore a patient's vision. Pioneering clinical trials on transplantation of retinal pigment epithelial cells differentiated from pluripotent stem cells in the United States and Japan confirmed the need for developing and optimizing such approaches to cell therapy. For effective use, pigment epithelial cells differentiated from pluripotent stem cells should have a set of functional properties characteristic of such cells in vivo. This review summarizes the current state of preclinical and clinical studies in the field of retinal pigment epithelial transplantation therapy. We also discuss different differentiation protocols based on data in the literature and our own data, and the problems holding back the widespread therapeutic application of retinal pigment epithelium differentiated from pluripotent stem cells.


Metallomics ◽  
2018 ◽  
Vol 10 (1) ◽  
pp. 83-92 ◽  
Author(s):  
Raquel González de Vega ◽  
Montserrat García ◽  
María Luisa Fernández-Sánchez ◽  
Héctor González-Iglesias ◽  
Alfredo Sanz-Medel

Selenium supplementation protects retinal pigment epithelial cells from glucose-induced stress by preserving glutathione peroxidase activity.


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