Abstract
The human retina is a highly structured and complex neurosensory tissue central to perceiving and processing visual signals. In a healthy individual, the close interplay between the neuronal retina, the adjacent retinal pigment epithelium and the underlying blood supply, the choriocapillaris, is critical for maintaining eyesight over a lifetime. An impairment of this delicate and metabolically highly active system, caused by genetic alteration, environmental impact or both, results in a multitude of pathological phenotypes of the retina. Understanding and treating these disease processes are motivated by a marked medical need in young as well as in older patients. While naturally occurring or gene-manipulated animal models have been used successfully in ophthalmological research for many years, recent advances in induced pluripotent stem cell technology have opened up new avenues to generate patient-derived retinal model systems. Here, we explore to what extent these cellular models can be useful to mirror human pathologies in vitro ultimately allowing to analyze disease mechanisms and testing treatment options in the cell type of interest on an individual patient-specific genetic background.
Highlights
We focus on induced pluripotent stem cell (iPSC)-retinal pigment epithelium (RPE) cells and their value in ophthalmic research and the challenges one encounters when modeling monogenic and complex retinal diseases
A major advantage offered by this technology is the possibility to establish cell lines from human patients affected by monogenic and by complex diseases with the option to enhance phenotypic expression of genetic variants by exposing the cell cultures to environmental stressors of choice
A further benefit of iPSC technology lies in the potential to differentiate the pluripotent cells in almost all cell types of interest, implicitly addressing cell type-specific effects which otherwise may remain undetected
Summary
ARPE-19 cells are widely used in ophthalmic research, while only defined culturing conditions ensure that the lines are not divergent from the original mother cell line in features characteristic for RPE cells in vivo, such as pigmentation, polarity and the ability to phagocytose POS [7]. Compounding this problem is the widespread use of ARPE-19 cells in an undifferentiated and possibly unpolarized state to attempt to model RPE functions.
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