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Review
. 2014:801:157-64.
doi: 10.1007/978-1-4614-3209-8_20.

Modeling retinal dystrophies using patient-derived induced pluripotent stem cells

Karl J Wahlin  1 , Julien Maruotti, Donald J Zack
Affiliations
Review

Modeling retinal dystrophies using patient-derived induced pluripotent stem cells

Karl J Wahlin et al. Adv Exp Med Biol. 2014.

Abstract

Retinal degenerative disease involving photoreceptor (PR) cell loss results in permanent vision loss and often blindness. Generation of induced pluripotent stem cell (iPSC)-derived retinal cells and tissues from individuals with retinal dystrophies is a relatively new and promising method for studying retinal degeneration mechanisms in vitro. Recent advancements in strategies to differentiate human iPSCs (hiPSCs) into 3D retinal eyecups with a strong resemblance to the mature retina raise the possibility that this system could offer a reliable model for translational drug studies. However, despite the potential benefits, there are challenges that remain to be overcome before stem-cell-derived retinal eyecups can be routinely used to model human retinal diseases. This chapter will discuss both the potential of these 3D eyecup approaches and the nature of some of the challenges that remain.

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Figures

Fig. 20.1
Fig. 20.1
A strategy to utilize human-induced pluripotent stem cell (hiPSC)-derived PR or retinal pigment epithelial (RPE) cells for cell replacement therapies or to generate retinal eyecups to be used to model human eye disease and for high-content drug screening
Fig. 20.2
Fig. 20.2
A representative RPE monolayer (a) and pseudo-stratified optic cup-like structure (b) from human iPS cells. Arrows indicate retinal eyecup structures in 18-day-old human stem-cell-derived retinas
See this image and copyright information in PMC

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