Development of a cellular model of rod opsin retinitis pigmentosa

Abstract

A cellular model was developed to investigate potential mechanisms of cell death in rod opsin retinitis pigmentosa (RP). This model is based on transient expression of the WT and mutant P23H rod opsin in human retinal pigmented epithelial cells (RPE). Cells expressing mutant P23H opsin, unlike those expressing WT opsin, have altered opsin trafficking, increased markers for ER stress, autophagy, and apoptosis. This cellular model will provide a better understanding of the disease mechanisms associated with rhodopsin RP, while also providing a relevant model to screen potential therapeutics for treatments. © 2012 Springer Science+Business Media, LLC.