Hideyuki Okano and colleagues report increased expression of endoplasmic reticulum (ER) stress and apoptotic markers in an induced pluripotent stem cell (iPSC) model of retinitis pigmentosa (Mol. Brain 7, 45, 2014). The authors derived iPSCs from an affected individual carrying a mutation encoding p.Glu181Lys in the rhodopsin gene. Control iPSCs were obtained by reverting the mutation to the normal allele. Upon differentiation into rod receptors, the cells with mutant rhodopsin had decreased survival and increased expression of transcripts for the ER stress markers BiP and CHOP as well as for the apoptotic markers BID and NOXA. The results were confirmed in an independent iPSC line in which the authors replaced the normal rhodopsin gene with the allele encoding p.Glu181Lys. They further demonstrated that the effects on cell survival and expression of ER stress and apoptotic markers could be attenuated by treatment with drugs that inhibit the mTOR pathway, ASK1 or protein synthesis, or that activate AMPK.