Rhodopsin misfolding mutations lead to rod photoreceptor death that is manifested as
autosomal dominant retinitis pigmentosa (RP), a progressive blinding disease that lacks
effective treatment. We hypothesize that the cytotoxicity of the misfolded rhodopsin mutant
can be alleviated by pharmacologically stabilizing the mutant rhodopsin protein. The P23H
mutation, among the other Class II rhodopsin mutations, encodes a structurally unstable
rhodopsin mutant protein that is accumulated in the endoplasmic reticulum (ER), whereas …