Comparison of Mouse Models of Autosomal Dominant Retinitis Pigmentosa Due to the P23H Mutation of Rhodopsin.

Abstract

The need for robust and reliable animal models is a crucial step in studying any disease. This certainly applies to inherited retinal degenerative diseases, in which mutations of retinal specific genes result in photoreceptor cell death and subsequent visual loss. Animal models of retinal gene mutations have proven valuable to our understanding of disease mechanisms and as tools to evaluate therapeutic intervention strategies. Notable among these models are mice with a mutation of the rhodopsin gene at amino acid 23 in which proline is substituted for histidine (Rho-P23H). The RHO-P23H mutation is the most common cause of autosomal dominant retinitis pigmentosa. Here, we provide a brief review of the Rho-P23H mouse models currently available for research.