Animal Models of Diseases of the Retinal Pigment Epithelium

Photoreceptor death accounts for approximately 50% of all cases of irreversible vision loss, contributing to both inherited retinal degenerations and age related macular degeneration. Photoreceptor integrity is maintained by a multitude of functions performed by the retinal pigment epithelium. Genetic, environmental, toxic and age changes affect various functions of the retinal pigment epithelium and in turn these changes can cause photoreceptor dysfunction and death. There are many laboratory and non-laboratory animals with mutations that affect RPE function. A great deal has been learned about diseases affecting photoreceptors from detailed analysis of structural and functional changes that occur in rodents and larger mammals that carry spontaneous mutations affecting RPE function. In addition transgenic laboratory animals have played a critical role in enhancing our understanding of RPE disease. In this chapter, we provide an overview of how genetic, environmental and ageing effects influence RPE function in laboratory and non-laboratory animals and how this in turn leads to retinal pathology.