Rat model for hereditary retinal degeneration.

Abstract

The disorder in Wag/Rij rats is a spontaneous, bilateral retinal deneration. It is characterized by an early onset, slowly progressive degeneration of the photoreceptor cells leading to destruction of the retina. Degeneration affects both rod cells and cone cells, and to a lesser degree the cells in the inner nuclear layer. The remarkable alterations in the retinal pigment epithelium during the course of the disease suggest a profound change in metabolism and function of the pigment epithelium and implicate a possibility of interaction between the pigment epithelium and the photoreceptors. Since degenerated cells have also been observed in the inner nuclear layers, there is a possibility that Muller's cells are involved in the retinal degeneration. Controlled experiments have demonstrated that the disease is not induced by light damaging effects of the retina, and initial breeding experiments suggest that the disease is inheritable, probably as an autosomal dominant trait. The retinal degeneration in Wag/Rij rats is a new, unique system and it is a potentially very useful animal model of retinitis pigmentosa.