Pathogenesis of progressive rod-cone degeneration in miniature poodles

Abstract

Visual cell pathologic changes and outer segment renewal were investigated in miniature poodles with progressive rod-cone degeneration. Early in this disease, visual cells in the posterior pole and equatorial regions show outer segment lamellar disorientation and vesicular profiles. Visual cells are normal in the periphery. Outer segment renewal determined after intravitreal injection of 3H-leucine was abnormally slower in affected animals than in controls. This renewal abnormality was similar in structurally normal and diseased photoreceptors, suggesting that the renewal defect is the earliest recognizable abnormality in the disease. The pigment epithelium was normal; the presence and density of pigment did not appear to affect the extent and severity of the disease or modify the abnormal renewal rate. As the disease progressed, photoreceptor outer segments were lost, and the remaining diminutive photoreceptors accumulated label in the inner segment and perinuclear zones. Sodium dodecyl sulfate gel electrophoresis of crude rod outer segment preparations showed no differences in opsin synthesis between normal and affected retinas early in the disease, but opsin synthesis decreased in the late stage of the disease.