Morphological and biochemical studies of canine progressive rod-cone degeneration. 3H-fucose autoradiography.

Visual cell pathology and rod outer segment renewal were investigated in normal and PRCD-affected miniature poodles using 3H-fucose autoradiography. Twenty-four hours following the intravitreal injection of 3H-fucose, label accumulated diffusely over cone OS and in a banded pattern at the rod OS base. In normal rods, the band of 3H-label was displaced sclerad with time. PRCD-affected rods in the early stages of the disease (stages 0-1) also showed a similar 3H-label pattern but a significantly (P less than 0.001) reduced renewal rate (control = 2.35 +/- 0.43 mu/24 hr; affected = 0.99 +/- mu/24 hr). This abnormal renewal rate was present in central, equatorial, and peripheral visual cells and was not associated with the presence or density of pigment in the RPE cell layer. Biochemical studies indicated that the 3H-label was present as an integral membrane component in the rod OS and confirmed that canine rhodopsin is a fucosylated glycoprotein. The 3H-band in the rod OS layer disappeared in stage 2 of the disease; diffuse label now was present over rod OS that had decreased length and were reduced in number. At this stage of the disease, interphotoreceptor space was invaded by phagocytic cells, and photoreceptor nuclei were lost from the outer nuclear layer. These late degenerative changes were more extensive in the superior and inferior retinal meridians.