Metabolic studies on retinal tissue from a donor with a dominantly inherited chorioretinal degeneration resembling sectoral retinitis pigmentosa.
Protein synthesis, glycosylation, RNA synthesis, and neurotransmitter uptake were monitored using biochemical and autoradiographic techniques following in vitro labeling of retinal tissue from a 79-year-old female with sectoral retinitis pigmentosa (RP). Comparisons were made between degenerate and non-degenerate regions of the RP retina, and normal retinal tissues from an age- and postmortem-matched donor. Autoradiographs of non-degenerate retina from the RP eye following 3H-uridine incubation revealed virtually identical silver grain density over nuclei in all retinal strata as compared to normal control retinas. In contrast, a photoreceptor-specific reduction in silver grain density in the non-degenerate RP retina was noted following 3H-leucine incubation. In the normal retina, rod photoreceptor labeling with 3H-mannose was always greater than cone photoreceptor labeling. This pattern of incorporation was reversed in the non-degenerate region of the RP retina where rod photoreceptor labeling was less pronounced than that observed for cone photoreceptors. In non-degenerate regions of the RP retina, a marked accumulation of 3H-GABA by the Müller's cells was observed. Few cells exhibited selective uptake of 3H-muscimol, a GABA analog, indicating that few GABAergic neurons remained in the degenerate retina. 3H-dopamine-accumulating cell terminals were observed in the usual positions in the non-degenerate RP retina. In the degenerate region of the RP retina, heavy and diffuse uptake of 3H-GABA and 3H-muscimol, respectively, into broad cellular processes were noted, whereas 3H-dopamine was accumulated by only a few punctate terminals.
D000368:Aged; D000596:Amino Acids / Q000378:metabolism*; D002829:Choroid / Q000378:metabolism; D046508:Culture Techniques; D005260:Female; D006801:Humans; D007930:Leucine / Q000378:metabolism; D008358:Mannose / Q000378:metabolism; D010786:Photoreceptor Cells / Q000378:metabolism; D012162:Retinal Degeneration / Q000175:diagnosis / Q000378:metabolism*; D012174:Retinitis Pigmentosa / Q000175:diagnosis / Q000378:metabolism*; D014238:Trichloroacetic Acid / Q000378:metabolism; D014529:Uridine / Q000378:metabolism; D014603:Uveal Diseases / Q000175:diagnosis / Q000378:metabolism*
D000596:Amino Acids; D014238:Trichloroacetic Acid; D007930:Leucine; D008358:Mannose; D014529:Uridine