Metabolic studies on retinal tissue from a donor with a dominantly inherited chorioretinal degeneration resembling sectoral retinitis pigmentosa

Ophthalmology. 1984 Feb;91(2):191-6. doi: 10.1016/s0161-6420(84)34311-1.

Abstract

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.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Aged
  • Amino Acids / metabolism*
  • Choroid* / metabolism
  • Culture Techniques
  • Female
  • Humans
  • Leucine / metabolism
  • Mannose / metabolism
  • Photoreceptor Cells / metabolism
  • Retinal Degeneration / diagnosis
  • Retinal Degeneration / metabolism*
  • Retinitis Pigmentosa / diagnosis
  • Retinitis Pigmentosa / metabolism*
  • Trichloroacetic Acid / metabolism
  • Uridine / metabolism
  • Uveal Diseases / diagnosis
  • Uveal Diseases / metabolism*

Substances

  • Amino Acids
  • Trichloroacetic Acid
  • Leucine
  • Mannose
  • Uridine