Retinitis pigmentosa and retinal degeneration in animals: a review.
Recent biochemical findings in the human disease, retinitis pigmentosa, and related retinal degenerative diseases in animals were reviewed and discussed. While the biochemical etiology of the human disease is not known, there are indications that retinal degeneration in the rd mouse and the Irish Setter dog are related to a deficiency in cGMP phosphodiesterase and the accumulation of cGMP in the photoreceptor outer segments. The biochemical basis of retinal degeneration in the Royal College of Surgeons (RCS) rat does not seem to be related to a defect in the metabolism of cGMP, but there are suggestions that a defect in retinoid metabolism may be involved. The possibility that the defect in RCS rats may involve receptors on the membranes of the cells of the retinal pigment epithelium or phagocytic markers on those of the rod outer segment disks was discussed.
D015106:3',5'-Cyclic-GMP Phosphodiesterases / Q000378:metabolism; D000818:Animals; D002118:Calcium / Q000378:metabolism; D004195:Disease Models, Animal / Q000473:pathology; D004285:Dogs; D005136:Eye Proteins / Q000378:metabolism; D006162:Guanylate Cyclase / Q000378:metabolism; D051379:Mice; D009712:Nucleotides, Cyclic / Q000378:metabolism; D051381:Rats; D012174:Retinitis Pigmentosa / Q000378:metabolism / Q000473:pathology*; D012243:Rhodopsin / Q000378:metabolism; D013654:Taurine / Q000378:metabolism; D014801:Vitamin A / Q000378:metabolism
D005136:Eye Proteins; D009712:Nucleotides, Cyclic; D014801:Vitamin A; D013654:Taurine; D012243:Rhodopsin; D015106:3',5'-Cyclic-GMP Phosphodiesterases; D006162:Guanylate Cyclase; D002118:Calcium