Development and degeneration of retina in rds mutant mice: ultraimmunohistochemical localization of opsin.

In normal retina the developing photoreceptor cells first show presence of opsin over the distal ends of the ciliary protrusions. In a fully differentiated cell intense activity is seen over the rod outer-segment discs; some activity is also seen over the Golgi zone and near the distal ends of the inner segments but the other parts of the receptor cell appear negative. In the pigment epithelium opsin is seen only over phagosomes containing rod outer segment debris. In the homozygous rds mutant retina, developing receptor cells show opsin activity over the ciliary protrusions as in the normal. These ciliary protrusions grow in size and show increased opsin activity and presumably constitute the site of phototransduction in the mutant retina. Although typical disc structures remain lacking, variable amounts of immunopositive, irregular, membranous structures are occasionally observed. The inner segments in the mutant cells show very little immunoreactivity but the perikarya and the spherule terminals show increased immunoreactivity in comparison with the normal. At the onset of degeneration, some of the receptor cells in the mutant retina show extrusion of small, membrane-bound vesicles which are immunopositive for opsin. Some receptor cells undergoing lysis disintegrate and also add to the opsin-positive vesicular structures in the interphotoreceptor space. The vesicles are phagocytized by pigment epithelial cells. In older mutant mice at an advanced stage of degeneration, the receptor cells show reduced opsin activity. In heterozygous mutant mice the outer segments are reduced in length and the discs are abnormal in form. However, the intensity and the pattern of opsin localization in the outer segments and at other sites are similar to normal.