Blue light, but not green or red light, inhibited growth of retinal pigment epithelial (RPE) cells, aortic endothelial cells, and fibroblasts in vitro. Significant inhibition was observed in all 3 cell types exposed for 18 hr to blue light (425-500 nm) at 42 J/cm2. Damage was prevented by inclusion of superoxide dismutase (SOD) and catalase, providing evidence for a photooxidative mechanism. Dopa (100 microM) also caused oxidative damage that suppressed growth of all 3 cell types. A synergism of dopa and light effects was observed in endothelial cells and fibroblasts, but the agents caused additive effects on RPE cells. Endothelial cells were the most sensitive to dopa, light, and the two combined. Fibroblasts were the only cell type that exhibited greater sensitivity to light than to dopa. These data suggest that oxygen-mediated damage to the growing blood vessels in the retina of a premature infant may be exacerbated by exposure to blue light. A further implication is that restriction of RPE melanogenesis to the prenatal period of darkness and lower oxygen protects the retina from simultaneous oxidative challenge by light and by reactive species generated during oxidation of dopa released to the extracellular environment.

We have screened for possible disease-causing mutations in the peripherin/retinal degeneration slow (RDS) gene in 13 Japanese families with autosomal dominant retinitis pigmentosa (ADRP). Using polymerase chain reaction-single strand conformation polymorphism analysis, a novel mutation at codon 214 was found in which the highly conserved cysteine was replaced with a serine in one family. The mutation at codon 214 was found in all three affected siblings of this family, but none of the 40 normal control individuals had this mutation. These results strongly suggest that the mutation is pathogenic for RP in this family. The clinical phenotype for this family is a late-onset form of ADRP.

No abstract

Purpose: High-contrast figures such as Landolt rings are insufficient to evaluate the function of the foveal cones of retinitis pigmentosa (RP) patients. We investigated the correlation between visual function as determined with Landolt rings and with the Vistech Contrast Sensitivity Function Test (VCTS) at various spatial frequencies, in addition to the Cambridge Low Contrast Grating (CLCG). Methods: The study included 30 retinitis pigmentosa patients (53 eyes). All patients were assessed with Landolt rings, the Vistech method, and the CLCG. We estimated the relative contribution of contrast sensitivity to visual acuity by VCTS at each spatial frequency and by CLCG by simple linear regression analysis. Results: The results of the regression analysis of VCTS at 1.5, 3.0, and 6.0 cycles/degree showed a significant correlation between Landolt rings and VCTS and between CLCG and VCTS that was strongest at 6.0 cycles/degree. There was no significant correlation between Landolt rings and VCTS or between CLCG and VCTS at 12.0 and 18.0 cycles/degree. Patients with a visual acuity of 20/25 and CLCG greater than 100 were divided into two groups according to their contrast sensitivity at 18.0 cycles/degree on VCTS. Conclusions: The VCTS at the highest frequency was useful for evaluating the foveal visual function in RP patients having good visual acuity with the Landolt rings. Thus, contrast sensitivity should be useful in detecting minute impairment or improvement of visual function in RP.