concanavalin-a and Retinitis-Pigmentosa

These studies have shown that these are some alterations in the complex carbohydrates associated with the cell surface of RPE cells from donors with dominant retinitis pigmentosa. Differences are found both in proteoglycans and in glycoproteins. Proteoglycans of different sizes and glycosaminoglycan composition are associated with distinct cellular compartments of normal RPE cells. In the case of dominant retinitis pigmentosa studied here, there are some alterations in RPE proteoglycans. Within a given cellular compartment, there may be differences in the size distribution and/or the ratios of the proteoglycan size classes. In addition, there may be altered glycosaminoglycan content found between peaks of comparable size. There also appears to be more microheterogenity in oligosaccharides derived from surface glycoproteins of retinitis pigmentosa RPE cells. This may be due to differences in the total number of carbohydrate units or in the organization of the branching structure as suggested by altered size distribution and lectin-reactivity, respectively. While the reason for these differences is not clear, they could be due to abnormal processing and/or increased degradation. Considering their importance, alterations in cell surface glycoconjugates could affect the ability of RPE cells to interact with their environment and to maintain a healthy status.

Topics: Adult; Cells, Cultured; Concanavalin A; Glycoproteins; Humans; Middle Aged; Oligosaccharides; Pigment Epithelium of Eye; Proteoglycans; Retinitis Pigmentosa

The lymphocyte subpopulations in the peripheral blood of 37 patients with retinitis pigmentosa (RP) and 24 controls were analyzed with the Leu series of monoclonal antibodies in conjunction with fluorescence-activated cell-sorter analysis. The peripheral blood lymphocytes (PBLs) from all RP genetic types had a significantly reduced frequency of Leu-4-positive T lymphocytes than controls, and a small but significant reduction in the frequency of Leu-3a-positive T lymphocytes was seen in patients with RP with the dominant trait. The reduced T-cell population seemed to be associated with an increased frequency of Leu-11a-positive cells. The PBLs from patients with RP did not react to retinal S-antigen, as assessed by the lymphocyte transformation or interleukin-2 assays. We conclude that patients with RP, although not clinically immunologically compromised, have a significantly reduced frequency of T lymphocytes in their PBLs. Furthermore, our study did not demonstrate reactivity to retinal tissue in PBLs from patients with RP.

Topics: Adolescent; Adult; Aged; Antibodies, Monoclonal; Antigens; Arrestin; Concanavalin A; Female; Humans; Interleukin-2; Lymphocyte Activation; Lymphocytes; Male; Middle Aged; Retinitis Pigmentosa

Topics: Animals; Cell Membrane; Concanavalin A; Disease Models, Animal; Ferritins; Methylmannosides; Photoreceptor Cells; Pigment Epithelium of Eye; Protein Binding; Rats; Rats, Inbred Strains; Retinal Degeneration; Retinitis Pigmentosa

Topics: Biological Transport; Carbohydrate Metabolism, Inborn Errors; Cells, Cultured; Chromatography, Affinity; Chromatography, DEAE-Cellulose; Concanavalin A; Drug Stability; Electrophoresis, Polyacrylamide Gel; Fibroblasts; Genetic Variation; Glycosaminoglycans; Hexosaminidases; Humans; Intellectual Disability; Lipidoses; Lysosomes; Mucopolysaccharidoses; Oxidation-Reduction; Periodic Acid; Retinitis Pigmentosa; Skin