Degenerative Diseases of the Retina, Softcover reprint of the original 1st ed. 1995

In 1984, we organized a two-day symposium on retinal degenerations as part of the biennial meeting of the VI International Society for Eye Research, held in Alicante, Spain. The success of this first meeting led to the second held, two years later in Sendai, Japan, organized as a satellite of the VII ISER. We were fortunate that these meetings began at a time of vigorous research activity in the area of retinal degenerations, thanks to the financial support of the Retinitis Pigmentosa Foundation and the strong encouragement of its scientific director, Dr. Alan Laties. Significant advances were made so that every two years scientists were eager to meet to share their findings. The programs included presentations by both basic and clinical researchers with ample time for informal discussions in a relaxed atmos­ phere. Many investigators met for the first time at these symposia and a number of fruitful collaborations were established. This book contains the proceedings of the VI International Symposium on Retinal Degenerations held November 6-10, 1994, in Jerusalem. As with the other meetings, some new areas were covered. One session was devoted to apoptosis, an important process involved in cell death in inherited retinal degenerations. Another session was on invertebrate photoreceptors, where numerous mutations have now been identified that lead to altered function or degeneration of the retina. All participants were invited to submit chapters and most complied. We thank them for their contributions.

I. Apoptosis.- 1. Apoptosis in Retinitis Pigmentosa.- 2. Oxidative Damage and Responses in Retinal Nuclei Arising from Intense Light Exposure.- 3. Light-Induced Apoptosis in the Rat Retina in Vivo: Morphological Features, Threshold and Time Course.- 4. Inhibitory Effects of Cycloheximide and Flunarizine on Light-Induced Apoptosis of Photoreceptor Cells.- 5. Apoptosis of Photoreceptors and Lens Fiber Cells with Cataract and Multiple Tumor Formation in the Eyes of Transgenic Mice Lacking the P53 Gene and Expressing the HPV 16 E7 Gene under the Control of the Irbp Promoter.- II. Role of Neurotrophic Factors in Retinal Degenerations.- 6. Function, Age-Related Expression and Molecular Characterization of PEDF, a Neurotrophic Serpin Secreted by Human RPE Cells.- 7. Nitric Oxide in the Retina: Potential Involvement in Retinal Degeneration and Its Control by Growth Factors and Cytokines.- 8. In Vitro Expression of Epidermal Growth Factor Receptor by Human Retinal Pigment Epithelial Cells.- III. Vertebrate Models of Retinal Degenerations.- 9. New Retinal Degenerations in the Mouse.- 10. Chorioretinal Interface: Age-Related Changes in Rodent Retina.- 11. Fractionation of Interphotoreceptor Matrix Metalloproteinases.- 12. Peptides from Rhodopsin Induce Experimental Autoimmune Uveoretinitis in Lewis Rats.- 13. Retinal Degeneration in Rats Induced by Vitamin E Deficiency.- 14. Retinal Pigment Epithelial Cells Cultured from RCS Rats Express an Increased Membrane Conductance for Calcium Compared to Normal Rats.- 15. Effect of Sugars on Photoreceptor Outer Segment Assembly.- 16. Regulatory Influences on the Glycosylation of Rhodopsin by Human and Bovine Retinas.- 17. The Dolichol Pathway in the Retinal Pigment Epithelium of the Embryonic Chick.- 18. Morphological and Biochemical Studies of the Retinal Degeneration in the Vitiligo Mouse: A Model with Perturbed Retinoid Metabolism.- 19. Immunological Aspects of Retinal Transplantation in Retinal Degeneration Rodents.- 20. Iron and Hereditary Degeneration of the Retina.- 21. Receptor Degeneration Is a Normal Part of Retinal Development.- 22. Development of Opsin and Synapses in Monkey Photoreceptors.- 23. The Nature of Newly Formed Capillaries in Experimental Naphthalene-Induced Retinal Degeneration in Rabbit.- 24. Retinal Cell Responses to Argon Laser Photocoagulation.- IV. Invertebrate Models of Retinal Degenerations.- 25. Drosophila as a Model for Photoreceptor Dystrophies and Cell Death.- 26. Abnormal Ca2+ Mobilization and Excessive Photopigment Phosphorylation Lead to Photoreceptor Degeneration in Drosophila Mutants.- 27. The Role of Dominant Rhodopsin Mutations in Drosophila Retinal Degeneration.- 28. The Role of the Retinal Degeneration B Protein in the Drosophila Visual System: Function of Drosophila rdgB Protein in Photoreceptors.- 29. Drosophila Visual Transduction, a Model System for Human EYE Disease? Retinal Degenerations.- 30. Characterization of Vertebrate Homologs of Drosophila Photoreceptor Proteins.- 31. Retinal Pathology in Retinitis Pigmentosa: Considerations for Therapy.- V. Molecular, Cellular and Clinical Studies.- 32. Genotype-Phenotype Correlation in Autosomal Dominant Retinal Degeneration with Mutations in the Peripherin/RDS Gene.- 33. Genetic Studies in Autosomal Recessive Forms of Retinitis Pigmentosa.- 34. Clinical Features of Autosomal Dominant Retinitis Pigmentosa Associated with the Ser 186TRP Mutation of Rhodopsin.- 35. Mutations in the Gene for the B-Subunit of Rod Photoreceptor CGMP-Specific Phosphodiesterase (PDEB) in Patients with Retinal Dystrophies and Dysfunctions.- 36. Molecular Genetic Studies of Retinal Dystrophies Principally Affecting the Macula.- 37. Molecular Analysis of the Human GAR1 Gene: A Candidate Gene for Retinal Degeneration.- 38. Guanylate Cyclase-Activating Protein (GCAP): A Novel Ca2+-Binding Protein in Vertebrate Photoreceptors.- 39. Abnormal Cone Receptor Activity in Patients with Hereditary Degeneration.- 40. Abnormal Rod Photoreceptor Function in Retinitis Pigmentosa.- 41. Scotopic Threshold Responses and Rod Intensity-Response Functions as Sensitive Indicators of the Carrier Status in X-Linked Recessive Retinitis Pigmentosa.- 42. ERG Findings in Two Patients with Autosomal Dominant Congenital Stationary Night Blindness and HIS258ASN Mutation of the ß-Subunit of Rod Photoreceptor CGMP-Specific Phosphodiesterase.- 43. Docosahexaenoic Acid Abnormalities in Red Blood Cells of Patients with Retinitis Pigmentosa.- 44. Ligand-Binding Properties of Recombinant Human IRBP.- 45. Cataractogenesis in Retinitis Pigmentosa: Spectroscopic Fluorescence Analysis of Aqueous Humor Composition.