This book contains the proceedings of the XVIII International Symposium on Retinal Degeneration (RD2018). A majority of those who spoke  and presented posters at the meeting contributed to this volume. The blinding diseases of inherited retinal degenerations have no treatments, and age-related macular degeneration has no cures, despite the fact that it is an epidemic among the elderly, with 1 in 3-4 affected by the age of 70. The RD Symposium focused on the exciting new developments aimed at understanding these diseases and providing therapies for them. Since most major scientists in the field of retinal degenerations attend the biennial RD Symposia, they are known by most as the “best� and “most important� meetings in the field. The volume presents representative state-of-the-art research in almost all areas of retinal degenerations, ranging from cytopathologic, physiologic, diagnostic and clinical aspects; animal models; mechanisms of cell death; candidate genes, cloning, mapping and other aspects of molecular genetics; and developing potential therapeutic measures such as gene therapy and neuroprotective agents for potential pharmaceutical therapy. While advances in these areas of retinal degenerations were described, there will be many new topics that either are in their infancy or did not exist at the time of the last RD Symposium. These include the role of inflammation and immunity, as well as other basic mechanisms, in age-related macular degeneration, several new aspects of gene therapy, and revolutionary new imaging and functional testing that will have a huge impact on the diagnosis and following the course of retinal degenerations, as well as to provide new quantitative endpoints for clinical trials. The retina is an approachable part of the central nervous system (CNS), and there is a major interest in neuroprotective and gene therapy for CNS diseases and neurodegenerations, in general. It should be noted that with successful and exciting initial clinical trials in neuroprotective and gene therapy, including the restoration of sight in blind children, the retinal degeneration therapies are leading the way towards new therapeutic measures for neurodegenerations of the CNS. Many of the successes recently reported in these areas of retinal degeneration sprang from collaborations established at previous RD Symposia, and many of those were reported at the RD2016 meeting and included in the current volume. We anticipate the excitement of those working in the field and those afflicted with retinal degenerations is reflected in the volume. 

The Macula, Aging and Macular Degeneration: Lipofuscin in Aged and AMD Eyes (C.D. Dorey et al.). Retinoid Reaction Products in AgeRelated Retinal Degeneration (G.E. Eldred). Retinitis Pigmentosa and Allied Retinal Degenerations: Heterogeneity of Usher Syndrome Type I (R. Ayyagari et al.). Studies Toward the Isolation of the RP3 Gene (A.F. Roux et al.). Studies of Retinal Degeneration Using Transgenic Mice and Other Animal Models: Creating Transgenic Mouse Models of Photoreceptor Degeneration Caused by Mutations in the Rhodopsin Gene (F. Wong). Systematic Alterations in Docosahexaenoic Acid Metabolism in Inherited Retinal Degenerations (N.G. Bazan et al.). Agents Which Cause or Prevent Retinal Degeneration: Growth Factors as Possible Therapeutic Agents for Retinal Degeneration (M.M. LaVail et al.). The Effect of Naphthalene on the Retina of Rabbit (N. Orzalesi et al.). 27 additional articles. Index.

TheInternationalSymposiumonRetinalDegenerationhasbeenheldinconjunction withthebiennialInternationalCongressofEyeResearch(ICER)since1984. These RDSymposiahaveallowedbasicandclinicianscientistsfromaroundtheworldto conveneandpresenttheirnewresearch?ndings. Theyhavebeenorganizedtoallow suf?cienttimefordiscussionsandone-on-oneinteractionsinarelaxedatmosphere, whereinternationalfriendshipsandcollaborationscanbefostered. The XIII International Symposium on Retinal Degeneration (also known as RD2008) was held from September 18-23, 2008 at the Hong Zhu Shan Hotel at thefootofEmeiMountainintheSichuanProvinceofChina, nearChengdu. The meeting brought together 152 basic and clinician scientists, retinal specialists in ophthalmology, andtraineesinthe?eldfromallpartsoftheworld. Inthecourse ofthemeeting,42platformand88posterpresentationsweregiven, andamajority ofthesearepresentedinthisproceedingsvolume. Newdiscoveriesandstateofthe art?ndingsfrommostresearchareasinthe?eldofretinaldegenerationswerep- sented. TheRD2008meetingwashighlightedbythreespeciallectures. The?rstwas givenby Glen Prusky, PhD, WeillCornellMedicalCollegeofCornellUniversity, New York City, NY. Dr. Prusky discussed the measures of vision in rodents as a tool for evaluating the treatment of retinal degenerative diseases. The second was given by Kang Zhang, MD, PhD, on the molecular genetics of Stargardt's Disease. Dr. Zhang's undergraduate degree in biochemistry is from West China University in Chengdu, and he currently is at the Shiley Eye Center, University ofCaliforniaatSanDiego, SanDiego, CA. Thethirdplenarylecturewasgivenby Peter Campochiaro, MD, oftheWilmerEyeInstitute, JohnsHopkinsUniversity, Baltimore, MD. Dr. Campochiaro discussed the role of oxidant stress in macular degeneration. This Symposium would not have been possible without the support of our colleagues at the Sichuan People's Provincial Hospital and the Department of Ophthalmology of West China Hospital, Sichuan University. Fan Ying Chuan, MD, ViceChairman, SichuanOphthalmologySociety, DirectorofOphthalmology Department, SichuanAcademyofMedicalScience&SichuanProvincialPeople's Hospitaland Chen Xiao Ming, MD, Chairman, SichuanOphthalmologySociety, Director of West China Eye Center, West China Hospital, Sichuan University, gave tirelessly to our effort from the beginning. We are especially grateful to the ix x Preface administration of the Sichuan People's Provincial Hospital, which provided the ?nancialguaranteesnecessarytosecurethemeetingvenue. Theassistanceof Chen Hui (Robert), MD, of the SPPH throughout the planning and the meeting itself were of enormous help to us.

The topics in this volume explore the etiology, cellular mechanisms, epidemiology, genetics, models and potential therapeutic measures for the blinding diseases of retinitis pigmentosa and age-related macular degeneration.
Special focus is highlighted in the areas of Mechanisms of Photoreceptor Degeneration and Cell Death (extremely important because very little is known how or why photoreceptors die in these diseases, despite an abundance of genetic information), Age-Related Macular Degeneration (with several novel approaches to its analysis), Usher Syndrome (the most severe form of retinitis pigmentosa, which includes an early or congenital loss of hearing along with blindness), and Gene Therapy. In addition, the section on Basic Science Related to Retinal Degeneration is particularly strong with several laboratories reporting on new discoveries in the area of outer segment phagocytosis, a key component of photoreceptor-retinal pigment epithelial cell interactions in normal and degenerating retinas.

Since 1984, we have organized satellite symposia on retinal degenerations that are held in conjunction with the biennial International Congress of Eye Research. The timing and location of our Retinal Degeneration Symposia have allowed scientists and clinicians from around the world to convene and present their exciting new findings. The symposia have been arranged to allow ample time for discussions and one-on-one interactions in a relaxed atmosphere, where international friendships and collaborations could be established. The IXth International Symposium on Retinal Degeneration was held on October 9-14, 2000 in Durango, Colorado and was attended by over 100 scientists from six continents. This book contains many of their presentations. Several events of note occurred at this meeting. First, thanks to the generous support of the Foundation Fighting Blindness, we were able to sponsor the travel of 11 young scientists from six countries. Most of them have contributed chapters to this volume. The response to the travel program was so overwhelming that we will make it regular feature of our meeting. This will allow other bright, young investigators to be introduced to the world experts who study retinal degenerations. Second, about 40% of the scientists who attended this meeting were there for the first time. We believe that this indicates a growing interest in retinal degeneration research and ensures that new talent will be attracted to this important area of investigation. The symposium received support from several organizations.

This book will contain the proceedings of the XV International Symposium on Retinal Degeneration (RD2012). A majority of those who will speak and present posters at the meeting will contribute to this volume. The blinding diseases of inherited retinal degenerations have no treatments, and age-related macular degeneration has no cures, despite the fact that it is an epidemic among the elderly, with 1 in 3-4 affected by the age of 70. The RD Symposium will focus on the exciting new developments aimed at understanding these diseases and providing therapies for them. Since most major scientists in the field of retinal degenerations attend the biennial RD Symposia, they are known by most as the "best" and "most important" meetings in the field. The volume will present representative state-of-the-art research in almost all areas of retinal degenerations, ranging from cytopathologic, physiologic, diagnostic and clinical aspects; animal models; mechanisms of cell death; candidate genes, cloning, mapping and other aspects of molecular genetics; and developing potential therapeutic measures such as gene therapy and neuroprotective agents for potential pharmaceutical therapy. While advances in these areas of retinal degenerations will be described, there will be many new topics that either were in their infancy or did not exist at the time of the last RD Symposium, RD2010. These include the role of inflammation and immunity, as well as other basic mechanisms, in age-related macular degeneration, several new aspects of gene therapy, and revolutionary new imaging and functional testing that will have a huge impact on the diagnosis and following the course of retinal degenerations, as well as to provide new quantitative endpoints for clinical trials. The retina is an approachable part of the central nervous system (CNS), and there is a major interest in neuroprotective and gene therapy for CNS diseases and neurodegenerations, in general. It should be noted that with successful and exciting initial clinical trials in neuroprotective and gene therapy, including the restoration of sight in blind children, the retinal degeneration therapies are leading the way towards new therapeutic measures for neurodegenerations of the CNS. Many of the successes recently reported in these areas of retinal degeneration sprang from collaborations established at previous RD Symposia, and many of those will be reported at the RD2010 meeting and included in the proposed volume. We anticipate the excitement of those working in the field and those afflicted with retinal degenerations will be reflected in the volume.

This book contains the proceedings of the XVIII International Symposium on Retinal Degeneration (RD2018). A majority of those who spoke and presented posters at the meeting contributed to this volume. The blinding diseases of inherited retinal degenerations have no treatments, and age-related macular degeneration has no cures, despite the fact that it is an epidemic among the elderly, with 1 in 3-4 affected by the age of 70. The RD Symposium focused on the exciting new developments aimed at understanding these diseases and providing therapies for them. Since most major scientists in the field of retinal degenerations attend the biennial RD Symposia, they are known by most as the "best" and "most important" meetings in the field. The volume presents representative state-of-the-art research in almost all areas of retinal degenerations, ranging from cytopathologic, physiologic, diagnostic and clinical aspects; animal models; mechanisms of cell death; candidate genes, cloning, mapping and other aspects of molecular genetics; and developing potential therapeutic measures such as gene therapy and neuroprotective agents for potential pharmaceutical therapy. While advances in these areas of retinal degenerations were described, there will be many new topics that either are in their infancy or did not exist at the time of the last RD Symposium, RD2016. These include the role of inflammation and immunity, as well as other basic mechanisms, in age-related macular degeneration, several new aspects of gene therapy, and revolutionary new imaging and functional testing that will have a huge impact on the diagnosis and following the course of retinal degenerations, as well as to provide new quantitative endpoints for clinical trials. The retina is an approachable part of the central nervous system (CNS), and there is a major interest in neuroprotective and gene therapy for CNS diseases and neurodegenerations, in general. It should be noted that with successful and exciting initial clinical trials in neuroprotective and gene therapy, including the restoration of sight in blind children, the retinal degeneration therapies are leading the way towards new therapeutic measures for neurodegenerations of the CNS. Many of the successes recently reported in these areas of retinal degeneration sprang from collaborations established at previous RD Symposia, and many of those were reported at the RD2016 meeting and included in the current volume. We anticipate the excitement of those working in the field and those afflicted with retinal degenerations is reflected in the volume.

The blinding diseases of inherited retinal degenerations have no treatments, and age-related macular degeneration has no cures, despite the fact that it is an epidemic among the elderly, with 1 in 3-4 affected by the age of 70. The RD Symposium will focus on the exciting new developments aimed at understanding these diseases and providing therapies for them. Since most major scientists in the field of retinal degenerations attend the biennial RD Symposia, they are known by most as the "best" and "most important" meetings in the field. The volume will present representative state-of-the-art research in almost all areas of retinal degenerations, ranging from cytopathologic, physiologic, diagnostic and clinical aspects; animal models; mechanisms of cell death; candidate genes, cloning, mapping and other aspects of molecular genetics; and developing potential therapeutic measures such as gene therapy and neuroprotective agents for potential pharmaceutical therapy. While advances in these areas of retinal degenerations will be described, there will be many new topics that either were in their infancy or did not exist at the time of the last RD Symposium, RD2014. These include the role of inflammation and immunity, as well as other basic mechanisms, in age-related macular degeneration, several new aspects of gene therapy, and revolutionary new imaging and functional testing that will have a huge impact on the diagnosis and following the course of retinal degenerations, as well as to provide new quantitative endpoints for clinical trials. The retina is an approachable part of the central nervous system (CNS), and there is a major interest in neuroprotective and gene therapy for CNS diseases and neurodegenerations, in general. It should be noted that with successful and exciting initial clinical trials in neuroprotective and gene therapy, including the restoration of sight in blind children, the retinal degeneration therapies are leading the way towards new therapeutic measures for neurodegenerations of the CNS. Many of the successes recently reported in these areas of retinal degeneration sprang from collaborations established at previous RD Symposia, and many of those will be reported at the RD2018 meeting and included in the proposed volume. We anticipate the excitement of those working in the field and those afflicted with retinal degenerations will be reflected in the volume.

Retinal Degenerations is the result of the International Symposium on Retinal degeneration which has become perhaps the most important research meeting in the field. THe topics in this volume explore the etiology, cellular mechanisms, epidemiology, genetics, models and potential therapeutic measures for the blinding diseases of retinitis pigmentosa and age-related macular degeneration.

This book will contain the proceedings of the XIV International Symposium on Retinal Degeneration (RD2010), held July 13-17, 2010, in Mont-Tremblant, Quebec, Canada. The volume will present representative state-of-the-art research in almost all areas of retinal degenerations, ranging from cytopathologic, physiologic, diagnostic and clinical aspects; animal models; mechanisms of cell death; candidate genes, cloning, mapping and other aspects of molecular genetics; and developing potential therapeutic measures such as gene therapy and neuroprotective agents for potential pharmaceutical therapy.

The blinding diseases of inherited retinal degenerations have no treatments, and age-related macular degeneration has no cures, despite the fact that it is an epidemic among the elderly, with 1 in 3-4 affected by the age of 70. The RD Symposium will focus on the exciting new developments aimed at understanding these diseases and providing therapies for them. Since most major scientists in the field of retinal degenerations attend the biennial RD Symposia, they are known by most as the "best" and "most important" meetings in the field. The volume will present representative state-of-the-art research in almost all areas of retinal degenerations, ranging from cytopathologic, physiologic, diagnostic and clinical aspects; animal models; mechanisms of cell death; candidate genes, cloning, mapping and other aspects of molecular genetics; and developing potential therapeutic measures such as gene therapy and neuroprotective agents for potential pharmaceutical therapy. While advances in these areas of retinal degenerations will be described, there will be many new topics that either were in their infancy or did not exist at the time of the last RD Symposium, RD2014. These include the role of inflammation and immunity, as well as other basic mechanisms, in age-related macular degeneration, several new aspects of gene therapy, and revolutionary new imaging and functional testing that will have a huge impact on the diagnosis and following the course of retinal degenerations, as well as to provide new quantitative endpoints for clinical trials. The retina is an approachable part of the central nervous system (CNS), and there is a major interest in neuroprotective and gene therapy for CNS diseases and neurodegenerations, in general. It should be noted that with successful and exciting initial clinical trials in neuroprotective and gene therapy, including the restoration of sight in blind children, the retinal degeneration therapies are leading the way towards new therapeutic measures for neurodegenerations of the CNS. Many of the successes recently reported in these areas of retinal degeneration sprang from collaborations established at previous RD Symposia, and many of those will be reported at the RD2018 meeting and included in the proposed volume. We anticipate the excitement of those working in the field and those afflicted with retinal degenerations will be reflected in the volume.

This book will contain the proceedings of the XIV International Symposium on Retinal Degeneration (RD2010), held July 13-17, 2010, in Mont-Tremblant, Quebec, Canada. The volume will present representative state-of-the-art research in almost all areas of retinal degenerations, ranging from cytopathologic, physiologic, diagnostic and clinical aspects; animal models; mechanisms of cell death; candidate genes, cloning, mapping and other aspects of molecular genetics; and developing potential therapeutic measures such as gene therapy and neuroprotective agents for potential pharmaceutical therapy.

This book will contain the proceedings of the XV International Symposium on Retinal Degeneration (RD2012). A majority of those who will speak and present posters at the meeting will contribute to this volume. The blinding diseases of inherited retinal degenerations have no treatments, and age-related macular degeneration has no cures, despite the fact that it is an epidemic among the elderly, with 1 in 3-4 affected by the age of 70. The RD Symposium will focus on the exciting new developments aimed at understanding these diseases and providing therapies for them. Since most major scientists in the field of retinal degenerations attend the biennial RD Symposia, they are known by most as the "best" and "most important" meetings in the field. The volume will present representative state-of-the-art research in almost all areas of retinal degenerations, ranging from cytopathologic, physiologic, diagnostic and clinical aspects; animal models; mechanisms of cell death; candidate genes, cloning, mapping and other aspects of molecular genetics; and developing potential therapeutic measures such as gene therapy and neuroprotective agents for potential pharmaceutical therapy. While advances in these areas of retinal degenerations will be described, there will be many new topics that either were in their infancy or did not exist at the time of the last RD Symposium, RD2010. These include the role of inflammation and immunity, as well as other basic mechanisms, in age-related macular degeneration, several new aspects of gene therapy, and revolutionary new imaging and functional testing that will have a huge impact on the diagnosis and following the course of retinal degenerations, as well as to provide new quantitative endpoints for clinical trials. The retina is an approachable part of the central nervous system (CNS), and there is a major interest in neuroprotective and gene therapy for CNS diseases and neurodegenerations, in general. It should be noted that with successful and exciting initial clinical trials in neuroprotective and gene therapy, including the restoration of sight in blind children, the retinal degeneration therapies are leading the way towards new therapeutic measures for neurodegenerations of the CNS. Many of the successes recently reported in these areas of retinal degeneration sprang from collaborations established at previous RD Symposia, and many of those will be reported at the RD2010 meeting and included in the proposed volume. We anticipate the excitement of those working in the field and those afflicted with retinal degenerations will be reflected in the volume.

Retinal Degenerations is the result of the International Symposium on Retinal degeneration which has become perhaps the most important research meeting in the field. THe topics in this volume explore the etiology, cellular mechanisms, epidemiology, genetics, models and potential therapeutic measures for the blinding diseases of retinitis pigmentosa and age-related macular degeneration.

The topics in this volume explore the etiology, cellular mechanisms, epidemiology, genetics, models and potential therapeutic measures for the blinding diseases of retinitis pigmentosa and age-related macular degeneration. Special focus is highlighted in the areas of Mechanisms of Photoreceptor Degeneration and Cell Death (extremely important because very little is known how or why photoreceptors die in these diseases, despite an abundance of genetic information), Age-Related Macular Degeneration (with several novel approaches to its analysis), Usher Syndrome (the most severe form of retinitis pigmentosa, which includes an early or congenital loss of hearing along with blindness), and Gene Therapy. In addition, the section on Basic Science Related to Retinal Degeneration is particularly strong with several laboratories reporting on new discoveries in the area of outer segment phagocytosis, a key component of photoreceptor-retinal pigment epithelial cell interactions in normal and degenerating retinas.

During the last few years, an explosion of infonnation has come from human genetics and molecular and cell biological studies as to the genetic basis for a number of fonns of inherited retinal degenerations. These disorders have plagued mankind for millennia because they take from otherwise healthy individuals the precious gift of sight. The fundamental advances in recent years have identified a number of genes involved in the groups of diseases which hopefully will lead to discoveries that may, in the not too distant future, allow the prevention and possible cure of some of these blinding eye disorders. To foster a forum for discussions of studies on degenerative retinal disorders, we convened a symposium on retinal degenerations in 1984, at the VIth International Congress of Eye Research Meeting, held in Alicante, Spain. Because of the success of this meeting and the subsequent publication, we have since organized a series of biennial satellite meetings on retinal degenerations for the ISER congresses held in Nagoya, Japan (1986), San Francisco (1988) and Helsinki (1990). Each of these satellite symposium on retinal degenerations was accompanied by a published proceedings volume. This volume is the fifth in this series and contains the proceedings of the Sardinia Symposium on Retinal Degeneration held September 15-20, 1992, as a satellite meeting of the 10th International Congress of Eye Research.

Since 1984, we have organized satellite symposia on retinal degenerations that are held in conjunction with the biennial International Congress of Eye Research. The timing and location of our Retinal Degeneration Symposia have allowed scientists and clinicians from around the world to convene and present their exciting new findings. The symposia have been arranged to allow ample time for discussions and one-on-one interactions in a relaxed atmosphere, where international friendships and collaborations could be established. The IXth International Symposium on Retinal Degeneration was held on October 9-14, 2000 in Durango, Colorado and was attended by over 100 scientists from six continents. This book contains many of their presentations. Several events of note occurred at this meeting. First, thanks to the generous support of the Foundation Fighting Blindness, we were able to sponsor the travel of 11 young scientists from six countries. Most of them have contributed chapters to this volume. The response to the travel program was so overwhelming that we will make it regular feature of our meeting. This will allow other bright, young investigators to be introduced to the world experts who study retinal degenerations. Second, about 40% of the scientists who attended this meeting were there for the first time. We believe that this indicates a growing interest in retinal degeneration research and ensures that new talent will be attracted to this important area of investigation. The symposium received support from several organizations.

Since 1984, we have organized Satellite Symposia on Retinal Degenerations that are held in conjunction with the biennial International Congress of Eye Research. The timing and location of our Retinal Degeneration Symposia have all owed scientists and clinicians from around the world to convene and present their exciting new findings. The symposia have been arranged to allow ample time for discussions and one-on-one interactions in a relaxed atmos phere, where international friendships and collaborations could be established. The II International Symposium (also known as the Sendai Symposium on Retinal Degeneration) was held in 1986 in Sendai, Japan, on the occasion of the retirement of Kat suyoshi Mizuno as Professor and Chairman of Ophthalmology at Tohoku Medical SchooL On October 5-9, 1996, we returned to Sendai, where the VII International Symposium was held at the Miyagi Zao resort hotel in the beautiful Mt. Zao region of northern Japan. This meeting was held on the occasion of the tenth anniversary of Makoto Tarnai as Pro fessor and Chairman of Ophthalmology at Tohoku Medical School. One afternoon of the meeting contained a special symposium in honor of Professor Tarnai, who has signifi cantly elevated the level and intensity of research on retinal degenerations at his university and in Japan during this past decade.

This book contains the proceedings of the XVIII International Symposium on Retinal Degeneration (RD2018). A majority of those who spoke and presented posters at the meeting contributed to this volume. The blinding diseases of inherited retinal degenerations have no treatments, and age-related macular degeneration has no cures, despite the fact that it is an epidemic among the elderly, with 1 in 3-4 affected by the age of 70. The RD Symposium focused on the exciting new developments aimed at understanding these diseases and providing therapies for them. Since most major scientists in the field of retinal degenerations attend the biennial RD Symposia, they are known by most as the "best" and "most important" meetings in the field. The volume presents representative state-of-the-art research in almost all areas of retinal degenerations, ranging from cytopathologic, physiologic, diagnostic and clinical aspects; animal models; mechanisms of cell death; candidate genes, cloning, mapping and other aspects of molecular genetics; and developing potential therapeutic measures such as gene therapy and neuroprotective agents for potential pharmaceutical therapy. While advances in these areas of retinal degenerations were described, there will be many new topics that either are in their infancy or did not exist at the time of the last RD Symposium, RD2016. These include the role of inflammation and immunity, as well as other basic mechanisms, in age-related macular degeneration, several new aspects of gene therapy, and revolutionary new imaging and functional testing that will have a huge impact on the diagnosis and following the course of retinal degenerations, as well as to provide new quantitative endpoints for clinical trials. The retina is an approachable part of the central nervous system (CNS), and there is a major interest in neuroprotective and gene therapy for CNS diseases and neurodegenerations, in general. It should be noted that with successful and exciting initial clinical trials in neuroprotective and gene therapy, including the restoration of sight in blind children, the retinal degeneration therapies are leading the way towards new therapeutic measures for neurodegenerations of the CNS. Many of the successes recently reported in these areas of retinal degeneration sprang from collaborations established at previous RD Symposia, and many of those were reported at the RD2016 meeting and included in the current volume. We anticipate the excitement of those working in the field and those afflicted with retinal degenerations is reflected in the volume.

Basic Science Underlying Retinal Degeneration.- Analysis of Genes Differentially Expressed During Retinal Degeneration in Three Mouse Models.- Regulation of Angiogenesis by Macrophages.- Protein Kinase C Regulates Rod Photoreceptor Differentiation Through Modulation of STAT3 Signaling.- Pigment Epithelium-derived Factor Receptor (PEDF-R): A Plasma Membrane-linked Phospholipase with PEDF Binding Affinity.- The Function of Oligomerization-Incompetent RDS in Rods.- The Association Between Telomere Length and Sensitivity to Apoptosis of HUVEC.- Photoreceptor Guanylate Cyclases and cGMP Phosphodiesterases in Zebrafish.- RDS in Cones Does Not Interact with the Beta Subunit of the Cyclic Nucleotide Gated Channel.- Increased Expression of TGF-?1 and Smad 4 on Oxygen-Induced Retinopathy in Neonatal Mice.- ZBED4, A Novel Retinal Protein Expressed in Cones and Muller Cells.- Tubby-Like Protein 1 (Tulp1) Is Required for Normal Photoreceptor Synaptic Development.- Growth-Associated Protein43 (GAP43) Is a Biochemical Marker for the Whole Period of Fish Optic Nerve Regeneration.- Multiprotein Complexes of Retinitis Pigmentosa GTPase Regulator (RPGR), a Ciliary Protein Mutated in X-Linked Retinitis Pigmentosa (XLRP).- Misfolded Proteins and Retinal Dystrophies.- Neural Retina and MerTK-Independent Apical Polarity of ?v?5 Integrin Receptors in the Retinal Pigment Epithelium.- Mertk in Daily Retinal Phagocytosis: A History in the Making.- The Interphotoreceptor Retinoid Binding (IRBP) Is Essential for Normal Retinoid Processing in Cone Photoreceptors.- Aseptic Injury to Epithelial Cells Alters Cell Surface Complement Regulation in a Tissue Specific Fashion.- Role of Metalloproteases in Retinal Degeneration Induced by Violet and Blue Light.- Mitochondrial Decay and Impairment of Antioxidant Defenses in Aging RPE Cells.- Ciliary Transport of Opsin.- Effect of Hesperidin on Expression of Inducible Nitric Oxide Synthase in Cultured Rabbit Retinal Pigment Epithelial Cells.- Profiling MicroRNAs Differentially Expressed in Rabbit Retina.- Unexpected Transcriptional Activity of the Human VMD2 Promoter in Retinal Development.- Microarray Analysis of Hyperoxia Stressed Mouse Retina: Differential Gene Expression in the Inferior and Superior Region.- Photoreceptor Sensory Cilia and Inherited Retinal Degeneration.- Role of Elovl4 Protein in the Biosynthesis of Docosahexaenoic Acid.- Molecular Genetics and Candidate Genes.- Molecular Pathogenesis of Achromatopsia Associated with Mutations in the Cone Cyclic Nucleotide-Gated Channel CNGA3 Subunit.- Mutation Spectra in Autosomal Dominant and Recessive Retinitis Pigmentosa in Northern Sweden.- 1 Rhodopsin Mutations in Congenital Night Blindness.- GCAP1 Mutations Associated with Autosomal Dominant Cone Dystrophy.- Genotypic Analysis of X-linked Retinoschisis in Western Australia.- Mutation Frequency of IMPDH1 Gene of Han Population in Ganzhou City.- Diagnostic, Clinical, Cytopathological and Physiologic Aspects of Retinal Degeneration.- Reversible and Size-Selective Opening of the Inner Blood-Retina Barrier: A Novel Therapeutic Strategy.- Spectral Domain Optical Coherence Tomography and Adaptive Optics: Imaging Photoreceptor Layer Morphology to Interpret Preclinical Phenotypes.- Pharmacological Manipulation of Rhodopsin Retinitis Pigmentosa.- Targeted High-Throughput DNA Sequencing for Gene Discovery in Retinitis Pigmentosa.- Advances in Imaging of Stargardt Disease.- Protamine Sulfate Downregulates Vascular Endothelial Growth Factor (VEGF) Expression and Inhibits VEGF and Its Receptor Binding in Vitro.- Computer-Assisted Semi-Quantitative Analysis of Mouse Choroidal Density.- Thioredoxins 1 and 2 Protect Retinal Ganglion Cells from Pharmacologically Induced Oxidative Stress, Optic Nerve Transection and Ocular Hypertension.- Near-Infrared Light Protect the Photoreceptor from Light-Induced Damage in Rats.- BDNF Improves the Efficacy ERG Amplitude Maintenance by Transplantation of Retinal Stem Cells in RCS Rats.-

To create a forum for scientists and clinicians interested in degenerative retinal diseases, we began in 1984 to organize a biennial symposium on Retinal Degeneration as a satellite meeting of the International Congress of Eye Research. The timing and varying location of these meetings provides an important assembly for investigators from throughout the world to convene for presentation of their new findings on the causes and potential therapies for degenerative retinal disorders. The VIII International Symposium on Retinal Degeneration was held from July 28-25, 1998, at the Hotel Vier Jahreszeiten in Schluchsee, a small town in the Black Forest of southwestern Germany. Most of the participants in this meeting contributed to this volume, and we are appreciative of the efforts of each author in making this publication possible. The research presented at the meeting, and described in this proceedings volume, reflects a strong emphasis on the molecular genetic approach to understa- ing these disorders. Several of the papers provide important new insights into the mechanism of photoreceptor degeneration and cell death. A number of the studies are targeted at retarding or reversing the degeneration process. Included for the first time are presentations from all the principal laboratories involved in the field of visual prostheses-implant (chip) technology-in which investigations are targeted at restoring vision in eyes that have lost photoreceptor cells. A variety of diagnostic, clinical, histopathological, and physiological assessments of retinal degeneration in patients are also included.

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."