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TitleRetinal Degenerative Diseases and Experimental Therapy [electronic resource] / edited by Joe G. Hollyfield, Robert E. Anderson, Matthew M. LaVail
ImprintBoston, MA : Springer US, 1999
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Descript XV, 587 p. 332 illus., 8 illus. in color. online resource


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


Specific Gene Defects Cause Photoreceptor Degeneration -- Evidence for Myosin VIIa-Driven Transport of Rhodopsin in the Plasma Membrane of the Photoreceptor-Connecting Cilium -- Blindness in Usher Syndrome 1B -- Different Mutations in RPE65 Are Associated with Variability in the Severity of Retinal Dystrophy -- The RPE65-Deficient Mouse as a Model for RPE65-Associated Leberโ{128}{153}s Congenital Amaurosis and Related Disorders -- CRALBP and Inherited Retinal Degeneration -- Guanylyl Cyclase Genes and Their Role in Retinal Degeneration -- Loss of Cone and Reduction in Rod ERG Responses in the Guanylyl Cyclase-E (GC-E) Deficient Mouse -- Guanylyl Cyclase Gene is the Disease Locus in the rd Chicken -- Leberโ{128}{153}s Congenital Amaurosis -- Retinal Degeneration is Accelerated when a Mutant Rhodopsin Transgene is Expressed on a Haploid or Null Rhodopsin Background -- Rhodopsin C-Terminal Sequence Qvs(A)Pa Directs Its Sorting To The Ros In Retinal Photoreceptors -- The Cytoplasmic Tail of Rhodopsin Acts as a Sorting Signal in Polarized Photoreceptors and MDCK Cells -- A Homozygous 1-Base Pair Deletion (1147dela) in the Arrestin Gene in Autosomal Recessive Retinitis Pigmentosa -- mRNA Analysis of Oguchi Patients with the Frequent 1147delA Mutation in the Arrestin Gene -- Mutation Spectrum in the Rep-1 Gene of Japanese Choroideremia Patients -- Phenotype-Genotype Correlations in Retinal Degenerations Caused by Abcr gene Mutations -- Autosomal Dominant Macular Degeneration Localized to Chromosome 6q by Linkage Analysis -- The Gene Product of IMPG1 is the Glycoprotein SPACR, not an IPM Proteoglycan -- Bardet-Biedl Syndrome -- Genetic Mapping and Evaluation of PDE6A in 49 Spanish Families with Autosomal Recessive Retinitis Pigmentosa -- Grading Device for Light Perception with Retinitis Pigmentosa -- Blue Cone Monochromacy -- The Peripherin/rds Gene -- Autosomal Dominant Retinitis Pigmentosa and Hyperopia -- Intraocular Pressure in Tapetoretinal Degenerations -- Gene Therapy for Photoreceptor Rescue -- Ribozymes Directed Against Messenger RNAs Associated With Autosomal Dominant Retinitis Pigmentosa -- Ribozyme-Mediated Gene Therapy for Autosomal Dominant Retinal Degeneration -- Targeting Mรผller Cells for Gene Therapy -- The Involvement of Light in Retinal Degeneration -- Dark Adaptation is Impaired in Diabetics before Photopic Visual Losses Can be Seen -- Age and Monocular Enucleation as Potential Determinants of Light Damage in the Mouse Retina -- Ultraviolet Light Damage and Reversal by Retinoic Acid in Juvenile Goldfish Retina -- A Comparison of Light-Induced Rod Degeneration in Two Teleost Models -- Regulation of Light Absorption in the Pigmented Rat Retina -- An Uncoupling Effect of Reactive Oxygen Species on the Retinal Horizontal Cells -- Animal Models with Photoreceptor Degeneration -- Animal Model for Retinitis Pigmentosa -- Development Of Rat Models For Choroidal Neovascularisation (CNV) -- Identification of Degeneration-Specific Genes in the Rcs Rat Retina By Subtractive Hybridization Techniques -- Analysis of Field Potentials and Spike Patterns Evoked by Local Electrical Stimulation of the Chicken Retina -- Molecular and Cell Biological Studies -- Antisense Inactivation of Rds/Peripherin in Xenopus Laevis Embryonic Retinal Cultures -- Phosducin-Like Protein 1 (Phlp1) and Phosducin-Like Orphan Protein 1 (Phlop1) -- Organization of The Interphotoreceptor Matrix -- Diverse Localization of Cyclic Nucleotide Gated Channels in the Outer Segments of Rods and Cones -- Retinal Prosthesis as a Replacement for Photoreceptors -- Development of an Epiretinal Electronic Visual Prosthesis -- Response Characteristics of Subretinal Microphotodiode-Based Implant-Mediated Cortical Potentials -- Electrical Stimulation of the Human Retina -- Goals,Concepts,and Current State of the Retina Implant Project -- Are Subretinal Microphotodiodes Suitable as a Replacement For Degenerated Photoreceptors? -- Neuroprotective Agents Romote Photoreceptor Survival -- Rods Produce a Diffusible Factor Promoting Cone Photoreceptor Survival In Vivo and in Vitro -- Pigment Epithelium-Derived Factor (PEDF) in the Retina -- Retinal Neurons in Primary Cell Culture -- Melatonin Receptor Blockers Enhance Photoreceptor Survival and Function in Light Damaged Rat Retina -- Transplantation Studies -- IRIS Pigment Epithelial Cell Transplantation in Monkey Eyes -- The Retinal Pigment Epithelial Cell Differentiation and Cell Marker Expression Following Cryopreservation at -80ยฐC and under Liquid Nitrogen at -196ยฐC

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