Recent advances in retinal degeneration : [Proceedings of the XII International Symposium on Retinal Degeneration (also known as RD2006) held October 23-28, 2006 in San Carlos de Bariloche, Argentina

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, generics, 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 of 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. Front Matter....Pages I-XXXIV Front Matter....Pages 1-1 Curing Blindness with Stem Cells: Hope, Reality, and Challenges....Pages 3-20 Retinal Degenerations: Planning for the Future....Pages 21-35 Front Matter....Pages 37-37 Neurotrophins Induce Neuroprotective Signaling in the Retinal Pigment Epithelial Cell by Activating the Synthesis nl of the Anti-inflammatory and Anti-apoptotic Neuroprotectin D1....Pages 39-44 On the Role of CNTF as a Pontential Therapy for Retinal Degeneration: Dr. Jekyll or Mr. Hyde?....Pages 45-51 Nanoceria Particles Prevent ROI-Induced Blindness....Pages 53-59 An in-vivo Assay to Identify Compounds Protective Against Light Induced Apoptosis....Pages 61-67 Role of BCL-XL in Photoreceptor Survival....Pages 69-74 The Hypoxic Transcriptome of the Retina: Identification of Factors with Potential Neuroprotective Activity....Pages 75-85 Front Matter....Pages 87-87 Lentiviral Gene Transfer-Mediated Cone Vision Restoration in RPE65 Knockout Mice....Pages 89-95 In vitro Analysis of Ribozyme-mediated Knockdown of an ADRP Associated Rhodopsin Mutation....Pages 97-106 Gene Therapy for Mouse Models of ADRP....Pages 107-112 Development of Viral Vectors with Optimal Transgene Expression for Ocular Gene Therapies....Pages 113-119 Adeno-Associated Viral Vectors and the Retina....Pages 121-128 Genetic Supplementation of RDS Alleviates a Loss-of-function Phenotype in C214S Model of Retinitis Pigmentosa....Pages 129-138 of the Retina in the 4 Years Following rAAV-mediated Gene Transfer in the RPE65 Null Mutation Dog....Pages 139-146 Virus-mediated Gene Delivery to Neuronal Progenitors....Pages 147-153 Front Matter....Pages 155-155 Loss of Visual and Retinal Function in Light-stressed Mice....Pages 157-164 ERG Responses and Microarray Analysis of Gene Expression in a Multifactorial Murine Model of Age-Related Retinal Degeneration....Pages 165-170 Oxygen Supply and Retinal Function: Insights from a Transgenic Animal Model....Pages 171-177 Characterization of Gene Expression Profiles of Normal Canine Retina and Brain Using a Retinal cDNA Microarray....Pages 179-184 Front Matter....Pages 155-155 Toward a Higher Fidelity Model of AMD....Pages 185-192 The Potential of Ambient Light Restriction to Restore Function to the Degenerating P23H-3 Rat Retina....Pages 193-199 Front Matter....Pages 201-201 Mutations in Known Genes Account for 58% of Autosomal Dominant Retinitis Pigmentosa (adRP)....Pages 203-209 Genetics of Age-related Macular Degeneration....Pages 211-219 Retinal Phenotype of an X-Linked Pseudo-usher Syndrome in Association with the G173R Mutation in the RPGR Gene....Pages 221-227 Mutation in the PYK2-Binding Domain of PITPNM3 Causes Autosomal Dominant Cone Dystrophy (CORD5) in Two Swedish Families....Pages 229-234 Identification and Characterization of Genes Expressed in Cone Photoreceptors....Pages 235-244 Clinical and Genetic Characterization of a Chinese Family with CSNB1....Pages 245-252 10q26 Is Associated with Increased Risk of Age-Related Macular Degeneration in the Utah Population....Pages 253-258 Front Matter....Pages 259-259 Carboxyethylpyrrole Adducts, Age-related Macular Degeneration and Neovascularization....Pages 261-267 A Possible Impaired Signaling Mechanism in Human Retinal Pigment Epithelial Cells from Patients with Macular Degeneration....Pages 269-275 Expression and Cell Compartmentalization of EFEMP1, a Protein Associated with Malattia Leventinese ....Pages 277-282 Role of ELOVL4 in Fatty Acid Metabolism....Pages 283-290 Organization and Molecular Interactions of Retinoschisin in Photoreceptors....Pages 291-297 Front Matter....Pages 299-299 Proteomics Profiling of the Cone Photoreceptor Cell Line, 661W....Pages 301-311 γ-Secretase Regulates VEGFR-1 Signalling in Vascular Endothelium and RPE....Pages 313-319 Analysis of the Rate of Disk Membrane Digestion by Cultured RPE Cells....Pages 321-326 Functional Expression of Cone Cyclic Nucleotide-Gated Channel in Cone Photoreceptor-Derived 661W Cells....Pages 327-334 Phosphorylation of Caveolin-1 in Bovine Rod Outer Segments in vitro by an Endogenous Tyrosine Kinase....Pages 335-341 Regulation of Neurotrophin Expression and Activity in the Retina....Pages 343-349 Front Matter....Pages 299-299 Involvement of Guanylate Cyclases in Transport of Photoreceptor Peripheral Membrane Proteins....Pages 351-359 Rod Progenitor Cells in the Mature Zebrafish Retina....Pages 361-368 αvβ5 Integrin Receptors at the Apical Surface of the RPE: One Receptor, Two Functions....Pages 369-375 Implantation of Mouse Eyes with a Subretinal Microphotodiode Array....Pages 377-382 Variation in the Electroretinogram of C57BL/6 Substrains of Mouse....Pages 383-391 A2E, A Pigment of RPE Lipofuscin, is Generated from the Precursor, A2PE by a Lysosomal Enzyme Activity....Pages 393-398 Endothelin Receptors: Do They Have a Role in Retinal Degeneration?....Pages 399-405 CNTF Negatively Regulates the Phototransduction Machinery in Rod Photoreceptors: Implication for Light-Induced Photostasis Plasticity....Pages 407-413 Back Matter....Pages 415-423 The product of perhaps the most important research meeting in the field, this essential text outlines all the latest research in retinal degeneration. Culled from the proceedings of the International Symposium on the subject, the topics in this volume explore the etiology, cellular mechanisms, epidemiology, models and potential therapeutic measures for the blinding diseases of retinitis pigmentosa and age-related macular degeneration. A must-read for researchers in the field. Robert E. Anderson, Matthew M. Lavail And Joe G. Hollyfield, Editors. Xii International Symposium On Retinal Degeneration (also Known As Rd2006) Held October 23-28, 2006 In San Carlos De Bariloche, Argentina--pref. Includes Bibliographical References And Index.