Retinal Degenerative Diseases / Edition 1

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Overview

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.

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Table of Contents

Molecular Genetics and Candidate Genes.- Genetic Factors Modifying Clinical Expression of Autosomal Dominant RP.- Disease-Associated Variants of the Rod-derived Cone Viability Factor (RdCVF) in Leber Congenital Amaurosis.- Leber Congenital Amaurosis: Survey of the Genetic Heterogeneity, Refinement of the Clinical Definition and Phenotype-Genotype Correlations as a Strategy for Molecular Diagnosis.- A First Locus for Isolated Autosomal Recessive Optic Atrophy (ROA1) Maps to Chromosome 8q21-q22.- RCC1-Like Domain and ORF15: Essentials in RPGR Gene.- Choroidal Neovascularization in Patients with Adult-Onset Foveomacular Dystrophy Caused by Mutations in the RDS/Peripherin Gene.- Biochemical Characterisation of the C1QTNF5 Gene Associated with Late-Onset Retinal Degeneration.- Bietti Crystalline Corneoretinal Dystrophy Associated with CYP4V2 Gene Mutations.- Diagnostic, Clinical, Cytopathological and Physiologic Aspects of Retinal Degeneration.- Fundus Appearance of Choroideremia Using Optical Coherence Tomograpy.- A2E, A Fluorophore of RPE Lipofuscin, Can Destabilize Membrane.- Amino-Retinoid Compounds in the Human Retinal Pigment Epithelium.- Annexins in Bruch’s Memberane and Drusen.- Animal Models of Retinal Degeneration.- Molecular Mechanisms of Photoreceptor Degeneration in RP Caused by IMPDH1 Mutations.- Biochemical Function of the LCA Linked Protien, Aryl Hydrocarbon Receptor Interacting Protein Like-1 (AIPL1).- Characterization of Mouse Mutants with Abnormal RPE Cells.- Rod and Cone Pigment Regeneration in RPE65 -/- Mice.- Initial Observations of Key Features of Age-Related Macular Degeneration in APOE Targeted Replacement Mice.- Altered Rhythm of Photoreceptor Outer Segment Phagocytosis in—5 Integrin Knockout Mice.- Light/Dark Translocation of Alphatransducin in Mouse Photoreceptor Cells Expressing G90D Mutant Opsin.- Slowed Photoresponse Recovery and Age-Related Degeneration in Cones Lacking Gprotein-Coupled Receptor Kinase 1.- Transgenic Animal Studies of Human Retinal Disease Caused by Mutations in Peripherin/RDS.- Transgenic Expression of Leukemia Inhibitory Factor Inhibits Both Rod and Cone Gene Expression.- A Role for bHLH Transcription Factors in Retinal Degeneration and Dysfunction.- Characterisation of a Model for Retinal Neovascularisation.- A Two-Alternative, Forced Choice Method for Assessing Mouse Vision.- Conditional Gene Knockout System in Cone Photoreceptors.- Regulation of Tight Junction Proteins in Cultured Retinal Pigment Epithelial Cells and in VEGF Overexpressing Transgenic Mouse Retinas.- Pathological Heterogeneity of Vasoproliferative Retinopathy in Transgenic Mice Overexpressing Vascular Endothelial Growth Factor in Photoreceptors.- Laser Phooagulation: Ocular Research and Therapy in Diabetic Retinopathy.- Applying Transgenic Zebrafish Technology to Study the Retina.- Bmi1 Loss Delays Photoreceptor Degeneration in Rd1 Mice.- Transcriptional and Post-Transcriptional Regulation of the Rod cGMP-Phosphodiesterase—-Subunit Gene.- Gene Therapy and Neuroprotection.- Down-Regulation of Rhodopsin Gene Expression by AAV-Vectored Short Interfering RNA.- Assessing the Efficacy of Gene Therapy in Rpe65 -/- Mice Using Photoentrainment of Circadian Phythm.- Lentiviral Vectors Containing a Retinal Pigment Epithelium Specific Promoter for Leber Congenital Amaurosis Gene Therapy.- Gene Delivery to the Retina Using Lentiviral Vectors.- Potential Use of Cellular Promoter(s) to Target RPE in AAV-Mediated Delivery.- Cytokine-Induced Retinal Degeneration: Role of Suppressors of Cytokine Signaling (SOCS) Proteins in Protection of the Neuroretina.- Disease Mechanisms and Gene Therapy in A Mouse Model for X-Linked Retinoschisis.- Molecular Mechanisms of Neuroprotection in the Eye.- Retinal Damage Caused by Photodynamic Therapy Can Be Reduced Using BDNF.- Controlling Vascular Endothelial Growth Factor: Therapies for Ocular Diseases Associated with Nevascularization.- Intravitreal Injection of Triamcinolone Acetonide for Macular Edema Due to Retinitis Pigmentosa and Other Retinal Diseases.- Cone Survival: Identification of RdCVF.- Neuroprotection of Photoreceptors in the RCS Rat After Implantation of a Subretinal Implant in the Superior or Inferior Retina.- Glutamate Transport Modulation: A Possible Role in Retinal Neuroprotection.- Activation of Cell Survival Signals in the Goldfish Retinal Ganglion Cells after Optic Nerve Injury.- Usher Syndrome.- Roles and Interactions of Usher 1 Proteins in the Outer Retina.- Molecular Analysis of the Supramolecular Usher Protein Complex in the Retina.- Stem Cells, Transplantation and Retinal Repair.- Limited Neural Differentiation of Retinal Pigment Epithelium.- Retinal Pigment Epithelial Cells from Thermally Responsive Polymer-Grafted Surface Reduce Apoptosis.- Retinal Transpilantation.- Microarray Analysis Reveals Retinal Stem Cell Characteristics of the Adult Human Eye.- Using Stem Cells to Repair the Degenerate Retina.- Opic Nerve Regeneration: Molecular Pre-Requisites and the Role of Training.- Retinal Ganglion Cell Remodelling in Experimental Glaucoma.- Induced Retinal Degenerations.- Neural Plasticity Revealed by Light-Induced Photoreceptor Lesions.- Factors Underlying Circadian Dependent Susceptibility to Light Induced Retinal Damage.- Space Flight Environment Induces Degeneration in the Retina of Rat Neonates.- Toxicity of Hyperoxia to the Retina: Evidence from the Mouse.- Treatment with Carbonic Anhydrase Inhibitors Depresses Electroretinogram Responsiveness in Mice.- Injury-Induced Retinal Ganglion Cell Loss in the Neonatal Rat Retina.- Basic Science Underlying Retinal Degeneration.- Arrestin Translocation in Rod Photoreceptors.- Binding of N-Retinylidene-Pe to BACA4 and a Model for its Transport Across Membranes.- The Chaperone Function of the LCA Protein AIPL1.- CRALBP Ligand and Protein Interactions.- Functional Study of Photoreceptor PDE?.- Localization of the Insulin Receptor and Phosphoinositide 3-Kinase in Detergent-Resistant Membrane Rafts of Rod Photoreceptor Outer Segments.- Mertk Activation During RPE Phagocytosis in Vivo Requires—Vβ5 Integrin.- Photoreceptor Retinol Dehydrogenases.- Pigment Epithelium-Derived Growth Factor Inhibits Fetal Bovine Serum Stimulated Vascular Endothelial Growth Factor Synthesis in Cultured Human Retinal Pigment Epithelial Cells.- The Retinal Pigment Epithelium Apical Microvilli and Retinal Function.- Upregulation of Transglutaminase in the Goldfish Retina During Optic Nerve Regeneration.- Survival Signaling in Retinal Pigment Epithelial Cells in Response to Oxidative Stress: Significance in Retinal Degenerations.

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