| Preface | p. vii |
| About the Editors | p. ix |
| Molecular Genetics and Candidate Genes | |
| Genetic Factors Modifying Clinical Expression of Autosomal Dominant RP | p. 3 |
| Disease-Associated Variants of the Rod-Derived Cone Viability Factor (RdCVF) in Leber Congenital Amaurosis. Rod-derived cone viability variants in LCA | p. 9 |
| Leber Congenital Amaurosis: Survey of the Genetic Heterogeneity, Refinement of the Clinical Definition and Phenotype-Genotype Correlations as a Strategy for Molecular Diagnosis. Clinical and molecular survey in LCA | p. 15 |
| A First Locus for Isolated Autosomal Recessive Optic Atrophy (ROA1) Maps to Chromosome 8q21-q22 | p. 21 |
| RCC1-Like Domain and ORF15: Essentials in RPGR Gene | p. 29 |
| Choroidal Neovascularization in Patients with Adult-onset Foveomacular Dystrophy Caused by Mutations in the RDS/Peripherin Gene | p. 35 |
| Biochemical Characterisation of the C1QTNF5 Gene Associated with Late-Onset Retinal Degeneration. A genetic model of age-related macular degeneration | p. 41 |
| Bietti Crystalline Corneoretinal Dystrophy Associated with CYP4V2 Gene Mutations | p. 49 |
| Diagnostic, Clinical, Cytopathological and Physiologic Aspects of Retinal Degeneration | |
| Fundus Appearance of Choroideremia using Optical Coherence Tomograpy | p. 57 |
| A2E, A Fluorophore of RPE Lipofuscin, Can Destabilie Membrane | p. 63 |
| Amino-Retinoid Compounds in the Human Retinal Pigment Epithelium | p. 69 |
| Annexins in Bruch's Membrane and Drusen | p. 75 |
| Animal Models of Retinal Degeneration | |
| Molecular Mechanisms of Photoreceptor Degeneration in RP Caused by IMPDH1 Mutations | p. 81 |
| Biochemical Function of the LCA Linked Protein, Aryl Hydrocarbon Receptor Interacting Protein Like-1 (AIPL1). Role of AIPL1 in retina | p. 89 |
| Characterization of Mouse Mutants with Abnormal RPE Cells | p. 95 |
| Rod and Cone Pigment Regeneration in RPE65-/- MICE | p. 101 |
| Initial Observations of Key Features of Age-Related Macular Degeneration in APOE Targeted Replacement Mice | p. 109 |
| Altered Rhythm of Photoreceptor Outer Segment Phagocytosis in ß5 Integrin Knockout Mice | p. 119 |
| Light/Dark Translocation of Alphatransducin in Mouse Photoreceptor Cells Expressing G90D Mutant Opsin | p. 125 |
| Slowed Photoresponse Recovery and Age-Related Degeneration in Cones Lacking G Protein-Coupled Receptor Kinase 1 | p. 133 |
| Transgenic Animal Studies of Human Retinal Disease Caused by Mutations in Peripherin/RDS | p. 141 |
| Transgenic Expression of Leukemia Inhibitory Factor Inhibits Both Rod and Cone Gene Expression. Gp130 regulates cone gene expression | p. 147 |
| A Role for BHLH Transcription Factors in Retinal Degeneration and Dysfunction | p. 155 |
| Characterisation of A Model for Retinal Neovascularisation. VEGF Model Characterisation | p. 163 |
| A Two-Alternative Forced Choice Method for Assessing Mouse Vision | p. 169 |
| Conditional Gene Knockout System in Cone Photoreceptors | p. 173 |
| Regulation of Tight Junction Proteins in Cultured Retinal Pigment Epithelial Cells and in VEGF Overexpressing Transgenic Mouse Retinas | p. 179 |
| Pathological Heterogeneity of Vasoproliferative Retinopathy in Transgenic Mice Overexpressing Vascular Endothelial Growth Factor in Photoreceptors | p. 187 |
| Laser Photocoagulation: Ocular Research and Therapy in Diabetic Retinopathy | p. 195 |
| Applying Transgenic Zebrafish Technology to Study the Retina | p. 201 |
| BMI1 Loss Delays Photoreceptor Degeneration in RD1 Mice. Bmil loss and neuroprotection in Rd1 mice | p. 209 |
| Transcriptional and Post-Transcriptional Regulation of the ROD cGMP-Phosphodiesterase ß-Subunit Gene. Recent advances and current concepts | p. 217 |
| Gene Therapy and Neuroprotection | |
| Down-Regulation of Rhodopsin Gene Expression by AAV-Vectored Short Interfering RNA | p. 233 |
| Assessing the Efficacy of Gene Therapy in Rpe65-/- Mice Using Photoentrainment of Circadian Rhythm | p. 239 |
| Lentiviral Vectors Containing a Retinal Pigment Epithelium Specific Promoter for Leber Congenital Amaurosis Gene Therapy. Lentiviral gene therapy for LCA | p. 247 |
| Gene Delivery to the Retina Using Lentiviral Vectors | p. 255 |
| Potential Use of Cellular Promoter(s) to Target Rpe in AAV-Mediated Delivery. Cellular promoters and RPE-targeting | p. 267 |
| Cytokine-Induce Retinal Degeneration: Role of Suppressors of Cytokine Signaling (SOCS) Proteins in Protection of the Neuroretina | p. 275 |
| Disease Mechanisms and Gene Therapy in a Mouse Model for X-Linked Retinoschisis | p. 283 |
| Molecular Mechanisms of Neuroprotection in the Eye | p. 291 |
| Retinal Damage Caused by Photodynamic Therapy can be Reduced using BDNF | p. 297 |
| Controlling Vascular Endothelial Growth Factor: Therapies for Ocular Diseases Associated with Neovascularization | p. 303 |
| Intravitreal Injection of Triamcinolone Acetonide for Macular Edema due to Retinitis Pigmentosa and other Retinal Diseases | p. 309 |
| Cone Survival: Identification of RdCVF | p. 315 |
| Neuroprotection of Photoreceptors in the RCS Rat After Implantation of a Subretinal Implant in the Superior or Inferior Retina | p. 321 |
| Glutamate Transport Modulation: A Possible Role in Retinal Neuroprotection | p. 327 |
| Activation of Cell Survival Signals in the Goldfish Retinal Ganglion Cells After Optic Nerve Injury | p. 333 |
| Usher Syndrome | |
| Roles and Interactions of Usher 1 Proteins in the Outer Retina | p. 341 |
| Molecular Analysis of the Supramolecular Usher Protein Complex in the Retina. Harmonin as the key protein of the Usher Syndrome | p. 349 |
| Stem Cells, Transplantation and Retinal Repair | |
| Limited Neural Differentiation of Retinal Pigment Epithelium | p. 357 |
| Retinal Pigment Epithelial Cells from Thermally Responsive Polymer-grafted Surface Reduce Apoptosis | p. 363 |
| Retinal Transplantation. A treatment strategy for retinal degenerative disease | p. 367 |
| Microarray Analysis Revelas Retinal Stem Cell Characteristics of the Adult Human Eye. For contribution volumes | p. 377 |
| Using Stem Cells to Repair the Degenerate Retina. Stem cells in the context of retinal degenerations | p. 381 |
| Optic Nerve Regeneration: Molecular Pre-requisites and the Role of Training. Restoring vision after optic nerve injury | p. 389 |
| Retinal Ganglion Cell Remodelling in Experimental Glaucoma | p. 397 |
| Induced Retinal Degenerations | |
| Neural Plasticity Revealed by Light-induced Photoreceptor Lesions | p. 405 |
| Factors Underlying Circadian Dependent Susceptibility to Light Induced Retinal Damage | p. 411 |
| Space Flight Environment Induces Degeneration in the Retina of Rat Neonates | p. 417 |
| Toxicity of Hyperoxia to the Retina: Evidence from the Mouse | p. 425 |
| Treatment with Carbonic Anhydrase Inhibitors Depresses Electroretinogram Responsiveness in Mice | p. 439 |
| Injury-induced Retinal Ganglion Cell Loss in the Neonatal Rat Retina | p. 447 |
| Basic Science Underlying Retinal Degeneration | |
| Arrestin Translocation in ROD Photoreceptors | p. 455 |
| Binding of N-Retinylidene-PO to ABCA4 and a Model for its Transport Across Membranes | p. 465 |
| The Chaperone Function of the LCA Protein AIPL1. AIPL1 chaperone function | p. 471 |
| Cralbp Ligand and Protein Interactions | p. 477 |
| Functional Study of Photoreceptor PDE | p. 485 |
| Localization of the Insulin Receptor and Phosphoinositide 3-Kinase in Detergent-Resistant Membrane Rafts of ROD Photoreceptor Outer Segments | p. 491 |
| Mertk Activation During RPE Phagocytosis in VIVO Requires vß5 Integrin | p. 499 |
| Photoreceptor Retinol Dehydrogenases. An attempt to characterize the function of Rdh11 | p. 505 |
| Pigment Epithelium-derived Growth Factor Inhibits Fetal Bovine Serum Stimulated Vascular Endothelial Growth Factor Synthesis in Cultured Human Retinal Pigment Epithelial Cells | p. 513 |
| The Retinal Pigment Epithelium Apical Microvilli and Retinal Function | p. 519 |
| Upregulation of Transglutaminase in the Goldfish Retina During Optic Nerve Regeneration | p. 525 |
| Survival Signaling in Retinal Pigment Epithelial Cells in Response to Oxidative Stress. Significance in retinal degenerations | p. 531 |
| Index | p. 541 |
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