Retinal Degenerations: Mechanisms and Experimental Therapy

Front Matter....Pages i-xvi
Identification of the RP1 and RP10 (IMPDH1) Genes Causing Autosomal Dominant RP....Pages 1-11
On the Role of IMPDH1 in Retinal Degeneration....Pages 13-18
An Integrated Genetic Approach to Identify Candidate Genes for Human Chromosome 6q-Linked Retinal Disorders....Pages 19-28
Mouse Genetic Approaches to Access Pathways Important in Retinal Function....Pages 29-34
Retinal Degenerative Disorders in Southern Africa: A Molecular Genetic Approach....Pages 35-40
Comparing Rod and Cone Function with Fundus Autofluorescence Images in Retinitis Pigmentosa....Pages 41-47
A Modified Protocol for the Assessment of Visual Function in Patients with Retinitis Pigmentosa....Pages 49-57
Prenatal Human Ocular Degeneration Occurs in Leber’s Congenital Amaurosis....Pages 59-68
Leber Congenital Amaurosis — Genotyping Required for Possible Inclusion in a Clinical Trial....Pages 69-77
Treatment of Cystoid Macular Edema Related to Retinitis Pigmentosa with Intravitreal Triamcinolone Acetonide: Case Report....Pages 79-81
Proteomic Approaches to Understanding Age-Related Macular Degeneration....Pages 83-89
Progressive Pathways in Age-Related Macular Degeneration....Pages 91-96
Tissue Inhibitor of Metalloproteinases-3 and Sorsby Fundus Dystrophy....Pages 97-105
Investigations of RPE Cells of Choriodal Neovascular Membranes from Patients with Age-Related Macula Degeneration....Pages 107-113
Retinal and Choroidal Alterations Following Photodynamic Therapy....Pages 115-122
Usher Syndrome: Correlation between Visual Field Size and Maximal ERG Response B-Wave Amplitude....Pages 123-131
The Cellular Function of the Usher Gene Product Myosin VIIa is Specified by Its Ligands....Pages 133-142
Mouse Models for Usher Syndrome 1b....Pages 143-150
Screen for Usher Syndrome 1b Mutations in the Ovine Myosin VIIa Gene....Pages 151-155
 Photoreceptor Intersegmental Transport and Retinal Degeneration ....Pages 157-164
Inherited Retinal Dystrophy in Mer Knockout Mice....Pages 165-172
Mouse Models of Human Retinal Disease Caused by Expression of Mutant Rhodopsin....Pages 173-179
Evaluation of Inner Retinal Structure in the Aged RCS Rat....Pages 181-188
The Intact Xenopus Laevis Eye Rudiment: A Quasi-In Vivo System for the Study of Retinal Development and Degenerations....Pages 189-196
Streptozotocin-Induced Diabetes — A Rat Model to Study Involvement of Retinal Cell Types in the Onset of Diabetic Retinopathy....Pages 197-203
A2E, a Fluorophore of RPE Lipofuscin: Can It Cause RPE Degeneration?....Pages 205-211
Bright Light Induces Retinal Degeneration by a Transducin- Independent Mechanism....Pages 213-219
Does Constitutive Phosphorylation Protect Against Photoreceptor Degeneration in Rpe65 -/- Mice?....Pages 221-227
Light-Induced Photoreceptor Damage Triggers DNA Repair: Differential Fate of Rods and Cones....Pages 229-240
Mitochondrial Deletions in Normal and Degenerating Rat Retina....Pages 241-248
Quantitative PCR Analysis of FosB mRNA Expression after Short Duration Oxygen and Light Stress....Pages 249-257
Metabolic Modulation of Visual Sensitivity....Pages 259-267
Mitochondrial Uncoupling Proteins: Regulators of Retinal Cell Death....Pages 269-275
Energy Depletion Hypothesis for Retinitis Pigmentosa....Pages 277-285
Functional Studies of AIPL1....Pages 287-295
Photoreceptor Degeneration in Pro23His and S334ter Transgenic Rats....Pages 297-302
Retinal Degeneration Caused by Mutations in Tulip1....Pages 303-308
Towards Understanding the Function of the Tubby Gene Family in the Retina....Pages 309-314
Identification of Downstream Mechanisms Involved in PEDF’s Activity in the Retina by Large Scale Gene Expression Profiling....Pages 315-326
The nob Mutation does Not Protect against Light-Induced Retinal Degeneration....Pages 327-330
The Phagocytosis of OS is Mediated by the PI3-Kinase Linked Tyrosine Kinase Receptor, Mer, and is Stimulated by Gas6....Pages 331-336
Role of αvβ5 Integrin in Regulating Phagocytosis by the Retinal Pigment Epithelium....Pages 337-342
Comparative Study of Cathepsin D and S in Rat IPE and RPE Cells....Pages 343-346
Isolation and Culture of Primary Mouse Retinal Pigmented Epithelial Cells....Pages 347-352
Regulation of the Visual Cycle: Retinol Dehydrogenase and Retinol Fluorescence Measurements In Vertebrate Retina....Pages 353-360
Melatonin Enhances Retinoic Acid Induction of Cone Arrestin Gene Expression in Retinoblastoma Cells....Pages 361-368
Regulation of Retinal Phosphoinositide 3-Kinase Activity in p85α-Subunit Knockout Mice....Pages 369-376
Peripherin/ Rds in Skate Retina....Pages 377-383
XIAP Protects Photoreceptors from N-Methyl-N-Nitrosourea-Induced Retinal Degeneration....Pages 385-393
Neuroprotective Effect of Estrogen upon Retinal Neurons in Vitro....Pages 395-402
Transgenic rd Mice Harboring Axokine Gene by Rpe65 Gene Promoter Does not Rescue Photoreceptor Degeneration....Pages 403-406
Marrow Stromal Cells (MSC): A species Comparison....Pages 407-414
Gene Therapy for Leber Congenital Amaurosis....Pages 415-422
Functional and Structural Evaluation after AAV.RPE65 Gene Transfer in the Canine Model of Leber’s Congenital Amaurosis....Pages 423-430
Assessment of rAAV-Mediated Gene Therapy in the Rpe65 -/- Mouse....Pages 431-438
Müller Cell Transduction by AAV2 in Normal and Degenerative Retinas....Pages 439-445
Virus-Mediated Secretion Gene Therapy — A Potential Treatment for Ocular Neovascularization....Pages 447-453
Back Matter....Pages 455-467