Recent Advances In Retinal Degeneration (eBook)

Dedication 6
Preface 7
Travel Awards 9
Contents 10
Contributors 16
Keynote Lectures 34
Curing Blindness with Stem Cells: Hope, Reality, and Challenges 35
Retinal Degenerations: Planning for the Future 53
Neuroprotection 68
Neurotrophins Induce Neuroprotective Signaling in the Retinal Pigment Epithelial Cell by Activating the Synthesis of the Anti- inflammatory and Anti- apoptotic Neuroprotectin D1 69
On the Role of CNTF as a Potential Therapy for Retinal Degeneration: Dr. Jekyll or Mr. Hyde? 75
Nanoceria Particles Prevent ROI-Induced Blindness 82
An in-vivo Assay to Identify Compounds Protective Against Light Induced Apoptosis 89
Role of BCL-XL in Photoreceptor Survival 96
The Hypoxic Transcriptome of the Retina: Identification of Factors with Potential Neuroprotective Activity 102
Gene Therapy and Neuroprotection 113
Lentiviral Gene Transfer-Mediated Cone Vision Restoration in RPE65 Knockout Mice 114
In vitro Analysis of Ribozyme-mediated Knockdown of an ADRP Associated Rhodopsin Mutation 121
Gene Therapy for Mouse Models of ADRP 131
Development of Viral Vectors with Optimal Transgene Expression for Ocular Gene Therapies 137
Adeno-Associated Viral Vectors and the Retina 144
Genetic Supplementation of RDS Alleviates a Loss- of- function Phenotype in C214S Model of Retinitis Pigmentosa 152
Morphological Aspects Related to Long-term Functional Improvement of the Retina in the 4 Years Following rAAV- mediated Gene Transfer in the RPE65 Null Mutation Dog 162
Virus-mediated Gene Delivery to Neuronal Progenitors 170
Animal Models of Retinal Degeneration 177
Loss of Visual and Retinal Function in Light- stressed Mice 178
ERG Responses and Microarray Analysis of Gene Expression in a Multifactorial Murine Model of Age- Related Retinal Degeneration 186
Oxygen Supply and Retinal Function: Insights from a Transgenic Animal Model 192
Characterization of Gene Expression Profiles of Normal Canine Retina and Brain Using a Retinal cDNA Microarray 199
Toward a Higher Fidelity Model of AMD 205
The Potential of Ambient Light Restriction to Restore Function to the Degenerating P23H- 3 Rat Retina 213
Molecular Genetics and Candidate Genes 220
Mutations in Known Genes Account for 58% of Autosomal Dominant Retinitis Pigmentosa ( adRP) 221
Genetics of Age-related Macular Degeneration 228
Retinal Phenotype of an X-Linked Pseudo-usherSyndrome in Association with the G173RMutation in the RPGR Gene 237
Mutation in the PYK2-Binding Domain of PITPNM3 Causes Autosomal Dominant Cone Dystrophy ( CORD5) in Two Swedish Families 244
Identification and Characterization of Genes Expressed in Cone Photoreceptors 250
Clinical and Genetic Characterization of a Chinese Family with CSNB1 260
10q26 Is Associated with Increased Risk of Age- Related Macular Degeneration in the Utah Population 268
Diagnostic, Clinical, Cytopathological and Physiologic Aspects of Retinal Degeneration 274
Carboxyethylpyrrole Adducts, Age-related Macular Degeneration and Neovascularization 275
A Possible Impaired Signaling Mechanism in Human Retinal Pigment Epithelial Cells from Patients with Macular Degeneration 282
Expression and Cell Compartmentalizationof EFEMP1, a Protein Associated with Malattia Leventinese 289
Role of ELOVL4 in Fatty Acid Metabolism 294
Organization and Molecular Interactions of Retinoschisin in Photoreceptors 302
Basic Science Underlying Retinal Degeneration 309
Proteomics Profiling of the Cone Photoreceptor Cell Line, 661W 310
.-Secretase Regulates VEGFR-1 Signallingin Vascular Endothelium and RPE 321
Analysis of the Rate of Disk Membrane Digestion by Cultured RPE Cells 328
Functional Expression of Cone Cyclic Nucleotide- Gated Channel in Cone Photoreceptor- Derived 661W Cells 334
Phosphorylation of Caveolin-1 in Bovine Rod Outer Segments in vitro by an Endogenous Tyrosine Kinase 342
Regulation of Neurotrophin Expression and Activity in the Retina 349
Involvement of Guanylate Cyclases in Transport of Photoreceptor Peripheral Membrane Proteins 356
Rod Progenitor Cells in the Mature Zebrafish Retina 365
avß5 Integrin Receptors at the Apical Surfaceof the RPE: One Receptor, Two Functions 373
Implantation of Mouse Eyes with a Subretinal Microphotodiode Array 380
Variation in the Electroretinogram of C57BL/6 Substrains of Mouse 386
A2E, A Pigment of RPE Lipofuscin, is Generated from the Precursor, A2PE by a Lysosomal Enzyme Activity 395
Endothelin Receptors: Do They Have a Role in Retinal Degeneration? 401
CNTF Negatively Regulates the Phototransduction Machinery in Rod Photoreceptors: Implication for Light- Induced Photostasis Plasticity 408
Index 418