Retinal Degenerative Diseases

Front Matter....Pages i-lxiv
Front Matter....Pages 1-1
A Window to Innate Neuroimmunity: Toll-Like Receptor-Mediated Cell Responses in the Retina....Pages 3-9
Autoimmune Biomarkers in Age-Related Macular Degeneration: A Possible Role Player in Disease Development and Progression....Pages 11-16
Local Vs. Systemic Mononuclear Phagocytes in Age-Related Macular Degeneration and Their Regulation by CCL2–CCR2 and CX3CL1–CX3CR1 Chemokine Signalling....Pages 17-22
Sublytic Membrane-Attack-Complex Activation and VEGF Secretion in Retinal Pigment Epithelial Cells....Pages 23-30
Complement Activation in Retinal Degeneration....Pages 31-36
Microglia in the Outer Retina and Their Relevance to Pathogenesis of Age-Related Macular Degeneration....Pages 37-42
Lutein or Zeaxanthin Supplementation Suppresses Inflammatory Responses in Retinal Pigment Epithelial Cells and Macrophages....Pages 43-50
Exploring the Potential Role of the Oxidant-Activated Transcription Factor Aryl Hydrocarbon Receptor in the Pathogenesis of AMD....Pages 51-59
Common Mechanisms for Separate Maculopathies?....Pages 61-66
The Role of Amyloid-β in Retinal Degeneration....Pages 67-74
Molecule-Specific Imaging and Quantitation of A2E in the RPE....Pages 75-81
Autophagy in the Retina: A Potential Role in Age-Related Macular Degeneration....Pages 83-90
Front Matter....Pages 91-91
Regeneration of Cone Outer Segments Induced by CNTF....Pages 93-99
Glucocorticoid-Dependent Mechanisms in Photoreceptor Survival....Pages 101-106
HDAC Inhibition Prevents Rd1 Mouse Photoreceptor Degeneration....Pages 107-113
Neuroprotective Dose Response in RCS Rats Implanted with Microphotodiode Arrays....Pages 115-120
Treatment with 670-nm Light Protects the Cone Photoreceptors from White Light-Induced Degeneration....Pages 121-128
Dark-Rearing the rd10 Mouse: Implications for Therapy....Pages 129-136
Intravitreal Injection of Erythropoietin Glycosylation Analogs Does Not Protect Rod Photoreceptor Cells from Light-Induced Damage....Pages 137-143
Relieving Bottlenecks in RNA Drug Discovery for Retinal Diseases....Pages 145-153
Front Matter....Pages 91-91
On Further Development of Barrier Modulation as a Technique for Systemic Ocular Drug Delivery....Pages 155-159
An Application for Mammalian Optic Nerve Repair by Fish Regeneration-Associated Genes....Pages 161-166
The Mechanism of Fenretinide (4-HPR) Inhibition of β-carotene Monooxygenase 1. New Suspect for the Visual Side Effects of Fenretinide....Pages 167-174
Front Matter....Pages 175-175
Gene Augmentation Trials Using the Rpe65-Deficient Dog: Contributions Towards Development and Refinement of Human Clinical Trials....Pages 177-182
Gene Therapy Restores Missing Cone-Mediated Vision in the CNGA3 −/− Mouse Model of Achromatopsia....Pages 183-189
Functional Rescue of P23H Rhodopsin Photoreceptors by Gene Delivery....Pages 191-197
Gene Delivery of Wild-Type Rhodopsin Rescues Retinal Function in an Autosomal Dominant Retinitis Pigmentosa Mouse Model....Pages 199-205
Retinal Degeneration and Cellular Suicide....Pages 207-214
Suppression of rds Expression by siRNA and Gene Replacement Strategies for Gene Therapy Using rAAV Vector....Pages 215-223
Silencing the Expression of CTRP5 / C1QTNF5 and ELOVL4 Genes by Small Interfering RNA....Pages 225-233
Gene Therapy Strategies for Usher Syndrome Type 1B....Pages 235-242
Front Matter....Pages 243-243
Neovascularization: Ocular Diseases, Animal Models and Therapies....Pages 245-252
Retinal Neovascular Disorders: Mouse Models for Drug Development Studies....Pages 253-259
A Review and Update on the Molecular Basis of Pathogenesis of Sorsby Fundus Dystrophy....Pages 261-267
The Importance of Hypoxia-Regulated, RPE-Targeted Gene Therapy for Choroidal Neovascularization....Pages 269-277
What Is the Role of CCR3 in Choroidal Neovascularization?....Pages 279-284
Intermittent But Not Constant High Glucose Induces ER Stress and Inflammation in Human Retinal Pericytes....Pages 285-292
Regulation of Retinal Vascular Permeability by Betacellulin....Pages 293-298
Presence of RPE-Produced VEGF in a Timely Manner Is Critical to Choroidal Vascular Development....Pages 299-304
Vasohibin-1 and Retinal Pigment Epithelium....Pages 305-310
Front Matter....Pages 311-311
Polymorphic Variation of RPGRIP1L and IQCB1 as Modifiers of X-Linked Retinitis Pigmentosa Caused by Mutations in RPGR....Pages 313-320
 RPGRIP1 and Cone–Rod Dystrophy in Dogs....Pages 321-328
High-Throughput Approaches for the Genetic Diagnosis of Retinal Dystrophies....Pages 329-335
Genes and Mutations in Autosomal Dominant Cone and Cone-Rod Dystrophy....Pages 337-343
The Power of Homozygosity Mapping: Discovery of New Genetic Defects in Patients with Retinal Dystrophy....Pages 345-351
Development and Validation of a Canine-Specific Profiling Array to Examine Expression of Pro-apoptotic and Pro-survival Genes in Retinal Degenerative Diseases....Pages 353-363
The Chromosome 10q26 Susceptibility Locus in Age-Related Macular Degeneration....Pages 365-370
Congenital Stationary Night Blindness: Mutation Update and Clinical Variability....Pages 371-379
Serum Biomarkers and Trafficking Defects in Peripheral Tissues Reflect the Severity of Retinopathy in Three Brothers Affected by Choroideremia....Pages 381-387
Front Matter....Pages 389-389
Translational Vision Research Models Program....Pages 391-397
Zebrafish: A Model System for the Investigation of Novel Treatments for Retinal Disease....Pages 399-405
Retinal Degeneration in the Fly....Pages 407-414
Looking into Eyes: Rhodopsin Pathologies in Drosophila ....Pages 415-423
Müller Glia as a Source of Neuronal Progenitor Cells to Regenerate the Damaged Zebrafish Retina....Pages 425-430
The Genetics of Outer Segment Morphogenesis in Zebrafish....Pages 431-441
Factor XIIIA Induction in the Retina and Optic Nerve After Optic Nerve Lesion in Goldfish....Pages 443-448
Front Matter....Pages 449-449
Imaging the Photoreceptor Mosaic with Adaptive Optics: Beyond Counting Cones....Pages 451-458
Baseline Imaging Reveals Preexisting Retinal Abnormalities in Mice....Pages 459-469
Correlation Between Spectral Domain OCT Retinal Nerve Fibre Layer Thickness and Multifocal Pattern Electroretinogram in Advanced Retinitis Pigmentosa....Pages 471-478
Imaging Human Postmortem Eyes with SLO and OCT....Pages 479-488
Front Matter....Pages 449-449
In Vivo Assessment of Rodent Retinal Structure Using Spectral Domain Optical Coherence Tomography....Pages 489-494
Rod Photoreceptor Temporal Properties in Retinal Degenerative Diseases....Pages 495-502
ERG Critical Flicker Frequency Assessment in Humans....Pages 503-509
Front Matter....Pages 511-511
Biology of Retinoschisin....Pages 513-518
Transcriptome Analyses to Investigate the Pathogenesis of RNA Splicing Factor Retinitis Pigmentosa....Pages 519-525
The Role of the X-linked Retinitis Pigmentosa Protein RP2 in Vesicle Traffic and Cilia Function....Pages 527-532
 Caenorhabditis elegans as a Model Organism for Ciliopathies and Related Forms of Photoreceptor Degeneration....Pages 533-538
Towards a Pathological Mechanism for IMPDH1-Linked Retinitis Pigmentosa....Pages 539-545
Calpain and Photoreceptor Apoptosis....Pages 547-552
Ceramide Signaling in Retinal Degeneration....Pages 553-558
Endoplasmic Reticulum-Associated Degradation (ERAD) of Misfolded Glycoproteins and Mutant P23H Rhodopsin in Photoreceptor Cells....Pages 559-565
Protein Misfolding and Potential Therapeutic Treatments in Inherited Retinopathies....Pages 567-572
Development of a Cellular Model of Rod Opsin Retinitis Pigmentosa....Pages 573-579
A Brief Account of Rho GTPases in Retinal Physiology and Pathophysiology....Pages 581-587
Molecular Clues to Bothnia-Type Retinal Dystrophy....Pages 589-594
A Novel Missense Mutation in Both OPN1LW and OPN1MW Cone Opsin Genes Causes X-Linked Cone Dystrophy (XLCOD5)....Pages 595-601
A Potential Cytosolic Function of Bestrophin-1....Pages 603-610
Modeling the Structural Consequences of BEST1 Missense Mutations....Pages 611-618
Microglial Activation and Transcriptomic Changes in the Blue Light-Exposed Mouse Retina....Pages 619-632
Overexpression of ROM-1 in the Cone-Dominant Retina....Pages 633-639
Front Matter....Pages 511-511
Analysis of the RPE Sheet in the rd10 Retinal Degeneration Model....Pages 641-647
Networks Modulating the Retinal Response to Injury: Insights from Microarrays, Expression Genetics, and Bioinformatics....Pages 649-656
Mislocalization of Oligomerization-Incompetent RDS is Associated with Mislocalization of Cone Opsins and Cone Transducin....Pages 657-662
HSP70 Gene Expression in the Zebrafish Retina After Optic Nerve Injury: A Comparative Study Under Heat Shock Stresses....Pages 663-668
Front Matter....Pages 669-669
Restoration of Retinal Development in Vsx2 Deficient Mice by Reduction of Gdf11 Levels....Pages 671-677
The Different Functions of Norrin....Pages 679-683
Roles of Homeobox Genes in Retinal Ganglion Cell Differentiation and Axonal Guidance....Pages 685-691
Unraveling the Molecular Mystery of Retinal Pigment Epithelium Phagocytosis....Pages 693-699
Isolating Photoreceptor Compartment-Specific Protein Complexes for Subsequent Proteomic Analysis....Pages 701-707
Expression of the Integrin Coreceptor Transglutaminase-2 in the RPE In Vivo and in Culture....Pages 709-715
On Your Marks… Get Bound… Internalize!....Pages 717-722
Endo-Lysosome Function in the Retinal Pigment Epithelium in Health and Disease....Pages 723-729
αvβ5 Integrin-Dependent Diurnal Phagocytosis of Shed Photoreceptor Outer Segments by RPE Cells Is Independent of the Integrin Coreceptor Transglutaminase-2....Pages 731-737
Trafficking of Presynaptic PMCA Signaling Complexes in Mouse Photoreceptors Requires Cav1.4 α 1 Subunits....Pages 739-744
Roles for AMP-Activated Protein Kinase in RPE Cell Function....Pages 745-751
Genome-Wide Occupancy Analysis by ChIP-chip and ChIP-Seq....Pages 753-759
The Bisretinoids of RPE Lipofuscin: A Complex Mixture....Pages 761-767
Biochemical Characterization of Cone Cyclic Nucleotide-Gated (CNG) Channel Using the Infrared Fluorescence Detection System....Pages 769-775
Ras-Associating Domain Proteins: A New Class of Cyclic Nucleotide-Gated Channel Modulators....Pages 777-782
Tulp1 Is Involved in Specific Photoreceptor Protein Transport Pathways....Pages 783-789
Front Matter....Pages 669-669
Potential Cellular Functions of N -Ethylmaleimide Sensitive Factor in the Photoreceptor....Pages 791-797
Identification of Pigment Epithelium-Derived Factor Receptor (PEDF-R) Antibody Epitopes....Pages 799-805
HCN1 Channels Significantly Shape Retinal Photoresponses....Pages 807-812
The Role of the P2X7 Receptor in the Retina: Cell Signalling and Dysfunction....Pages 813-819
A Tale of Two Kinases in Rods and Cones....Pages 821-827
Protein Tyrosine Phosphatase 1B: A Novel Molecular Target for Retinal Degenerative Diseases....Pages 829-834
Protein Tyrosine- O -Sulfation in Bovine Ocular Tissues....Pages 835-841
Erratum to: HCN1 Channels Significantly Shape Retinal Photoresponses....Pages E1-E1
Back Matter....Pages 843-864