Retinal Degenerative Diseases: Mechanisms and Experimental Therapy

Front Matter....Pages i-lxi
Front Matter....Pages 1-1
Cell Type-Specific Epigenetic Signatures Accompany Late Stages of Mouse Retina Development....Pages 3-8
Programmed Cell Death During Retinal Development of the Mouse Eye....Pages 9-13
Spatial and Temporal Localization of Caveolin-1 Protein in the Developing Retina....Pages 15-21
Glutathione S-Transferase Pi Isoform (GSTP1) Expression in Murine Retina Increases with Developmental Maturity....Pages 23-30
RETINA-Specific Expression of Kcnv2 Is Controlled by Cone-Rod Homeobox (Crx) and Neural Retina Leucine Zipper (Nrl)....Pages 31-41
AIPL1 Protein and its Indispensable Role in Cone Photoreceptor Function and Survival....Pages 43-48
Primate Short-Wavelength Cones Share Molecular Markers with Rods....Pages 49-56
Exploration of Cone Cyclic Nucleotide-Gated Channel-Interacting Proteins Using Affinity Purification and Mass Spectrometry....Pages 57-65
Electrophysiological Characterization of Rod and Cone Responses in the Baboon Nonhuman Primate Model....Pages 67-73
Front Matter....Pages 75-75
Animal Models, in “ The Quest to Decipher RPE Phagocytosis ”....Pages 77-83
In Vivo and in Vitro Monitoring of Phagosome Maturation in Retinal Pigment Epithelium Cells....Pages 85-90
Lack of Effect of Microfilament or Microtubule Cytoskeleton-Disrupting Agents on Restriction of Externalized Phosphatidylserine to Rod Photoreceptor Outer Segment Tips....Pages 91-96
Vacuolar ATPases and Their Role in Vision....Pages 97-103
Rescue of Compromised Lysosomes Enhances Degradation of Photoreceptor Outer Segments and Reduces Lipofuscin-Like Autofluorescence in Retinal Pigmented Epithelial Cells....Pages 105-111
The Role of Bestrophin-1 in Intracellular Ca 2+ Signaling....Pages 113-119
Front Matter....Pages 121-121
Application of Next-Generation Sequencing to Identify Genes and Mutations Causing Autosomal Dominant Retinitis Pigmentosa (adRP)....Pages 123-129
Digital Quantification of Goldmann Visual Fields (GVFs) as a Means for Genotype–Phenotype Comparisons and Detection of Progression in Retinal Degenerations....Pages 131-137
Simplified System to Investigate Alteration of Retinal Neurons in Diabetes....Pages 139-143
What is the Nature of the RGC-5 Cell Line?....Pages 145-154
Front Matter....Pages 155-155
Modeling Retinal Dystrophies Using Patient-Derived Induced Pluripotent Stem Cells....Pages 157-164
Front Matter....Pages 155-155
Mutation K42E in Dehydrodolichol Diphosphate Synthase (DHDDS) Causes Recessive Retinitis Pigmentosa....Pages 165-170
IROme, a New High-Throughput Molecular Tool for the Diagnosis of Inherited Retinal Dystrophies—A Price Comparison with Sanger Sequencing....Pages 171-176
Genetic Heterogeneity and Clinical Outcome in a Swedish Family with Retinal Degeneration Caused by Mutations in CRB1 and ABCA4 Genes....Pages 177-183
FAM161A, a Novel Centrosomal-Ciliary Protein Implicated in Autosomal Recessive Retinitis Pigmentosa....Pages 185-190
Front Matter....Pages 191-191
Molecular Pathology of Macrophages and Interleukin-17 in Age-Related Macular Degeneration....Pages 193-198
The Role of Monocytes and Macrophages in Age-Related Macular Degeneration....Pages 199-205
Microglia in the Aging Retina....Pages 207-212
The Role of Complement Dysregulation in AMD Mouse Models....Pages 213-219
Prolonged Src Kinase Activation, a Mechanism to Turn Transient, Sublytic Complement Activation into a Sustained Pathological Condition in Retinal Pigment Epithelium Cells....Pages 221-227
Inflammation in Age-Related Macular Degeneration....Pages 229-235
Impairment of the Ubiquitin-Proteasome Pathway in RPE Alters the Expression of Inflammation Related Genes....Pages 237-250
Inflammatory Biomarkers for AMD....Pages 251-257
Oxidized Low-Density-Lipoprotein-Induced Injury in Retinal Pigment Epithelium Alters Expression of the Membrane Complement Regulatory Factors CD46 and CD59 through Exosomal and Apoptotic Bleb Release....Pages 259-265
Should I Stay or Should I Go? Trafficking of Sub-Lytic MAC in the Retinal Pigment Epithelium....Pages 267-274
Hypoxia-Inducible Factor (HIF)/Vascular Endothelial Growth Factor (VEGF) Signaling in the Retina....Pages 275-281
Is Age-Related Macular Degeneration a Microvascular Disease?....Pages 283-289
Genetic Risk Models in Age-Related Macular Degeneration....Pages 291-300
A Mechanistic Review of Cigarette Smoke and Age-Related Macular Degeneration....Pages 301-307
Measuring Cone Density in a Japanese Macaque ( Macaca fuscata ) Model of Age-Related Macular Degeneration with Commercially Available Adaptive Optics....Pages 309-316
Nuclear Receptors as Potential Therapeutic Targets for Age-Related Macular Degeneration....Pages 317-321
Front Matter....Pages 191-191
Utilizing Stem Cell-Derived RPE Cells as A Therapeutic Intervention for Age-Related Macular Degeneration....Pages 323-329
Front Matter....Pages 331-331
Microglia-Müller Cell Interactions in the Retina....Pages 333-338
Isolation and Ex Vivo Characterization of the Immunophenotype and Function of Microglia/Macrophage Populations in Normal Dog Retina....Pages 339-345
Müller Cells and Microglia of the Mouse Eye React Throughout the Entire Retina in Response to the Procedure of an Intravitreal Injection....Pages 347-353
Subretinal Infiltration of Monocyte Derived Cells and Complement Misregulation in Mice with AMD-Like Pathology....Pages 355-363
Ambiguous Role of Glucocorticoids on Survival of Retinal Neurons....Pages 365-371
Microglia-Müller Glia Crosstalk in the rd10 Mouse Model of Retinitis Pigmentosa....Pages 373-379
The Neuroprotective Potential of Retinal Müller Glial Cells....Pages 381-387
Leukemia Inhibitory Factor Signaling in Degenerating Retinas....Pages 389-394
In Vivo Function of the ER-Golgi Transport Protein LMAN1 in Photoreceptor Homeostasis....Pages 395-399
Investigating the Role of Retinal Müller Cells with Approaches in Genetics and Cell Biology....Pages 401-405
Front Matter....Pages 407-407
An Overview of the Involvement of Interleukin-18 in Degenerative Retinopathies....Pages 409-415
Chronic Intraocular Inflammation and Development of Retinal Degenerative Disease....Pages 417-425
The Relevance of Chemokine Signalling in Modulating Inherited and Age-Related Retinal Degenerations....Pages 427-433
The Complement Regulatory Protein CD59: Insights into Attenuation of Choroidal Neovascularization....Pages 435-440
Regeneration-Associated Genes on Optic Nerve Regeneration in Fish Retina....Pages 441-446
Dominant Stargardt Macular Dystrophy (STGD3) and ELOVL4....Pages 447-453
Modulation of the Rate of Retinal Degeneration in T17M RHO Mice by Reprogramming the Unfolded Protein Response.....Pages 455-462
Expression of Poly(ADP-Ribose) Glycohydrolase in Wild-Type and PARG-110 Knock-Out Retina....Pages 463-469
Current Therapeutic Strategies for P23H RHO-Linked RP....Pages 471-476
Front Matter....Pages 407-407
Pathogenesis of X-linked RP3: Insights from Animal Models....Pages 477-485
Retinal Function in Aging Homozygous Cln3 Δex7/8 Knock-In Mice....Pages 487-493
Synergistic Interaction of Tubby and Tubby-Like Protein 1 (Tulp1)....Pages 495-501
Interaction of Tubby-Like Protein-1 (Tulp1) and Microtubule-Associated Protein (MAP) 1A and MAP1B in the Mouse Retina....Pages 503-509
CEP290 and the Primary Cilium....Pages 511-518
Usher Syndrome Protein Network Functions in the Retina and their Relation to Other Retinal Ciliopathies....Pages 519-525
The Phenotype of the Good Effort Mutant Zebrafish is Retinal Degeneration by Cell Death and is Linked to the Chromosome Assembly Factor 1b Gene....Pages 527-533
Knock-Down DHDDS Expression Induces Photoreceptor Degeneration in Zebrafish....Pages 535-541
Spectral Domain Optical Coherence Tomography Findings in CNGB3-Associated Achromatopsia and Therapeutic Implications....Pages 543-550
Photoreceptor Pathology in the X-Linked Retinoschisis (XLRS) Mouse Results in Delayed Rod Maturation and Impaired Light Driven Transducin Translocation....Pages 551-557
Mouse Models for Cone Degeneration....Pages 559-566
How Long Does a Photoreceptor Cell Take to Die? Implications for the Causative Cell Death Mechanisms....Pages 567-573
Front Matter....Pages 575-581
Endoplasmic Reticulum Stress in Vertebrate Mutant Rhodopsin Models of Retinal Degeneration....Pages 583-583
Bisretinoid Degradation and the Ubiquitin-Proteasome System....Pages 585-592
Analysis of Mouse RPE Sheet Morphology Gives Discriminatory Categories....Pages 593-600
High Glucose Activates ChREBP-Mediated HIF-1α and VEGF Expression in Human RPE Cells Under Normoxia....Pages 601-607
Sphingolipids in Ocular Inflammation....Pages 609-621
Biosynthesis of Very Long-Chain Polyunsaturated Fatty Acids in Hepatocytes Expressing ELOVL4....Pages 623-629
Very Long Chain Polyunsaturated Fatty Acids and Rod Cell Structure and Function....Pages 631-636
Front Matter....Pages 637-645
Oxidative Stress Regulation by DJ-1 in the Retinal Pigment Epithelium....Pages 647-647
The Role of Reactive Oxygen Species in Ocular Malignancy....Pages 649-654
The Effects of IRE1, ATF6, and PERK Signaling on adRP-Linked Rhodopsins....Pages 655-659
Role of Endothelial Cell and Pericyte Dysfunction in Diabetic Retinopathy: Review of Techniques in Rodent Models....Pages 661-667
Autophagy Induction Does Not Protect Retina Against Apoptosis in Ischemia/Reperfusion Model....Pages 669-675
Front Matter....Pages 677-683
Advances in AAV Vector Development for Gene Therapy in the Retina....Pages 685-685
Cone Specific Promoter for Use in Gene Therapy of Retinal Degenerative Diseases....Pages 687-693
Episomal Maintenance of S/MAR-Containing Non-Viral Vectors for RPE-Based Diseases....Pages 695-701
Gene Therapy in the Rd6 Mouse Model of Retinal Degeneration....Pages 703-709
Gene Therapy for Stargardt Disease Associated with ABCA4 Gene....Pages 711-718
Assessment of Different Virus-Mediated Approaches for Retinal Gene Therapy of Usher 1B....Pages 719-724
Gene Therapy Restores Vision and Delays Degeneration in the CNGB1 −/− Mouse Model of Retinitis Pigmentosa....Pages 725-731
Therapy Strategies for Usher Syndrome Type 1C in the Retina....Pages 733-739
Front Matter....Pages 741-747
Nipradilol Promotes Axon Regeneration Through S-Nitrosylation of PTEN in Retinal Ganglion Cells....Pages 749-749
Reciprocal Changes in Factor XIII and Retinal Transglutaminase Expressions in the Fish Retina During Optic Nerve Regeneration....Pages 751-757
 N -Acetylserotonin: Circadian Activation of the BDNF Receptor and Neuroprotection in the Retina and Brain....Pages 759-764
A High Content Screening Approach to Identify Molecules Neuroprotective for Photoreceptor Cells....Pages 765-771
Antioxidant Therapy for Retinal Disease....Pages 773-781
Pathophysilogical Mechanism and Treatment Strategies for Leber Congenital Amaurosis....Pages 783-789
Current and Emerging Therapies for Ocular Neovascularisation....Pages 791-796
Front Matter....Pages 797-804
Targeting the PI3K/Akt/mTOR Pathway in Ocular Neovascularization....Pages 749-749
Pigment Epithelium-Derived Factor Protects Cone Photoreceptor-Derived 661W Cells from Light Damage Through Akt Activation....Pages 805-811
Nanoceria as Bona Fide Catalytic Antioxidants in Medicine: What We Know and What We Want to Know…....Pages 813-820
Nanoceria and Thioredoxin Regulate a Common Antioxidative Gene Network in tubby Mice....Pages 821-828
Intrascleral Transplantation of a Collagen Sheet with Cultured Brain-Derived Neurotrophic Factor Expressing Cells Partially Rescues the Retina from Damage due to Acute High Intraocular Pressure....Pages 829-836
Neuroprotective Effects of Low Level Electrical Stimulation Therapy on Retinal Degeneration....Pages 837-843
Back Matter....Pages 845-851
....Pages 853-862