Retinal Degenerative Diseases: Mechanisms and Experimental Therapy

Front Matter....Pages i-lv
Front Matter....Pages 1-1
Apolipoprotein E Isoforms and AMD....Pages 3-9
Role of Chemokines in Shaping Macrophage Activity in AMD....Pages 11-16
Biology of p62/sequestosome-1 in Age-Related Macular Degeneration (AMD)....Pages 17-22
Gene Structure of the 10q26 Locus: A Clue to Cracking the ARMS2/HTRA1 Riddle?....Pages 23-29
Conditional Induction of Oxidative Stress in RPE: A Mouse Model of Progressive Retinal Degeneration....Pages 31-37
Therapeutic Approaches to Histone Reprogramming in Retinal Degeneration....Pages 39-44
A Brief Discussion on Lipid Activated Nuclear Receptors and their Potential Role in Regulating Microglia in Age-Related Macular Degeneration (AMD)....Pages 45-51
Extracellular Matrix Alterations and Deposit Formation in AMD....Pages 53-58
The NLRP3 Inflammasome and its Role in Age-Related Macular Degeneration....Pages 59-65
Oxidative Stress and the Nrf2 Anti-Oxidant Transcription Factor in Age-Related Macular Degeneration....Pages 67-72
Aging Changes in Retinal Microglia and their Relevance to Age-related Retinal Disease....Pages 73-78
VEGF-A and the NLRP3 Inflammasome in Age-Related Macular Degeneration....Pages 79-85
Interrelation Between Oxidative Stress and Complement Activation in Models of Age-Related Macular Degeneration....Pages 87-93
Gene-Diet Interactions in Age-Related Macular Degeneration....Pages 95-101
Challenges in the Development of Therapy for Dry Age-Related Macular Degeneration....Pages 103-109
Nanoceria: a Potential Therapeutic for Dry AMD....Pages 111-118
β-amyloidopathy in the Pathogenesis of Age-Related Macular Degeneration in Correlation with Neurodegenerative Diseases....Pages 119-125
Front Matter....Pages 127-127
Different Mutations in ELOVL4 Affect Very Long Chain Fatty Acid Biosynthesis to Cause Variable Neurological Disorders in Humans....Pages 129-135
Mouse Models of Stargardt 3 Dominant Macular Degeneration....Pages 137-143
Current Progress in Deciphering Importance of VLC-PUFA in the Retina....Pages 145-151
Front Matter....Pages 127-127
Malattia Leventinese/Doyne Honeycomb Retinal Dystrophy: Similarities to Age-Related Macular Degeneration and Potential Therapies....Pages 153-158
Front Matter....Pages 159-159
Hsp90 as a Potential Therapeutic Target in Retinal Disease....Pages 161-167
Leber Congenital Amaurosis: Genotypes and Retinal Structure Phenotypes....Pages 169-175
A Chemical Mutagenesis Screen Identifies Mouse Models with ERG Defects....Pages 177-183
Ablation of Chop Transiently Enhances Photoreceptor Survival but Does Not Prevent Retinal Degeneration in Transgenic Mice Expressing Human P23H Rhodopsin....Pages 185-191
Identification of a Novel Gene on 10q22.1 Causing Autosomal Dominant Retinitis Pigmentosa (adRP)....Pages 193-200
Mutations in the Dynein1 Complex are Permissible for Basal Body Migration in Photoreceptors but Alter Rab6 Localization....Pages 201-207
RDS Functional Domains and Dysfunction in Disease....Pages 209-215
Understanding Cone Photoreceptor Cell Death in Achromatopsia....Pages 217-222
Geranylgeranylacetone Suppresses N-Methyl-N-nitrosourea-Induced Photoreceptor Cell Loss in Mice....Pages 223-230
My Retina Tracker™: An On-line International Registry for People Affected with Inherited Orphan Retinal Degenerative Diseases and their Genetic Relatives - A New Resource....Pages 231-236
A Mini-review: Animal Models of GUCY2D Leber Congenital Amaurosis (LCA1)....Pages 237-243
A Comprehensive Review of Mutations in the MERTK Proto-Oncogene....Pages 245-251
Front Matter....Pages 253-258
Reliability and Repeatability of Cone Density Measurements in Patients with Congenital Achromatopsia....Pages 259-265
Quantitative Fundus Autofluorescence in Best Vitelliform Macular Dystrophy: RPE Lipofuscin is not Increased in Non-Lesion Areas of Retina....Pages 267-267
Intra-familial Similarity of Wide-Field Fundus Autofluorescence in Inherited Retinal Dystrophy....Pages 269-275
Front Matter....Pages 277-283
Wide-Field Fundus Autofluorescence for Retinitis Pigmentosa and Cone/Cone-Rod Dystrophy....Pages 285-290
The Development of a Cat Model of Retinal Detachment and Re-attachment....Pages 291-297
Front Matter....Pages 299-305
The Role of X-Chromosome Inactivation in Retinal Development and Disease....Pages 267-267
A Non-Canonical Role for β-Secretase in the Retina....Pages 307-313
The Consequences of Hypomorphic RPE65 for Rod and Cone Photoreceptors....Pages 315-321
The Rate of Vitamin A Dimerization in Lipofuscinogenesis, Fundus Autofluorescence, Retinal Senescence and Degeneration....Pages 323-323
Can Vitamin A be Improved to Prevent Blindness due to Age-Related Macular Degeneration, Stargardt Disease and Other Retinal Dystrophies?....Pages 325-331
Class I Phosphoinositide 3-Kinase Exerts a Differential Role on Cell Survival and Cell Trafficking in Retina....Pages 333-339
Cell Cycle Proteins and Retinal Degeneration: Evidences of New Potential Therapeutic Targets....Pages 341-346
Nitric Oxide Synthase Activation as a Trigger of N-methyl-N-nitrosourea-Induced Photoreceptor Cell Death....Pages 347-353
Molecular Principles for Decoding Homeostasis Disruptions in the Retinal Pigment Epithelium: Significance of Lipid Mediators to Retinal Degenerative Diseases....Pages 355-361
Aging and Vision....Pages 363-369
Front Matter....Pages 371-377
The Potential Use of PGC-1α and PGC-1β to Protect the Retina by Stimulating Mitochondrial Repair....Pages 379-384
Retinal Caveolin-1 Modulates Neuroprotective Signaling....Pages 385-391
Photoreceptor Neuroprotection: Regulation of Akt Activation Through Serine/Threonine Phosphatases, PHLPP and PHLPPL....Pages 393-399
The Role of AMPK Pathway in Neuroprotection....Pages 401-401
Tauroursodeoxycholic Acid Protects Retinal Function and Structure in rd1 Mice....Pages 403-409
Near-Infrared Photobiomodulation in Retinal Injury and Disease....Pages 411-418
Exercise and Cyclic Light Preconditioning Protect Against Light-Induced Retinal Degeneration and Evoke Similar Gene Expression Patterns....Pages 419-424
Small Molecules that Protect Mitochondrial Function from Metabolic Stress Decelerate Loss of Photoreceptor Cells in Murine Retinal Degeneration Models....Pages 425-430
Front Matter....Pages 431-436
Histone Deacetylase: Therapeutic Targets in Retinal Degeneration....Pages 437-441
Therapeutic Approach of Nanotechnology for Oxidative Stress Induced Ocular Neurodegenerative Diseases....Pages 443-448
Transscleral Controlled Delivery of Geranylgeranylaceton Using a Polymeric Device Protects Rat Retina Against Light Injury....Pages 449-454
Targeting the Proteostasis Network in Rhodopsin Retinitis Pigmentosa....Pages 401-401
Front Matter....Pages 455-461
Gene Therapy for MERTK-Associated Retinal Degenerations....Pages 463-469
Tamoxifen-Containing Eye Drops Successfully Trigger Cre-Mediated Recombination in the Entire Eye....Pages 471-477
Distinct Expression Patterns of AAV8 Vectors with Broadly Active Promoters from Subretinal Injections of Neonatal Mouse Eyes at Two Different Ages....Pages 479-484
Characterization of Ribozymes Targeting a Congenital Night Blindness Mutation in Rhodopsin Mutation....Pages 485-485
Antisense Oligonucleotide Therapy for Inherited Retinal Dystrophies....Pages 487-493
Functional Rescue of Retinal Degeneration-Associated Mutant RPE65 Proteins....Pages 495-500
Evaluation of Ocular Gene Therapy in an Italian Patient Affected by Congenital Leber Amaurosis Type 2 Treated in Both Eyes....Pages 501-507
Front Matter....Pages 509-515
Regenerative Medicine: Solution in Sight....Pages 517-524
Personalized Medicine: Cell and Gene Therapy Based on Patient-Specific iPSC-Derived Retinal Pigment Epithelium Cells....Pages 525-532
Human Retinal Pigment Epithelium Stem Cell (RPESC)....Pages 533-539
Embryonic Stem Cell-Derived Microvesicles: Could They be Used for Retinal Regeneration?....Pages 541-541
Intravitreal Implantation of Genetically Modified Autologous Bone Marrow-Derived Stem Cells for Treating Retinal Disorders....Pages 543-548
Gliosis Can Impede Integration Following Photoreceptor Transplantation into the Diseased Retina....Pages 549-555
Interkinetic Nuclear Migration in the Regenerating Retina....Pages 557-562
Front Matter....Pages 563-569
Use of a Machine Learning-Based High Content Analysis Approach to Identify Photoreceptor Neurite Promoting Molecules....Pages 571-577
A Novel Approach to Identify Photoreceptor Compartment-Specific Tulp1 Binding Partners....Pages 579-585
Front Matter....Pages 587-593
Thyroid Hormone Signaling and Cone Photoreceptor Viability....Pages 595-595
In-Depth Functional Diagnostics of Mouse Models by Single-Flash and Flicker Electroretinograms without Adapting Background Illumination....Pages 597-603
The Role of Intraflagellar Transport in the Photoreceptor Sensory Cilium....Pages 605-611
Regulation of Retinal Development via the Epigenetic Modification of Histone H3....Pages 595-595
The Potential Role of Flavins and Retbindin in Retinal Function and Homeostasis....Pages 613-618
Identification of Tyrosine O Sulfated Proteins in Cow Retina and the 661W Cell Line....Pages 619-625
The Function of Arf-like Proteins ARL2 and ARL3 in Photoreceptors....Pages 627-633
Characterization of Antibodies to Identify Cellular Expression of Dopamine Receptor 4....Pages 635-641
A Possible Role of Neuroglobin in the Retina After Optic Nerve Injury: A Comparative Study of Zebrafish and Mouse Retina....Pages 643-648
Cell Fate of Müller Cells During Photoreceptor Regeneration in an N-Methyl-N-nitrosourea-Induced Retinal Degeneration Model of Zebrafish....Pages 649-654
Polymodal Sensory Integration in Retinal Ganglion Cells....Pages 655-661
Front Matter....Pages 663-670
The mTOR Kinase Inhibitor INK128 Blunts Migration of Cultured Retinal Pigment Epithelial Cells....Pages 671-675
Cre Recombinase: You Can’t Live with It, and You Can’t Live Without It....Pages 677-683
Efficiency of Membrane Protein Expression Following Infection with Recombinant Adenovirus of Polarized Non-Transformed Human Retinal Pigment Epithelial Cells....Pages 685-692
Contribution of Ion Channels in Calcium Signaling Regulating Phagocytosis: MaxiK, Cav1.3 and Bestrophin-1....Pages 693-698
Lysosomal Trafficking Regulator (LYST)....Pages 699-706
Live-Cell Imaging of Phagosome Motility in Primary Mouse RPE Cells....Pages 707-707
Front Matter....Pages 709-715
RPE Cell and Sheet Properties in Normal and Diseased Eyes....Pages 717-723
Valproic Acid Induced Human Retinal Pigment Epithelial Cell Death as Well as its Survival after Hydrogen Peroxide Damage is Mediated by P38 Kinase.....Pages 725-730
Blockade of MerTK Activation by AMPK Inhibits RPE Cell Phagocytosis....Pages 731-737
Modulation of V-ATPase by βA3/A1-Crystallin in Retinal Pigment Epithelial Cells....Pages 739-744
Proteomic Profiling of Cigarette Smoke Induced Changes in Retinal Pigment Epithelium Cells....Pages 745-750
Reduced Metabolic Capacity in Aged Primary Retinal Pigment Epithelium (RPE) is Correlated with Increased Susceptibility to Oxidative Stress....Pages 751-755
Back Matter....Pages 707-707
....Pages 757-763