RETINAL DEGENERATIVE DISEASES. MECHANISMS AND EXPERIMENTAL THERAPY

1. Apolipoprotein E Isoforms and AMD
2. Role of Chemokines in Shaping Macrophage Activity in AMD
3. Biology of p62/sequestosome-1 in Age-Related Macular Degeneration (AMD)
4. Gene Structure of the 10q26 Locus: A Clue to Cracking the ARMS2/HTRA1 Riddle?
5. Conditional Induction of Oxidative Stress in RPE: A Mouse Model of Progressive Retinal Degeneration
6. Therapeutic Approaches to Histone Reprogramming in Retinal Degeneration
7. A Brief Discussion on Lipid Activated Nuclear Receptors and their Potential Role in Regulating Microglia in Age-Related Macular Degeneration (AMD)
8. Extracellular Matrix Alterations and Deposit Formation in AMD
9. The NLRP3 Inflammasome and its Role in Age-Related Macular Degeneration
10. Oxidative Stress and the Nrf2 Anti-Oxidant Transcription Factor in Age-Related Macular Degeneration
11. Aging Changes in Retinal Microglia and their Relevance to Age-related Retinal Disease
12. VEGF-A and the NLRP3 Inflammasome in Age-Related Macular Degeneration
13. Interrelation Between Oxidative Stress and Complement Activation in Models of Age-Related Macular Degeneration
14. Gene-Diet Interactions in Age-Related Macular Degeneration
15. Challenges in the Development of Therapy for Dry Age-Related Macular Degeneration
16. Nanoceria: a Potential Therapeutic for Dry AMD
17. ß-amyloidopathy in the Pathogenesis of Age-Related Macular Degeneration in Correlation with Neurodegenerative Diseases
18. Different Mutations in ELOVL4 Affect Very Long Chain Fatty Acid Biosynthesis to Cause Variable Neurological Disorders in Humans
19. Mouse Models of Stargardt 3 Dominant Macular Degeneration
20. Current Progress in Deciphering Importance of VLC-PUFA in the Retina
21. Malattia Leventinese/Doyne Honeycomb Retinal Dystrophy: Similarities to Age-Related Macular Degeneration and Potential Therapies
22. Hsp90 as a Potential Therapeutic Target in Retinal Disease
23. Leber Congenital Amaurosis: Genotypes and Retinal Structure Phenotypes
24. A Chemical Mutagenesis Screen Identifies Mouse Models with ERG Defects
25. Ablation of
26. Identification of a Novel Gene on 10q22.1 Causing Autosomal Dominant Retinitis Pigmentosa (adRP)
27. FAM161A and TTC8 are Differentially Expressed in Non-Allelelic Early Onset Retinal Degeneration
28. Mutations in the Dynein1 Complex are Permissible for Basal Body Migration in Photoreceptors but Alter Rab6 Localization
29. RDS Functional Domains and Dysfunction in Disease
30. TULP1 Missense Mutations Induces the Endoplasmic Reticulum Unfolded Protein Response Stress Complex (ER-UPR)
31. Understanding Cone Photoreceptor Cell Death in Achromatopsia
32. Geranylgeranylacetone Suppresses
33. My Retina Tracker
34. A Mini-review: Animal Models of
35. A Comprehensive Review of Mutations in the
36. New Developments in Murine Imaging for Assessing Photoreceptor Degeneration
37. Reliability and Repeatability of Cone Density Measurements in Patients with Congenital Achromatopsia
38. Quantitative Fundus Autofluorescence in Best Vitelliform Macular Dystrophy: RPE Lipofuscin is not Increased in Non-Lesion Areas of Retina
39. Interpretation of Flood-Illuminated Adaptive Optics Images in Subjects with
40. Intra-familial Similarity of Wide-Field Fundus Autofluorescence in Inherited Retinal Dystrophy
41. Wide-Field Fundus Autofluorescence for Retinitis Pigmentosa and Cone/Cone-Rod Dystrophy
42. The Development of a Cat Model of Retinal Detachment and Re-attachment
43. The Role of X-Chromosome Inactivation in Retinal Development and Disease
44. A Non-Canonical Role for ß-Secretase in the Retina
45. The Consequences of Hypomorphic RPE65 for Rod and Cone Photoreceptors
46. The Rate of Vitamin A Dimerization in Lipofuscinogenesis, Fundus Autofluorescence, Retinal Senescence and Degeneration
47. Can Vitamin A be Improved to Prevent Blindness due to Age-Related Macular Degeneration, Stargardt Disease and Other Retinal Dystrophies?
48. Class I Phosphoinositide 3-Kinase Exerts a Differential Role on Cell Survival and Cell Trafficking in Retina
49. Cell Cycle Proteins and Retinal Degeneration: Evidences of New Potential Therapeutic Targets
50. Nitric Oxide Synthase Activation as a Trigger of
51. Molecular Principles for Decoding Homeostasis Disruptions in the Retinal Pigment Epithelium: Significance of Lipid Mediators to Retinal Degenerative Diseases
52. Aging and Vision
53. The Potential Use of PGC-1a and PGC-1ß to Protect the Retina by Stimulating Mitochondrial Repair
54. Retinal Caveolin-1 Modulates Neuroprotective Signaling
55. Photoreceptor Neuroprotection: Regulation of Akt Activation Through Serine/Threonine Phosphatases, PHLPP and PHLPPL
56. The Role of AMPK Pathway in Neuroprotection
57. Tauroursodeoxycholic Acid Protects Retinal Function and Structure in
58. Near-Infrared Photobiomodulation in Retinal Injury and Disease
59. Exercise and Cyclic Light Preconditioning Protect Against Light-Induced Retinal Degeneration and Evoke Similar Gene Expression Patterns
60. Small Molecules that Protect Mitochondrial Function from Metabolic Stress Decelerate Loss of Photoreceptor Cells in Murine Retinal Degeneration Models
61. Histone Deacetylase: Therapeutic Targets in Retinal Degeneration
62. Therapeutic Approach of Nanotechnology for Oxidative Stress Induced Ocular Neurodegenerative Diseases
63. Transscleral Controlled Delivery of Geranylgeranylaceton Using a Polymeric Device Protects Rat Retina Against Light Injury
64. Targeting the Proteostasis Network in Rhodopsin Retinitis Pigmentosa
65. Gene Therapy for
66. Tamoxifen-Containing Eye Drops Successfully Trigger
67. Distinct Expression Patterns of AAV8 Vectors with Broadly Active Promoters from Subretinal Injections of Neonatal Mouse Eyes at Two Different Ages
68. Characterization of Ribozymes Targeting a Congenital Night Blindness Mutation in Rhodopsin Mutation
69. Antisense Oligonucleotide Therapy for Inherited Retinal Dystrophies
70. Functional Rescue of Retinal Degeneration-Associated Mutant RPE65 Proteins
71. Evaluation of Ocular Gene Therapy in an Italian Patient Affected by Congenital Leber Amaurosis Type 2 Treated in Both Eyes
72. Regenerative Medicine: Solution in Sight
73. Personalized Medicine: Cell and Gene Therapy Based on Patient-Specific iPSC-Derived Retinal Pigment Epithelium Cells
74. Human Retinal Pigment Epithelium Stem Cell (RPESC)
75. Embryonic Stem Cell-Derived Microvesicles: Could They be Used for Retinal Regeneration?
76. Intravitreal Implantation of Genetically Modified Autologous Bone Marrow-Derived Stem Cells for Treating Retinal Disorders
77. Gliosis Can Impede Integration Following Photoreceptor Transplantation into the Diseased Retina
78. Interkinetic Nuclear Migration in the Regenerating Retina
79. Use of a Machine Learning-Based High Content Analysis Approach to Identify Photoreceptor Neurite Promoting Molecules
80. A Novel Approach to Identify Photoreceptor Compartment-Specific Tulp1 Binding Partners
81. Thyroid Hormone Signaling and Cone Photoreceptor Viability
82. In-Depth Functional Diagnostics of Mouse Models by Single-Flash and Flicker Electroretinograms without Adapting Background Illumination
83. The Role of Intraflagellar Transport in the Photoreceptor Sensory Cilium
84. Regulation of Retinal Development via the Epigenetic Modification of Histone H3
85. The Potential Role of Flavins and Retbindin in Retinal Function and Homeostasis
86. Identification of Tyrosine
87. The Function of Arf-like Proteins ARL2 and ARL3 in Photoreceptors
88. Characterization of Antibodies to Identify Cellular Expression of Dopamine Receptor 4
89. A Possible Role of Neuroglobin in the Retina After Optic Nerve Injury: A Comparative Study of Zebrafish and Mouse Retina
90. JNK Inhibition Reduced Retinal Ganglion Cell Death after Ischemia/Reperfusion
91. Cell Fate of Müller Cells During Photoreceptor Regeneration in an
92. Polymodal Sensory Integration in Retinal Ganglion Cells
93. Pigment Epithelium-Derived Factor, a Protective Factor for Photoreceptors
94. The mTOR Kinase Inhibitor INK128 Blunts Migration of Cultured Retinal Pigment Epithelial Cells
95. Live Imaging of LysoTracker-Labelled Phagolysosomes Tracks Diurnal Phagocytosis of Photoreceptor Outer Segment Fragments in Rat RPE Tissue
96. Cre Recombinase: You Can’t Live with It, and You Can’t Live Without It
97. Efficiency of Membrane Protein Expression Following Infection with Recombinant Adenovirus of Polarized Non-Transformed Human Retinal Pigment Epithelial Cells
98. Contribution of Ion Channels in Calcium Signaling Regulating Phagocytosis: MaxiK, Cav1.3 and Bestrophin-1
99. Lysosomal Trafficking Regulator (LYST)
100. Live-Cell Imaging of Phagosome Motility in Primary Mouse RPE Cells
101. RPE Cell and Sheet Properties in Normal and Diseased Eyes
102. Valproic Acid Induced Human Retinal Pigment Epithelial Cell Death as Well as its Survival after Hydrogen Peroxide Damage is Mediated by P38 Kinase.
103. Blockade of MerTK Activation by AMPK Inhibits RPE Cell Phagocytosis
104. Modulation of V-ATPase by ßA3/A1-Crystallin in Retinal Pigment Epithelial Cells
105. Proteomic Profiling of Cigarette Smoke Induced Changes in Retinal Pigment Epithelium Cells
106. Reduced Metabolic Capacity in Aged Primary Retinal Pigment Epithelium (RPE) is Correlated with Increased Susceptibility to Oxidative Stress
107. Erratum to: The Potential Use of PGC-1a and PGC-1ß to Protect the Retina by Stimulating Mitochondrial Repair

Contains the proceedings of the XVI International Symposium on Retinal Degeneration (RD2014), to be held July 13-18, 2014 at the Asilomar Conference Center in Pacific Grove, California. A majority of those who will speak and present posters at the meeting will contribute to this volume. The Symposium addresses the blinding diseases of inherited retinal degenerations, which have no effective treatments and age-related macular degeneration, which has no cures, despite the fact that it is an epidemic among the elderly, with 1 in 3-4 affected by the age of 75. The RD2014 Symposium will focus on the exciting new developments aimed at understanding these diseases and providing therapies for them.
The volume will present representative state-of-the-art research in almost all areas of retinal degenerations, ranging from cytopathologic, physiologic, diagnostic and clinical aspects; animal models; mechanisms of cell death; molecular genetics; and developing potential therapeutic measures such as gene therapy and neuroprotective agents for potential pharmaceutical therapy; and several sight restoration approaches, including optogenetics. While advances in these areas of retinal degenerations will be included, several new topics either were in their infancy or did not exist at the time of the last RD Symposium, RD2012. These include many new developments in sight restoration using optogenetics, retinal or RPE cell transplantation, stem cell approaches and visual prosthetic devices. In addition, major advances will be presented in other basic mechanisms in age-related macular degeneration, several new aspects of gene and antioxidant therapy and revolutionary new imaging and functional testing that will have a huge impact on the diagnosis and following the course of retinal degenerations, as well as to provide new quantitative endpoints for clinical trials. The retina is an approachable part of the central nervous system (CNS), and there is a major interest in neuroprotective and gene therapy for CNS diseases and neurodegenerations, in general. It should be noted that with successful and exciting initial clinical trials in neuroprotective and gene therapy, including the restoration of sight in blind children, the retinal degeneration therapies are leading the way towards new therapeutic measures for neurodegenerations of the CNS. Many of the successes recently reported in these areas of retinal degeneration sprang from collaborations established at previous RD Symposia, and many of those will be reported at the RD2014 meeting and included.

Features
• Presents representative state-of-the-art research in almost all areas of retinal degenerations
• Includes many new developments in sight restoration using optogenetics, retinal or RPE cell transplantation, stem cell approaches and visual prosthetic devices
• Contain the proceedings of the XVI International Symposium on Retinal Degeneration (RD2014)