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Файл:Ординатура / Офтальмология / Английские материалы / Retinal Degenerative Diseases Laboratory and Therapeutic Investigations_Anderson_2008.pdf
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- •Dedication
- •Dedication
- •Preface
- •Contents
- •Contributors
- •Travel Awards
- •About the Editors
- •1.1 Introduction
- •1.2 Methods
- •1.2.1 RNA Preparation and cDNA Labeling
- •1.2.2 Hybridization of Slides, Image Acquisition and Bioinformatics
- •1.2.3 Real-Time PCR
- •1.3.2 Microarray Analysis of Bouse C Model
- •1.3.3 Microarray Analysis of MOT1 Mouse
- •1.4 Discussion
- •References
- •2 Regulation of Angiogenesis by Macrophages
- •2.1 Macrophage Polarization and Its Role in Angiogenesis
- •References
- •3.1 Introduction
- •3.2 Materials and Methods
- •3.2.1 Reagents
- •3.2.2 Animals and Retina Explant Culture
- •3.2.3 Cell Culture
- •3.2.4 Western Blot Assay
- •3.3 Results
- •3.3.1 Phorbol Esters Increase Rod Generation
- •3.3.2 Expression of PKC Isoforms in Developing Retina
- •3.3.3 Activation of PKC Decreases Phosphorylation of STAT3
- •References
- •4.1 Introduction
- •4.3 In Silico Information
- •4.4 Expression and Distribution in the Retina
- •4.5 Transmembrane Topology
- •4.6 Binding to PEDF Ligands
- •4.7 Phospholipase Activity
- •4.8 PEDF-R Activity in Retinal Cells
- •4.9 Conclusions
- •References
- •References
- •6 The Association Between Telomere Length and Sensitivity to Apoptosis of HUVEC
- •6.1 Introduction
- •6.2 Methods
- •6.2.1 The Culture of HUVEC and the Construction of Cell Division Model
- •6.2.2 Construction of an Apoptosis Model of HUVEC with Free Hydroxyl Radicals
- •6.2.3 Measurement of Apoptosis Rates and Telomere Lengths
- •6.2.4 Statistics Analysis
- •6.3 Results
- •6.3.1 Relationship Between the Time of Culture and the Telomere Length
- •6.3.2 Relationship Among Apoptosis Rates, Culture Times and Oxidation
- •6.3.3 Oxidation Enhances the Telomere Shortening
- •6.4 Discussion
- •References
- •7.1 Regulation of cGMP Levels in Photoreceptor Outer Segments
- •7.2 Retinal Disorders Associated with Mutations in RetGCs and PDE6
- •7.3 Analysis of Teleost RetGC and PDEs in Retinal Function and Disorders
- •References
- •8 RDS in Cones Does Not Interact with the Beta Subunit of the Cyclic Nucleotide Gated Channel
- •References
- •9.1 Introduction
- •9.2 Material and Methods
- •9.2.1 Animals
- •9.2.2 Methods
- •9.2.3 Statistical Analysis
- •9.3 Results
- •9.4 Discussion
- •References
- •10.1 Introduction
- •10.2 Methods and Results
- •10.2.1 ZBED4 mRNA is Expressed in Human Retina
- •10.2.2 ZBED4 mRNA is Expressed in Mouse and Human Cones
- •10.2.3 ZBED4 is Expressed Both in Nuclei and Cytoplasm of Human Cones
- •10.2.3.1 Human ZBED4 is Also Expressed in Müller Cells Endfeet
- •10.2.4 Human ZBED4 is Distributed Between Nuclear and Cytoplasmic Retinal Fractions
- •10.2.5 Subcellular Localization of ZBED4 in Stably Transfected Cells
- •10.3 Discussion
- •References
- •11 Tubby-Like Protein 1 (Tulp1) Is Required for Normal Photoreceptor Synaptic Development
- •11.1 Introduction
- •11.2 Methods
- •11.2.1 Animals
- •11.3 Results
- •11.4 Discussion
- •References
- •12.1 Introduction
- •12.2 Experimental Procedures
- •12.2.1 Animal
- •12.2.2 Immunohistochemistry
- •12.2.3 RT-PCR Analysis
- •12.2.4 Behavioral Analysis
- •12.3 Results
- •12.4 Discussion
- •12.4.2 GAP43 Is a Good Marker for Monitoring the Long Process of Optic Nerve Regeneration in Fish
- •References
- •13 Multiprotein Complexes of Retinitis Pigmentosa GTPase Regulator (RPGR), a Ciliary Protein Mutated in X-Linked Retinitis Pigmentosa (XLRP)
- •13.1 X-Linked RP (XLRP)
- •13.2 Retinitis Pigmentosa GTPase Regulator (RPGR)
- •13.3 RPGR Isoforms in the Retina
- •13.4 Animal Models of RPGR
- •13.5 Sensory Cilia
- •13.6 Retinal Degeneration Caused by Mutations in Ciliary Proteins
- •13.9 Conclusion
- •References
- •14 Misfolded Proteins and Retinal Dystrophies
- •14.1 Endoplasmic Reticulum Stress and Retinal Degeneration
- •14.2 Misfolded Proteins in Photoreceptors
- •14.3 Misfolded Proteins in Retinal Pigment Epithelial Cells
- •14.4 Pharmacologic Targeting of Protein Misfolding to Prevent Retinal Degeneration
- •References
- •15.1 Introduction
- •15.6 Perspective
- •References
- •16.1 Introduction
- •16.2 RCS Rat and MerTK Receptor: An Intimate Story
- •16.3 Changes Associated with Absence of MerTK in the Rat Retina
- •16.4 Daily Rhythmic Activation of Mertk: The Intracellular Way
- •16.5 The Debate About MerTK Ligands In Vivo
- •16.6 Perspectives
- •References
- •17.1 Introduction
- •17.3 Implications for IRBP and Cone Function
- •17.4 The Cone Visual Cycle
- •References
- •18.1 Introduction
- •18.2 Material and Methods
- •18.2.1 Reagents
- •18.2.2 Cell Culture
- •18.2.3 Flow Cytometry
- •18.3 Results
- •18.3.2 Oxidative Stress of Renal Tubular Epithelial Cells Does Not Alter Surface Expression of Crry by the Cells
- •18.4 Discussion
- •References
- •19 Role of Metalloproteases in Retinal Degeneration Induced by Violet and Blue Light
- •19.1 Introduction
- •19.2 Objective
- •19.3 Materials and Methods
- •19.4 Results
- •19.5 Conclusion
- •References
- •20.1 Summary
- •20.2 Introduction
- •20.3 Materials and Methods
- •20.3.1 Primary Human RPE Cell Culture
- •20.3.3 Mitochondrial Morphometrics
- •20.3.4 Protein and Weight Estimation of RPE Cells and Mitochondria
- •20.3.7 Expression of Mitochondrial Associated Genes
- •20.4 Results
- •20.4.1 Age Related Sensitivity of RPE Cells to Oxidative Stress
- •20.4.2 Variation in Mitochondrial Number, Structure, and Size
- •20.4.5 Expression of Genes Associated with Mitochondrial Function
- •20.5 Discussion
- •References
- •21 Ciliary Transport of Opsin
- •21.1 Introduction
- •21.2 Methods
- •21.3 Results
- •21.4 Discussion
- •References
- •22 Effect of Hesperidin on Expression of Inducible Nitric Oxide Synthase in Cultured Rabbit Retinal Pigment Epithelial Cells
- •22.1 Introduction
- •22.2 Materials and Methods
- •22.2.1 Preparing Hesperidin Extract of Pericarpium Citri Reticulatae
- •22.2.3 Cell Culture
- •22.2.4 MTT Cell Viability Assay
- •22.2.5 Assay of NO Production
- •22.2.6 Cellular Immunohistochemistry of iNOS
- •22.2.7 Statistical Analysis
- •22.3 Results
- •22.3.2 RPE Cells Morphology
- •22.3.4 Assay of NO and iNOS
- •22.4 Discussion
- •References
- •23.1 Introduction
- •23.2 Materials and Methods
- •23.2.1 Rabbit Retina Tissues
- •23.2.2 RNA Extraction
- •23.2.3 miRNA Microarray Analysis
- •23.2.4 Data Analysis
- •23.2.5 Bioinformatics Analysis of the Selected Mirnas
- •23.3 Results and Discussion
- •23.3.1 miRNA Microarray Analysis
- •23.3.2 Putative miRNA Target Gene Prediction
- •References
- •24.1 Introduction
- •24.2 Materials and Methods
- •24.2.1 Experiment with Animals
- •24.2.2 -Galactosidase Assay
- •24.3 Results
- •24.3.1 Generation of Transgenic Mice
- •24.3.2 Localization of Cre Function in Transgenic Mice
- •24.4 Discussion
- •References
- •25.1 Introduction
- •25.2 Methods
- •25.3 Result
- •25.4 Conclusions
- •References
- •26.1 PSC Proteins Involved in Inherited Retinal Degenerations
- •26.2 Structure of Photoreceptor Sensory Cilium Complex
- •26.3 Protein Components of Photoreceptor Sensory Cilium: PSC Proteome
- •26.4 Novel Photoreceptor Cilia Proteins in PSC Proteome
- •26.4.1 Subcellular Locations of Candidate Novel PSC Proteins
- •26.4.2 Functional Analysis of Novel PSC Proteins in Photoreceptor and Renal Cilia
- •26.4.2.1 shRNAs Against Novel PSC Genes
- •26.4.2.2 Evaluation of Phenotypes of shRNA Knockdown in mIMCD3 Cells and PSCs
- •26.5 TTC21B Protein in Photoreceptor Sensory Cilia and Renal Primary Cilia
- •26.5.1 TTC21B Localizes to the Basal Bodies and Transition Zone of Primary and Photoreceptor Sensory Cilia
- •26.5.2 TTC21B is Required for Primary Cilia and Photoreceptor Sensory Cilia Formation
- •26.6 Future Direction: Screening Novel PSC Genes for Mutations that Cause IRDs
- •References
- •27.1 Introduction
- •27.2 Materials and Methods
- •27.2.1 RNA Interference
- •27.2.2 Construction of Mouse Anti Elovl4 Gene shRNA
- •27.2.3 Tissue Culture
- •27.2.4 Fatty Acid Analysis
- •27.3 Results
- •27.3.1 661W Cells Express Elovl4 and Can Elongate 18:3n3 and 22:5n3 to Longer Chain Fatty Acids
- •27.4 Discussion
- •References
- •28 Molecular Pathogenesis of Achromatopsia Associated with Mutations in the Cone Cyclic Nucleotide-Gated Channel CNGA3 Subunit
- •28.1 Introduction
- •28.2 Materials and Methods
- •28.2.1 Constructs, Cell Culture and Transfection
- •28.2.3 Electrophysiological Recordings
- •28.2.4 SDS-PAGE and Western Blot Analysis
- •28.3 Results
- •28.3.1 The R218C and R224W Mutations Cause Loss of Channel Function
- •28.4 Discussion
- •References
- •29.1 Introduction
- •29.2 Materials and Methods
- •29.2.1 Patients and Ophthalmologic Examinations
- •29.2.2 Molecular Genetic Analysis
- •29.3 Results and Discussion
- •29.3.1 adRP
- •29.3.2 Bothnia Dystrophy
- •29.4 Conclusions
- •References
- •30.1 Introduction
- •30.2 Properties of Rhodopsin CSNB Mutants
- •30.2.1 Spectral and Photochemical Properties
- •30.2.2 Retinal Binding Kinetics of Rhodopsin CSNB Mutants
- •30.2.3 Activity of CSNB Mutants
- •30.2.3.1 In Vitro Assays of CSNB Mutants
- •30.2.3.2 Electrophysiological Studies on Transgenic Animal Models
- •30.3 Proposed Mechanisms of CSNB Mutations
- •30.3.1 Desensitization Due to Mutant Opsin Activity in Xenopus
- •30.3.2 Proposed Dark-Active Rhodopsin in Mouse
- •30.4 Future Studies
- •References
- •31 GCAP1 Mutations Associated with Autosomal Dominant Cone Dystrophy
- •31.2 Guanylate Cyclase 1 (GC1) and GCAP1
- •31.3 The EF Hand Motifs of GCAP1
- •31.5 EF3: The GCAP1(Y99C) and GCAP1(N104K) Mutations
- •31.6 EF4: The GCAP1(I143NT), GCAP1(L151F) and GCAP1(E155G) Mutations
- •31.7 Conclusion
- •References
- •32.1 Introduction
- •32.2 Methodology
- •32.2.1 Molecular Genetic Studies
- •32.2.2 Electrophysiological Studies
- •32.3 Results
- •32.3.1 RS1 Mutations in Western Australian Families
- •32.3.3.1 Family Information
- •32.3.3.2 Patient Information
- •32.3.3.3 Genetic Information
- •32.4 Discussion
- •References
- •33.1 Introduction
- •33.2 Materials and Methods
- •33.2.1 Subjects
- •33.2.2 DNA Extraction
- •33.2.4 RFLP Analysis
- •33.2.5 Statistical Analysis
- •33.3 Results
- •33.4 Discussion
- •References
- •34.1 Introduction
- •34.2 Materials and Methods
- •34.2.1 Animal Experiments and Experimental Groups
- •34.2.2 Web-Based siRNA Design Protocols Targeting Claudin-5
- •34.2.4 Indirect Immunostaining of Retinal Flatmounts
- •34.2.5 Assessment of BRB Integrity by Perfusion of Hoechst (H33342)
- •34.2.6 Magnetic Resonance Imaging (MRI)
- •34.3 Results
- •34.3.1 Claudin-5 Levels in Retinal Flatmounts
- •34.3.3 MRI Analysis of Ibrb Integrity Following Rnai of Claudin-5
- •34.4 Discussion
- •References
- •35 Spectral Domain Optical Coherence Tomography and Adaptive Optics: Imaging Photoreceptor Layer Morphology to Interpret Preclinical Phenotypes
- •35.1 Introduction
- •35.2 Materials and Methods
- •35.2.1 Subjects
- •35.2.2 Adaptive Optics Retinal Imaging
- •35.2.3 Spectral Domain Optical Coherence Tomography
- •35.3 Results
- •35.3.1 Cone Photoreceptor Mosaic Topography
- •35.3.2 Outer Nuclear Layer Thickness
- •35.4 Discussion
- •References
- •36.1 Introduction
- •36.2 Pharmacological Strategies for Misfolding Mutant Rod Opsin
- •36.2.1 Pharmacological Chaperones
- •36.2.2 Kosmotropes
- •36.2.3 Molecular Chaperone Inducers
- •36.2.4 Autophagy Inducers
- •36.3 Conclusion
- •References
- •37 Targeted High-Throughput DNA Sequencing for Gene Discovery in Retinitis Pigmentosa
- •37.1 Introduction
- •37.2 Methods
- •37.2.1 Selection of Families
- •37.2.2 VisionCHIP Gene Selection
- •37.2.3 VisionCHIP Validation
- •37.2.4 Evaluating Potentially Pathogenic Variants
- •37.3 Conclusion
- •References
- •38 Advances in Imaging of Stargardt Disease
- •38.1 Introduction
- •38.4 Adaptive Optics Scanning Laser Ophthalmoscope
- •38.5 Conclusion
- •References
- •39.1 Materials and Methods
- •39.1.1 Cell Culture
- •39.1.3 VEGF Expression was Determined by ELISA
- •39.1.4 Statistical Analysis
- •39.2 Results
- •39.2.1 The Maximum Inhibition of VEGF Expression by Protamine Sulfate
- •39.2.2 Protamine Sulfate Inhibits the RF/6A Cell VEGF Expression at the Hypoxic Condition
- •39.2.3 Protamine Sulfate Inhibits the Binding of VEGF to Its Receptor
- •39.3 Discussions
- •39.3.1 The Inhibition Effect of Protamine Sulfate on VEGF
- •39.3.2 Inhibition of the Binding Between VEGF and Its Receptor
- •39.3.3 The Potential Use of Protamine Sulfate Inhibition of Angiogenic Eye Diseases
- •References
- •40.1 Introduction
- •40.2 Methods
- •40.2.1 Immunohistochemial Staining of Choroidal Endothelia
- •40.2.2 Analysis of Choriodal Density with Photoshop 8.0
- •40.3 Results and Discussion
- •40.3.1 Analysis Of Choroidal Density
- •40.3.2 Usefulness of the Methodology
- •40.3.3 Summary
- •References
- •41 Thioredoxins 1 and 2 Protect Retinal Ganglion Cells from Pharmacologically Induced Oxidative Stress, Optic Nerve Transection and Ocular Hypertension
- •41.1 Introduction
- •41.2 Methods
- •41.2.1 Animals
- •41.2.2 RGC Counting
- •41.2.3 RGC Isolation
- •41.2.4 Western Blot Analysis
- •41.2.5 RGC-5 Culture and Transfection
- •41.2.6 Cell Viability Assay
- •41.2.7 In Vivo Electroporation (ELP)
- •41.2.8 Statistical Analysis
- •41.3 Results
- •41.3.1.1 TRX Expression in RGC-5 Cells in Response to Oxidative Stress
- •41.3.1.2 The Levels of TRX Proteins After ONT
- •41.3.1.3 The Levels of TRX Proteins After IOP Elevation
- •41.3.2 The Effect of TRX1 and TRX2 Overexpression on RGC Survival
- •41.3.2.2 TRX1 and TRX2 Overexpression Increases RGC Survival After ONT
- •41.3.2.3 TRX1 and TRX2 Overexpression Increases RGC Survival After IOP Elevation
- •41.4 Discussion
- •References
- •42 Near-Infrared Light Protect the Photoreceptor from Light-Induced Damage in Rats
- •42.1 Introduction
- •42.2 Material and Methods
- •42.2.1 Animal
- •42.2.2 Light Damage
- •42.2.3 670 nm LED Treatment
- •42.2.4 Evaluation of Photoreceptor Cell Function by Electroretinography
- •42.2.5 Morphological Evaluation of Photoreceptor Rescue by Quantitative Histology
- •42.2.6 Statistical Analysis
- •42.3 Results
- •42.3.1 LED Attenuated the Light Damage Area in Retinas
- •42.3.2 LED Protected the Morphology of Light Damage Retina
- •42.3.3 LED Protected the Function of Light Damage Retina
- •References
- •43.1 Introduction
- •43.2 Methods
- •43.2.1 Animals
- •43.2.2 Cell Preparation and Subretinal Transplantation
- •43.2.3 Flash-Electroretinogram (F-ERG) Recordings
- •43.2.5 Data Analysis
- •43.3 Results
- •43.3.1 ERG Amplitudes and Latencies
- •43.3.2 ONL Thickness
- •43.3.3 Graft Cells Survival After Subretinal Transplantation
- •43.4 Discussion
- •References
- •44.1 Introduction
- •44.2 Mechanisms of ATP Release and Degradation
- •44.2.1 ATP Release
- •44.2.2 Degradation of ATP
- •44.3 Purinergic Signaling in the Retina
- •44.3.1 Purinergic Modulation of Neuronal Signaling
- •44.3.2 ATP and Glial Transmission
- •44.4 The Role of Purinergic Receptors in Retinal Disease
- •44.5 Concluding Remarks
- •References
- •45.1 Background
- •45.3 FAF Findings in Early AMD with Drusen Only
- •45.4 FAF Findings in Late AMD with Geographic Atrophy
- •45.5 Progression of Geographic Atrophy
- •45.6 Mechanisms of Progression
- •45.7 Research to Prevent Progression
- •45.8 Discussion
- •References
- •46 Endoplasmic Reticulum Stress as a Primary Pathogenic Mechanism Leading to Age-Related Macular Degeneration
- •46.1 Age Related Macular Degeneration Is a Leading Cause of Vision Loss
- •46.3 ER Stress and Oxidative Stress Interact
- •46.5 Future Experimental Approaches
- •References
- •47 Proteomic and Genomic Biomarkers for Age-Related Macular Degeneration
- •47.1 Introduction
- •47.2 Methods
- •47.3 Results
- •47.3.1 CEP Adducts and Autoantibodies Are Elevated in AMD Plasma
- •47.3.2 AMD Risk Based on CEP Biomarkers and Genotype
- •47.3.3 The Association Between CEP Biomarkers and AMD Risk Genotypes
- •47.4 Discussion
- •References
- •48.1 Introduction
- •48.2 Methods
- •48.2.1 Chemicals
- •48.2.2 Establishment and Maintenance of hRPE Cell Cultures
- •48.2.3 Cellular Proliferation
- •48.2.4 Immunoprecipitation Assay
- •48.2.5 Statistical Analysis
- •48.3 Results
- •48.3.1 Effect of Glucose on 14C-CTGF Synthesis in hRPE Cells
- •48.3.2 Effect of IGF-1 on 14C-CTGF Synthesis in hRPE cells
- •48.4 Discussion
- •References
- •49.1 Introduction
- •49.1.3 Peroxisome Proliferator Activated Receptors (PPARs) are Expressed in ARPE19 Cells
- •49.2 LcPUFA Regulates Gene Expression in ARPE19 Cells
- •49.2.1 Purpose and Methods
- •49.2.2 Results
- •49.2.3 Discussion
- •References
- •50.1 Introduction
- •50.2 Cigarette Smoking as a Risk Factor for AMD
- •50.2.1 AMD and Cigarette Smoke
- •50.2.2 Cigarette Smoke Constituents
- •50.3 Oxidative Stress
- •50.3.1 Oxidative Damage in AMD
- •50.3.2 Reactive Oxygen Species in Cigarette Smoke
- •50.3.3 Acrolein-Induced Oxidative Stress
- •50.3.4 Cadmium-Induced Oxidative Stress
- •50.4 Cigarette Smoke Depletion of Antioxidant Protection
- •50.4.1 Systemic Antioxidant Mechanisms
- •50.4.2 Local Ocular Antioxidants
- •50.5 Non-oxidative Chemical Damage by Cigarette Smoke
- •50.5.1 Nicotine
- •50.5.2 Polycyclic Aromatic Hydrocarbons
- •50.6.2 Cigarette Smoke and Complement Pathway
- •50.7 Vascular Changes
- •50.8 Conclusions
- •References
- •51.1 Oxidative Stress and Age-Related Macular Degeneration
- •51.2 The Ubiquitin Proteolytic System (UPS) and Oxidative Stress in the Retina
- •51.3 The UPS and the Cytoprotective Transcription Factor, Nrf2
- •References
- •52 Slit-Robo Signaling in Ocular Angiogenesis
- •52.1 Ocular Angiogenesis
- •52.2 Slit-Robo Signaling in Axon Guidance
- •52.3 Slit-Robo Signaling in Angiogenesis
- •52.4 Slit-Robo Signaling in Ocular Angiogenesis
- •52.5 Signaling Pathway of Slit-Robo System in Angiogenesis
- •52.6 Perspective
- •References
- •53.1 Introduction
- •53.2 Materials and Methods
- •53.2.1 Animals and Biosafety
- •53.2.2 MNU-Induced Retinal Degeneration
- •53.2.3 Electroretinography
- •53.2.4 Histological Examination and Immunohistochemistry
- •53.3 Results
- •53.3.1 Fundus Examination and Histology of the Retina
- •53.3.3 BrdU Incorporation
- •53.3.4 Immunohistology of Nestin
- •References
- •54 Differences in Photoreceptor Sensitivity to Oxygen Stress Between Long Evans and Sprague-Dawley Rats
- •54.1 Introduction
- •54.2 Methods
- •54.2.1 Animal Strains and Oxygen Exposure
- •54.2.2 Electroretinography
- •54.2.3 Immunohistochemistry and TUNEL Labeling
- •54.3 Results
- •54.3.1 Rod and Cone Components of the ERG after Hyperoxia
- •54.3.2 Impact of Hyperoxia on the Rate of Photo receptor Death
- •54.3.3 Impact of Hyperoxia on GFAP Expression
- •54.4 Discussion
- •References
- •55.1 Introduction
- •55.2 The AY9944 Rat Model of SLOS: Biochemical Findings
- •55.3 Retinal Degeneration in the SLOS Rat Model: Histology and Ultrastructure
- •References
- •56.1 Introduction
- •56.1.1 The Pde6brd1 Mouse and Increased [cGMP]
- •56.1.2 Calcium Regulation and Overload in the Photoreceptor Inner Segment
- •56.2 D-cis-diltiazem and Neuroprotection in the Retina
- •56.2.1 Criticism of the Frasson Study
- •56.3 Other Players May Be Involved
- •References
- •57.1 Introduction
- •57.2 Materials and Methods
- •57.2.1 Animals and Reagents
- •57.2.2 Induction of Retinal I/R
- •57.2.4 Statistical Analysis
- •57.3 Results
- •57.3.1 Effect of PBNA on Serum NO Content in Retinal I/R Injury
- •57.3.2 Effect of PBNA on T-NOS Activity in Retinal I/R Injury
- •57.3.3 Effect of PBNA on iNOS Activity in Retinal I/R Injury
- •57.3.4 Effect of PBNA on Serum eNOS Activity in Retinal I/R Injury
- •57.4 Discussion
- •References
- •References
- •59.1 Introduction
- •59.2 Materials and Methods
- •59.2.1 Experimental Animals
- •59.2.3 Construction of the pur-GFP Reporter Vector
- •59.2.4 Morpholino and Microinjections
- •59.2.5 In Situ Hybridization
- •59.2.6 RNA Isolation, RT-PCR and mRNA Synthesis
- •59.3 Results
- •59.3.2 Similar Phenotypes of Purpurin and Crx Morphant
- •References
- •60.1 Introduction
- •60.2 Bipolar Cell Function in Retinal Degeneration
- •60.2.1 Glutamate Receptors of Bipolar Cells in the Normal and Degenerating Retina
- •60.2.2 Evidence for Bipolar Cell Dysfunction
- •60.2.2.1 Rod Bipolar Cells
- •60.2.2.2 Cone Bipolar Cells
- •60.3 Ganglion Cell Function in Retinal Degeneration
- •References
- •61.1 Introduction
- •61.2 Methods
- •61.2.1 Animals and Rearing
- •61.2.2 Measurement of Outer Nuclear Layer Thickness
- •61.2.3 Counting Photoreceptor Nuclei
- •61.3 Results
- •61.4 Discussion
- •References
- •62.1 Introduction
- •62.2 Retinitis Pigmentosa
- •62.4 IMPDH Structure and Function
- •62.5 IMPDH Binds Single Stranded Nucleic Acids
- •62.6 Retinal Isoforms of IMPDH1
- •62.7 Kinetic and Nucleic Acid Binding Properties of Retinal IMPDH1
- •62.8 Conclusion
- •References
- •63.1 Introduction
- •63.2 Methods
- •63.3 Results
- •63.4 Discussion
- •63.5 Conclusion
- •References
- •64.1 Introduction
- •64.2 Results
- •64.2.1 Evaluation of Optimal IMPDH1 Suppressors
- •64.2.2 RP10 Mouse Model
- •64.3 Discussion
- •References
- •65 Correlation Between Tissue Docosahexaenoic Acid Levels and Susceptibility to Light-Induced Retinal Degeneration
- •65.1 Introduction
- •65.2 Methods
- •65.3 Results
- •65.4 Discussion
- •References
- •66.1 Introduction
- •66.2 Materials and Methods
- •66.2.1 Animal
- •66.2.2 Immunohistochemical Staining
- •66.2.3 Western Blot Test
- •66.2.4 Müller Cell Cultures
- •66.2.5 Data Analysis
- •66.3 Results
- •66.3.1 Morphology and Quantity Changes of Müller Cells
- •66.3.2 Expression of GFAP and ERK in RCS Rat Müller Cells
- •66.3.3 Effect of Mixed Retinal Cells of RCS Rats on Normal Müller Cells
- •66.4 Discussion
- •References
- •67.1 Introduction
- •67.2 Materials and Methods
- •67.2.1 Animals and Reagents
- •67.2.2 Induction of RI/R
- •67.2.4 Statistical Analysis
- •67.3 Results
- •67.3.1 The Effect of DSS on the Concentration of MDA in Serum After RI/R Injury
- •67.3.2 The Effect of DSS on the Activity of SOD in Serum After RI/R Injury
- •67.3.4 The Effect of DSS on the Concentration of Serum NO After RI/R Injury
- •67.4 Discussion
- •References
- •68.1 Introduction
- •68.2 Materials and Methods
- •68.2.1 Animals
- •68.2.2 Functional Testing
- •68.2.3 In Vivo Imaging
- •68.3 Results
- •68.3.1 Function
- •68.3.2 Morphology
- •68.4 Discussion
- •References
- •69.1 Introduction
- •69.2 Materials and Methods
- •69.2.1 Mice and Light Exposure
- •69.3 Results
- •69.3.3 Jak3 mRNA Is Induced Similarly in the Model of Light Induced Photoreceptor Cell Death and the rd1 Mouse Model
- •69.4 Discussion
- •References
- •70.1 Introduction
- •70.2 Diseases Associated with RDS Mutations
- •70.3 Current Animal Models
- •70.4 Gene Therapy in rds Models
- •70.5 Viral Gene Therapy Approaches
- •70.6 Non-viral Approaches
- •References
- •71.1 Introduction
- •71.2 Materials and Methods
- •71.2.1 Retinal Stem Cell Isolation and Culture
- •71.2.2 Single Sphere Passaging
- •71.2.3 Bromodeoxyuridine Labeling
- •71.2.4 Retinal Stem Cell Differentiation
- •71.3 Results
- •71.3.2 Retinal Neurosphere Proliferation
- •71.3.3 Differentiation of Retinal Cells Precursors from RSCs
- •71.4 Discussion
- •References
- •72 A Multi-Stage Color Model Revisited: Implications for a Gene Therapy Cure for Red-Green Colorblindness
- •72.1 Introduction
- •72.2 A Brief History of Color Vision Theory
- •72.3 Color Vision from an Evolutionary Perspective
- •References
- •73 Achromatopsia as a Potential Candidate for Gene Therapy
- •73.1 Human Achromatopsia
- •73.1.1 Clinical Manifestations
- •73.1.2 Current Achromatopsia Treatments
- •73.2 Genetics of Human Achromatopsia
- •73.2.1 GNAT2 Achromatopsia
- •73.2.2 CNG Achromatopsia
- •73.2.3 Achromatopsia Gene Therapy
- •73.3 The Mutant Gnat2 Mouse and Gene Therapy
- •73.3.1 The Cnga3 Mutant Mouse and Gene Therapy
- •73.3.2 The Cngb3 Mutant Dog and Gene Therapy
- •73.4 Prospects for Achromatopsia Gene Therapy
- •References
- •74.1 Introduction
- •74.2 Effects of CNTF/LIF on Photoreceptor and Bipolar Neuron Differentiation
- •74.3 Effects of CNTF/LIF on Muller Glia Genesis and Late Progenitor Proliferation
- •74.4 Effects of LIF Misexpression on Retinal Vasculature Development
- •74.5 Expression of CNTF/LIF Signaling Components in the Developing Retina
- •74.6 Signaling Events Triggered by CNTF/LIF During Retinogenesis
- •74.7 CNTF/LIF Regulate Numerous Genes Involved in Retinogenesis
- •74.8 Perspective
- •References
- •75.1 Introduction
- •75.4 Discussion
- •References
- •76.1 The Importance of RPE Cell Function and Integrity for Photoreceptor Survival
- •76.2 The Loss of RPE Cells in Retinal Degeneration
- •76.3 DHA and NPD1 Properties and Neuroprotection
- •References
- •77 Adeno-Associated Virus Serotype-9 Mediated Retinal Outer Plexiform Layer Transduction is Mainly Through the Photoreceptors
- •77.1 Introduction
- •77.2 AAV9-Mediated Gene Transfer in the Retina
- •77.5 Subretinal Injection of AAV9 Vector Did Not Cause Acute Retinal Damage
- •77.6 Conclusions
- •References
- •Index
5 The Function of Oligomerization-Incompetent RDS in Rods |
45 |
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