Retinal Degenerative Diseases

1. A Window to Innate Neuroimmunity: Toll-Like Receptor-Mediated Cell Responses in the Retina
Mark E. Kleinman, Jayakrishna Ambati

2. Autoimmune Biomarkers in Age-Related Macular Degeneration: A Possible Role Player in Disease Development and Progression
Alessandro Iannaccone, Indira Neeli, Pratheebha Krishnamurthy, Nataliya I. Lenchik, Haibao Wan, Ivan C. Gerling, Dominic M. Desiderio, Marko Z. Radic

3. Local Vs. Systemic Mononuclear Phagocytes in Age-Related Macular Degeneration and Their Regulation by CCL2–CCR2 and CX3CL1–CX3CR1 Chemokine Signalling
Ulrich F. O. Luhmann, Robin R. Ali

4. Sublytic Membrane-Attack-Complex Activation and VEGF Secretion in Retinal Pigment Epithelial Cells
Kannan Kunchithapautham, Mausumi Bandyopadhyay, Mohammad Dahrouj, Joshua M. Thurman, Bärbel Rohrer

5. Complement Activation in Retinal Degeneration
Matt Rutar, Riccardo Natoli, Jan Provis, Krisztina Valter

6. Microglia in the Outer Retina and Their Relevance to Pathogenesis of Age-Related Macular Degeneration
Wenxin Ma, Lian Zhao, Wai T. Wong

7. Lutein or Zeaxanthin Supplementation Suppresses Inflammatory Responses in Retinal Pigment Epithelial Cells and Macrophages
Qingning Bian, Tingyu Qin, Zhihong Ren, Dayong Wu, Fu Shang

8. Exploring the Potential Role of the Oxidant-Activated Transcription Factor Aryl Hydrocarbon Receptor in the Pathogenesis of AMD
Goldis Malek, Mary Dwyer, Donald McDonnell

9. Common Mechanisms for Separate Maculopathies?
Elod Kortvely, Marius Ueffing

10. The Role of Amyloid-β in Retinal Degeneration
Julien Bruban, Virginie Dinet, Frédéric Mascarelli

11. Molecule-Specific Imaging and Quantitation of A2E in the RPE
Zsolt Ablonczy, Danielle B. Gutierrez, Angus C. Grey, Kevin L. Schey, Rosalie K. Crouch

12. Autophagy in the Retina: A Potential Role in Age-Related Macular Degeneration
Sayak K. Mitter, Haripriya Vittal Rao, Xiaoping Qi, Jun Cai, Andrew Sugrue, William A. Dunn, Maria B. Grant, Michael E. Boulton

13. Regeneration of Cone Outer Segments Induced by CNTF
Rong Wen, Weng Tao, Lingyu Luo, Deqiang Huang, Konrad Kauper, Paul Stabila, Matthew M. LaVail, Alan M. Laties, Yiwen Li

14. Glucocorticoid-Dependent Mechanisms in Photoreceptor Survival
Marisa A. Cubilla, Mauricio M. Castañeda, Tomás P. Bachor, Angela M. Suburo

15. HDAC Inhibition Prevents Rd1 Mouse Photoreceptor Degeneration
Javier Sancho-Pelluz, François Paquet-Durand

16. Neuroprotective Dose Response in RCS Rats Implanted with Microphotodiode Arrays
Machelle T. Pardue, Moon K. Kim, Tiffany A. Walker, Amanda E. Faulkner, Alan Y. Chow, Vincent T. Ciavatta

17. Treatment with 670-nm Light Protects the Cone Photoreceptors from White Light-Induced Degeneration
Rizalyn S. Albarracin, Krisztina Valter

18. Dark-Rearing the rd10 Mouse: Implications for Therapy
Therese Cronin, Arkady Lyubarsky, Jean Bennett

19. Intravitreal Injection of Erythropoietin Glycosylation Analogs Does Not Protect Rod Photoreceptor Cells from Light-Induced Damage
Masaki Tanito, Feng Li, Robert E. Anderson

20. Relieving Bottlenecks in RNA Drug Discovery for Retinal Diseases
Jack M. Sullivan, Edwin H. Yau, R. Thomas Taggart, Mark C. Butler, Tiffany A. Kolniak

21. On Further Development of Barrier Modulation as a Technique for Systemic Ocular Drug Delivery
Finnian Hanrahan, Matthew Campbell, Anh T. Nguyen, Mayu Suzuki, Anna-Sophia Kiang, Lawrence C. Tam, Oliviero L. Gobbo, Sorcha Ní Dhubhghaill, Marian M. Humphries, Paul F. Kenna, Pete Humphries

22. An Application for Mammalian Optic Nerve Repair by Fish Regeneration-Associated Genes
Yoshiki Koriyama, Kayo Sugitani, Toru Matsukawa, Satoru Kato

23. The Mechanism of Fenretinide (4-HPR) Inhibition of β-carotene Monooxygenase 1. New Suspect for the Visual Side Effects of Fenretinide
Eugenia Poliakov, Alexander Gubin, James Laird, Susan Gentleman, Robert G. Salomon, T. Michael Redmond

24. Gene Augmentation Trials Using the Rpe65-Deficient Dog: Contributions Towards Development and Refinement of Human Clinical Trials
Simon M. Petersen-Jones, Matthew J. Annear, Joshua T. Bartoe, Freya M. Mowat, Susie E. Barker, Alexander J. Smith, James W. Bainbridge, Robin R. Ali

25. Gene Therapy Restores Missing Cone-Mediated Vision in the CNGA3−/− Mouse Model of Achromatopsia
Stylianos Michalakis, Regine Mühlfriedel, Naoyuki Tanimoto, Vidhyasankar Krishnamoorthy, Susanne Koch, M. Dominik Fischer, Elvir Becirovic, Lin Bai, Gesine Huber, Susanne C. Beck, Edda Fahl, Hildegard Büning, Jennifer Schmidt, Xiangang Zong, Tim Gollisch, Martin Biel, Mathias W. Seeliger

26. Functional Rescue of P23H Rhodopsin Photoreceptors by Gene Delivery
Marina S. Gorbatyuk, Oleg S. Gorbatyuk, Matthew M. LaVail, Jonathan H. Lin, William W. Hauswirth, Alfred S. Lewin

27. Gene Delivery of Wild-Type Rhodopsin Rescues Retinal Function in an Autosomal Dominant Retinitis Pigmentosa Mouse Model
Haoyu Mao, Marina S. Gorbatyuk, William W. Hauswirth, Alfred S. Lewin

28. Retinal Degeneration and Cellular Suicide
Wai Gin Fong, Catherine Tsilfidis

29. Suppression of rds Expression by siRNA and Gene Replacement Strategies for Gene Therapy Using rAAV Vector
Hilda Petrs-Silva, Douglas Yasumura, Michael T. Matthes, Matthew M. LaVail, Alfred S. Lewin, William W. Hauswirth

30. Silencing the Expression of CTRP5/C1QTNF5 and ELOVL4 Genes by Small Interfering RNA
Venkata Ramana Murthy Chavali, Vidyullatha Vasireddy, Radha Ayyagari

31. Gene Therapy Strategies for Usher Syndrome Type 1B
David S. Williams, Vanda S. Lopes

32. Neovascularization: Ocular Diseases, Animal Models and Therapies
Xue Cai, Steven A. Sezate, James F. McGinnis

33. Retinal Neovascular Disorders: Mouse Models for Drug Development Studies
Rosanne M. Yetemian, Cheryl M. Craft

34. A Review and Update on the Molecular Basis of Pathogenesis of Sorsby Fundus Dystrophy
Heidi Stöhr, Bela Anand-Apte

35. The Importance of Hypoxia-Regulated, RPE-Targeted Gene Therapy for Choroidal Neovascularization
George W. Smith, C. Kathleen Dorey, Howard Prentice, Janet Blanks

36. What Is the Role of CCR3 in Choroidal Neovascularization?
Yiwen Li, Deqiang Huang, Xin Xia, Zhengying Wang, Lingyu Luo, Rong Wen

37. Intermittent But Not Constant High Glucose Induces ER Stress and Inflammation in Human Retinal Pericytes
Yimin Zhong, Joshua J. Wang, Sarah X. Zhang

38. Regulation of Retinal Vascular Permeability by Betacellulin
Masahiko Sugimoto, Alecia Cutler, Gregory Grossman, Bela Anand-Apte

39. Presence of RPE-Produced VEGF in a Timely Manner Is Critical to Choroidal Vascular Development
Meili Zhu, Yanyan Bai, Lixin Zheng, Yun-Zheng Le

40. Vasohibin-1 and Retinal Pigment Epithelium
Yumi Ishikawa, Nobuhiro Nagai, Hideyuki Onami, Norihiro Kumasaka, Ryosuke Wakusawa, Hikaru Sonoda, Yasufumi Sato, Toshiaki Abe

41. Polymorphic Variation of RPGRIP1L and IQCB1 as Modifiers of X-Linked Retinitis Pigmentosa Caused by Mutations in RPGR
Abigail T. Fahim, Sara J. Bowne, Lori S. Sullivan, Kaylie D. Webb, Jessica T. Williams, Dianna K. Wheaton, David G. Birch, Stephen P. Daiger

42.
RPGRIP1 and Cone–Rod Dystrophy in Dogs
Tatyana Kuznetsova, Barbara Zangerl, Gustavo D. Aguirre

43. High-Throughput Approaches for the Genetic Diagnosis of Retinal Dystrophies
Esther Pomares, Gemma Marfany, Roser Gonzàlez-Duarte

44. Genes and Mutations in Autosomal Dominant Cone and Cone-Rod Dystrophy
Susanne Kohl, Veronique Kitiratschky, Monika Papke, Simone Schaich, Alexandra Sauer, Bernd Wissinger

45. The Power of Homozygosity Mapping: Discovery of New Genetic Defects in Patients with Retinal Dystrophy
Karin W. Littink, Anneke I. Hollander, Frans P. M. Cremers, Rob W. J. Collin

46. Development and Validation of a Canine-Specific Profiling Array to Examine Expression of Pro-apoptotic and Pro-survival Genes in Retinal Degenerative Diseases
Sem Genini, William A. Beltran, Gustavo D. Aguirre

47. The Chromosome 10q26 Susceptibility Locus in Age-Related Macular Degeneration
Chloe M. Stanton, Kevin J. Chalmers, Alan F. Wright

48. Congenital Stationary Night Blindness: Mutation Update and Clinical Variability
Nidhi Lodha, Catrina M. Loucks, Chandree Beaulieu, Jillian S. Parboosingh, N. Torben Bech-Hansen

49. Serum Biomarkers and Trafficking Defects in Peripheral Tissues Reflect the Severity of Retinopathy in Three Brothers Affected by Choroideremia
Natalia Strunnikova, Wadih M. Zein, Chris Silvin, Ian M. MacDonald

50. Translational Vision Research Models Program
Jungyeon Won, Lan Ying Shi, Wanda Hicks, Jieping Wang, Juergen K. Naggert, Patsy M. Nishina

51. Zebrafish: A Model System for the Investigation of Novel Treatments for Retinal Disease
Cheryl Y. Gregory-Evans

52. Retinal Degeneration in the Fly
Nansi Jo Colley

53. Looking into Eyes: Rhodopsin Pathologies in Drosophila

Ana Griciuc, Liviu Aron, Marius Ueffing

54. Müller Glia as a Source of Neuronal Progenitor Cells to Regenerate the Damaged Zebrafish Retina
Craig M. Nelson, David R. Hyde

55. The Genetics of Outer Segment Morphogenesis in Zebrafish
Alison L. Reynolds, Oliver E. Blacque, Breandán N. Kennedy

56. Factor XIIIA Induction in the Retina and Optic Nerve After Optic Nerve Lesion in Goldfish
Kayo Sugitani, Kazuhiro Oogai, Hiroshi Nakashima, Satoru Kato

57. Imaging the Photoreceptor Mosaic with Adaptive Optics: Beyond Counting Cones
Pooja Godara, Melissa Wagner-Schuman, Jungtae Rha, Thomas B. Connor, Kimberly E. Stepien, Joseph Carroll

58. Baseline Imaging Reveals Preexisting Retinal Abnormalities in Mice
Brent A. Bell, Charles Kaul, Mary E. Rayborn, Joe G. Hollyfield

59. Correlation Between Spectral Domain OCT Retinal Nerve Fibre Layer Thickness and Multifocal Pattern Electroretinogram in Advanced Retinitis Pigmentosa
Ieva Sliesoraityte, Eric Troeger, Antje Bernd, Anne Kurtenbach, Eberhart Zrenner

60. Imaging Human Postmortem Eyes with SLO and OCT
Nika Bagheri, Brent A. Bell, Vera L. Bonilha, Joe G. Hollyfield

61. In Vivo Assessment of Rodent Retinal Structure Using Spectral Domain Optical Coherence Tomography
M. Dominik Fischer, Gesine Huber, Francois Paquet-Durand, Peter Humphries, T. Michael Redmond, Christian Grimm, Mathias W. Seeliger

62. Rod Photoreceptor Temporal Properties in Retinal Degenerative Diseases
Yuquan Wen, Kirsten G. Locke, Donald C. Hood, David G. Birch

63. ERG Critical Flicker Frequency Assessment in Humans
Kristen E. Bowles, Timothy W. Kraft

64. Biology of Retinoschisin
Camasamudram Vijayasarathy, Lucia Ziccardi, Paul A. Sieving

65. Transcriptome Analyses to Investigate the Pathogenesis of RNA Splicing Factor Retinitis Pigmentosa
Michael H. Farkas, Greg R. Grant, Eric A. Pierce

66. The Role of the X-linked Retinitis Pigmentosa Protein RP2 in Vesicle Traffic and Cilia Function
Nele Schwarz, Alison J. Hardcastle, Michael E. Cheetham

67.
Caenorhabditis elegans as a Model Organism for Ciliopathies and Related Forms of Photoreceptor Degeneration
Calvin A. Mok, Elise Héon

68. Towards a Pathological Mechanism for IMPDH1-Linked Retinitis Pigmentosa
Dharia A. McGrew, Lizbeth Hedstrom

69. Calpain and Photoreceptor Apoptosis
Anh T. H. Nguyen, Matthew Campbell, Paul F. Kenna, Anna-Sophia Kiang, Lawrence Tam, Marian M. Humphries, Peter Humphries

70. Ceramide Signaling in Retinal Degeneration
Hui Chen, Julie-Thu A. Tran, Richard S. Brush, Anisse Saadi, Abul K. Rahman, Man Yu, Douglas Yasumura, Michael T. Matthes, Kelly Ahern, Haidong Yang, Matthew M. LaVail, Md Nawajes A. Mandal

71. Endoplasmic Reticulum-Associated Degradation (ERAD) of Misfolded Glycoproteins and Mutant P23H Rhodopsin in Photoreceptor Cells
Heike Kroeger, Wei-Chieh Chiang, Jonathan H. Lin

72. Protein Misfolding and Potential Therapeutic Treatments in Inherited Retinopathies
Lawrence C. S. Tam, Anna-Sophia Kiang, Matthew Campbell, James Keaney, G. Jane Farrar, Marian M. Humphries, Paul F. Kenna, Pete Humphries

73. Development of a Cellular Model of Rod Opsin Retinitis Pigmentosa
Matthew Adamowicz, Antonius Song, Samuel Wadsworth, Abraham Scaria, Catherine O’Riordan

74. A Brief Account of Rho GTPases in Retinal Physiology and Pathophysiology
Severin Reinhard Heynen, Omolara O. Ogunshola, Christian Grimm

75. Molecular Clues to Bothnia-Type Retinal Dystrophy
Xiaoqin He, Joel Lobsiger, Achim Stocker

76. A Novel Missense Mutation in Both OPN1LW and OPN1MW Cone Opsin Genes Causes X-Linked Cone Dystrophy (XLCOD5)
Jessica C. Gardner, Tom R. Webb, Naheed Kanuga, Anthony G. Robson, Graham E. Holder, Andrew Stockman, Caterina Ripamonti, Neil D. Ebenezer, Olufunmilola Ogun, Sophie Devery, Genevieve A. Wright, Eamonn R. Maher, Michael E. Cheetham, Anthony T. Moore, Michel Michaelides, Alison J. Hardcastle

77. A Potential Cytosolic Function of Bestrophin-1
Olaf Strauß, Rudgar Neussert, Claudia Müller, Vladimir M. Milenkovic

78. Modeling the Structural Consequences of BEST1 Missense Mutations
Karina E. Guziewicz, Gustavo D. Aguirre, Barbara Zangerl

79. Microglial Activation and Transcriptomic Changes in the Blue Light-Exposed Mouse Retina
Stefanie Ebert, Yana Walczak, Charlotte Remé, Thomas Langmann

80. Overexpression of ROM-1 in the Cone-Dominant Retina
Dibyendu Chakraborty, Shannon M. Conley, Zack Nash, Xi-Qin Ding, Muna I. Naash

81. Analysis of the RPE Sheet in the rd10 Retinal Degeneration Model
Micah A. Chrenek, Nupur Dalal, Christopher Gardner, Hans GrossniklausGrossniklaus, Yi Jiang, Jeffrey H. Boatright, John M. Nickerson

82. Networks Modulating the Retinal Response to Injury: Insights from Microarrays, Expression Genetics, and Bioinformatics
Félix R. Vázquez-Chona, Eldon E. Geisert

83. Mislocalization of Oligomerization-Incompetent RDS is Associated with Mislocalization of Cone Opsins and Cone Transducin
Shannon M. Conley, Dibyendu Chakraborty, Muna I. Naash

84. HSP70 Gene Expression in the Zebrafish Retina After Optic Nerve Injury: A Comparative Study Under Heat Shock Stresses
Chieko Fujikawa, Mikiko Nagashima, Kazuhiro Mawatari, Satoru Kato

85. Restoration of Retinal Development in Vsx2 Deficient Mice by Reduction of Gdf11 Levels
Rosaysela Santos, Jeffry Wu, Jason A. Hamilton, Rita Pinter, Robert Hindges, Anne L. Calof

86. The Different Functions of Norrin
Barbara M. Braunger, Ernst R. Tamm

87. Roles of Homeobox Genes in Retinal Ganglion Cell Differentiation and Axonal Guidance
Qi Zhang, David D. Eisenstat

88. Unraveling the Molecular Mystery of Retinal Pigment Epithelium Phagocytosis
Nora B. Caberoy, Wei Li

89. Isolating Photoreceptor Compartment-Specific Protein Complexes for Subsequent Proteomic Analysis
Gregory H. Grossman, Gayle J. T. Pauer, George Hoppe, Stephanie A. Hagstrom

90. Expression of the Integrin Coreceptor Transglutaminase-2 in the RPE In Vivo and in Culture
Linda Ruggiero, Silvia C. Finnemann

91. On Your Marks… Get Bound… Internalize!
Ah-Lai Law, Emeline F. Nandrot

92. Endo-Lysosome Function in the Retinal Pigment Epithelium in Health and Disease
Aparna Lakkaraju

93. αvβ5 Integrin-Dependent Diurnal Phagocytosis of Shed Photoreceptor Outer Segments by RPE Cells Is Independent of the Integrin Coreceptor Transglutaminase-2
Linda Ruggiero, Zsolt Sarang, Zsuzsa Szondy, Silvia C. Finnemann

94. Trafficking of Presynaptic PMCA Signaling Complexes in Mouse Photoreceptors Requires Cav1.4 α1 Subunits
Wei Xing, Abram Akopian, David Križaj

95. Roles for AMP-Activated Protein Kinase in RPE Cell Function
Suofu Qin

96. Genome-Wide Occupancy Analysis by ChIP-chip and ChIP-Seq
Hong Hao

97. The Bisretinoids of RPE Lipofuscin: A Complex Mixture
Janet R. Sparrow, Kazunori Yamamoto

98. Biochemical Characterization of Cone Cyclic Nucleotide-Gated (CNG) Channel Using the Infrared Fluorescence Detection System
Xi-Qin Ding, Alexander Matveev, Anil Singh, Naoka Komori, Hiroyuki Matsumoto

99. Ras-Associating Domain Proteins: A New Class of Cyclic Nucleotide-Gated Channel Modulators
Vivek K. Gupta, Ammaji Rajala, Raju V. S. Rajala

100. Tulp1 Is Involved in Specific Photoreceptor Protein Transport Pathways
Stephanie A. Hagstrom, Rao F. Watson, Gayle J. T. Pauer, Gregory H. Grossman

101. Potential Cellular Functions of N-Ethylmaleimide Sensitive Factor in the Photoreceptor
Shun-Ping Huang, Cheryl M. Craft

102. Identification of Pigment Epithelium-Derived Factor Receptor (PEDF-R) Antibody Epitopes
Preeti Subramanian, Matthew Rapp, S. Patricia Becerra

103. HCN1 Channels Significantly Shape Retinal Photoresponses
Naoyuki Tanimoto, Arne Brombas, Frank Müller, Mathias W. Seeliger

104. The Role of the P2X7 Receptor in the Retina: Cell Signalling and Dysfunction
Kirstan A. Vessey, Andrew I. Jobling, Ursula Greferath, Erica L. Fletcher

105. A Tale of Two Kinases in Rods and Cones
Shoji Osawa, Ellen R. Weiss

106. Protein Tyrosine Phosphatase 1B: A Novel Molecular Target for Retinal Degenerative Diseases
Devaraj K. Basavarajappa, Vivek K. Gupta, Raju V. S. Rajala

107. Protein Tyrosine-O-Sulfation in Bovine Ocular Tissues
Yogita Kanan, Robert A. Hamilton, Kevin L. Moore, Muayyad R. Al-Ubaidi

108. Erratum to: HCN1 Channels Significantly Shape Retinal Photoresponses
Naoyuki Tanimoto, Arne Brombas, Frank Müller, Mathias W. Seeliger

Nyckelord: Biomedicine, Immunology, Ophthalmology, Geriatrics/Gerontology, Gene Therapy