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Towards a better understanding of non-exudative choroidal and macular neovascularization Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2022-08-13 Riccardo Sacconi, Serena Fragiotta, David Sarraf, SriniVas R. Sadda, K. Bailey Freund, Mariacristina Parravano, Giulia Corradetti, Diogo Cabral, Vittorio Capuano, Alexandra Miere, Eliana Costanzo, Francesco Bandello, Eric Souied, Giuseppe Querques
Non-exudative macular and choroidal neovascularization (MNV and CNV) usually refers to the entity of treatment-naïve type 1 neovascularization in the absence of associated signs of exudation. Histopathological studies, dating back in the early 70s, identified the presence of non-exudative MNV, but the first clinical report of this finding was in the late 90s using indocyanine green angiography in eyes
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Quantitative approaches in multimodal fundus imaging: State of the art and future perspectives Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2022-08-04 Alessandro Arrigo, Emanuela Aragona, Maurizio Battaglia Parodi, Francesco Bandello
When it first appeared, multimodal fundus imaging revolutionized the diagnostic workup and provided extremely useful new insights into the pathogenesis of fundus diseases. The recent addition of quantitative approaches has further expanded the amount of information that can be obtained. In spite of the growing interest in advanced quantitative metrics, the scientific community has not reached a stable
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Non-vasogenic cystoid maculopathies Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2022-08-01 Alain Gaudric, Isabelle Audo, Catherine Vignal, Aude Couturier, Élise Boulanger-Scemama, Ramin Tadayoni, Salomon Yves Cohen
Besides cystoid macular edema due to a blood-retinal barrier breakdown, another type of macular cystoid spaces referred to as non-vasogenic cystoid maculopathies (NVCM) may be detected on optical coherence tomography but not on fluorescein angiography. Various causes may disrupt retinal cell cohesion or impair retinal pigment epithelium (RPE) and Müller cell functions in the maintenance of retinal
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Neuroimmune crosstalk in the cornea: The role of immune cells in corneal nerve maintenance during homeostasis and inflammation Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2022-07-19 Mengliang Wu, Lisa J. Hill, Laura E. Downie, Holly R. Chinnery
In the cornea, resident immune cells are in close proximity to sensory nerves, consistent with their important roles in the maintenance of nerves in both homeostasis and inflammation. Using in vivo confocal microscopy in humans, and ex vivo immunostaining and fluorescent reporter mice to visualize corneal sensory nerves and immune cells, remarkable progress has been made to advance our understanding
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RNA-targeting strategies as a platform for ocular gene therapy Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2022-07-13 Satheesh Kumar, Lewis E. Fry, Jiang-Hui Wang, Keith R. Martin, Alex W. Hewitt, Fred K. Chen, Guei-Sheung Liu
Genetic medicine is offering hope as new therapies are emerging for many previously untreatable diseases. The eye is at the forefront of these advances, as exemplified by the approval of Luxturna® by the United States Food and Drug Administration (US FDA) in 2017 for the treatment of one form of Leber Congenital Amaurosis (LCA), an inherited blindness. Luxturna® was also the first in vivo human gene
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Bioengineering approaches for modelling retinal pathologies of the outer blood-retinal barrier Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2022-07-13 Blanca Molins, Marina Mesquida, Alfredo Adan
Alterations of the junctional complex of the outer blood-retinal barrier (oBRB), which is integrated by the close interaction of the retinal pigment epithelium, the Bruch's membrane, and the choriocapillaris, contribute to the loss of neuronal signalling and subsequent vision impairment in several retinal inflammatory disorders such as age-related macular degeneration and diabetic retinopathy. Reductionist
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Cell fate decisions, transcription factors and signaling during early retinal development Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2022-07-08 Raven Diacou, Prithviraj Nandigrami, Andras Fiser, Wei Liu, Ruth Ashery-Padan, Ales Cvekl
The development of the vertebrate eyes is a complex process starting from anterior-posterior and dorso-ventral patterning of the anterior neural tube, resulting in the formation of the eye field. Symmetrical separation of the eye field at the anterior neural plate is followed by two symmetrical evaginations to generate a pair of optic vesicles. Next, reciprocal invagination of the optic vesicles with
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Zebrafish and inherited photoreceptor disease: Models and insights Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2022-07-08 Nicole C.L. Noel, W.Ted Allison, Ian M. MacDonald, Jennifer C. Hocking
Photoreceptor dysfunctions and degenerative diseases are significant causes of vision loss in patients, with few effective treatments available. Targeted interventions to prevent or reverse photoreceptor-related vision loss are not possible without a thorough understanding of the underlying mechanism leading to disease, which is exceedingly difficult to accomplish in the human system. Cone diseases
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Retinal vessel diameters and function in cardiovascular risk and disease Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2022-06-25 Henner Hanssen, Lukas Streese, Walthard Vilser
In the last two decades evidence has gradually accumulated suggesting that the eye may be a unique window for cardiovascular risk stratification based on the assessment of subclinical damage of retinal microvascular structure and function. This can be facilitated by non-invasive analysis of static retinal vessel diameters and dynamic recording of flicker light-induced and endothelial function-related
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The retinal pigmentation pathway in human albinism: Not so black and white Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2022-06-18 Reinier Bakker, Philip E. Wagstaff, Charlotte C. Kruijt, Eszter Emri, Clara D.M. van Karnebeek, Michael B. Hoffmann, Brian P. Brooks, Camiel J.F. Boon, Lluis Montoliu, Maria M. van Genderen, Arthur A. Bergen
Albinism is a pigment disorder affecting eye, skin and/or hair. Patients usually have decreased melanin in affected tissues and suffer from severe visual abnormalities, including foveal hypoplasia and chiasmal misrouting. Combining our data with those of the literature, we propose a single functional genetic retinal signalling pathway that includes all 22 currently known human albinism disease genes
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Caveolar and non-Caveolar Caveolin-1 in ocular homeostasis and disease Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2022-06-18 Eric N. Enyong, Jami M. Gurley, Michael L. De Ieso, W. Daniel Stamer, Michael H. Elliott
Caveolae, specialized plasma membrane invaginations present in most cell types, play important roles in multiple cellular processes including cell signaling, lipid uptake and metabolism, endocytosis and mechanotransduction. They are found in almost all cell types but most abundant in endothelial cells, adipocytes and fibroblasts. Caveolin-1 (Cav1), the signature structural protein of caveolae was the
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Optogenetics for visual restoration: From proof of principle to translational challenges Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2022-06-09 Moritz Lindner, Michael J. Gilhooley, Steven Hughes, Mark W. Hankins
Degenerative retinal disorders are a diverse family of diseases commonly leading to irreversible photoreceptor death, while leaving the inner retina relatively intact. Over recent years, innovative gene replacement therapies aiming to halt the progression of certain inherited retinal disorders have made their way into clinics. By rendering surviving retinal neurons light sensitive optogenetic gene
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Corneal stromal repair and regeneration Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2022-05-29 Rajiv R. Mohan, Duraisamy Kempuraj, Sharon D'Souza, Arkasubhra Ghosh
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Insights into the developing fovea revealed by imaging Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2022-05-17 Ye He, Xi Chen, Irena Tsui, Lejla Vajzovic, Srinivas R. Sadda
Early development of the fovea has been documented by histological studies over the past few decades. However, structural distortion due to sample processing and the paucity of high-quality post-mortem tissue has limited the effectiveness of this approach. With the continuous progress in high-resolution non-invasive imaging technology, most notably optical coherence tomography (OCT) and OCT angiography
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The genetic basis for adult onset glaucoma: Recent advances and future directions Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2022-05-17 Zhenxun Wang, Janey L. Wiggs, Tin Aung, Anthony P. Khawaja, Chiea Chuen Khor
Glaucoma, a diverse group of eye disorders that results in the degeneration of retinal ganglion cells, is the world's leading cause of irreversible blindness. Apart from age and ancestry, the major risk factor for glaucoma is increased intraocular pressure (IOP). In primary open-angle glaucoma (POAG), the anterior chamber angle is open but there is resistance to aqueous outflow. In primary angle-closure
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Gene-independent therapeutic interventions to maintain and restore light sensitivity in degenerating photoreceptors Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2022-05-11 Marta Zuzic, Johannes Striebel, Julia S. Pawlick, Kritika Sharma, Frank G. Holz, Volker Busskamp
Neurodegenerative retinal diseases are a prime cause of blindness in industrialized countries. In many cases, there are no therapeutic treatments, although they are essential to improve patients' quality of life. A set of disease-causing genes, which primarily affect photoreceptors, has already been identified and is of major interest for developing gene therapies. Nevertheless, depending on the nature
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A novel glaucoma approach: Stem cell regeneration of the trabecular meshwork Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2022-04-06 Sara J. Coulon, Joel S. Schuman, Yiqin Du, Mohammad Reza Bahrani Fard, C. Ross Ethier, W. Daniel Stamer
Glaucoma is the leading cause of global irreversible blindness, necessitating research for new, more efficacious treatment options than currently exist. Trabecular meshwork (TM) cells play an important role in the maintenance and function of the aqueous outflow pathway, and studies have found that there is decreased cellularity of the TM in glaucoma. Regeneration of the TM with stem cells has been
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The physiological and pathophysiological roles of the autophagy lysosomal system in the conventional aqueous humor outflow pathway: More than cellular clean up Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2022-04-01 Myoung Sup Shim, Paloma B. Liton
During the last few years, the autophagy lysosomal system is emerging as a central cellular pathway with roles in survival, acting as a housekeeper and stress response mechanism. Studies by our and other labs suggest that autophagy might play an essential role in maintaining aqueous humor outflow homeostasis, and that malfunction of autophagy in outflow pathway cells might predispose to ocular hypertension
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Detecting glaucoma with only OCT: Implications for the clinic, research, screening, and AI development Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2022-02-23 Donald C. Hood, Sol La Bruna, Emmanouil Tsamis, Kaveri A. Thakoor, Anvit Rai, Ari Leshno, Carlos G.V. de Moraes, George A. Cioffi, Jeffrey M. Liebmann
A method for detecting glaucoma based only on optical coherence tomography (OCT) is of potential value for routine clinical decisions, for inclusion criteria for research studies and trials, for large-scale clinical screening, as well as for the development of artificial intelligence (AI) decision models. Recent work suggests that the OCT probability (p-) maps, also known as deviation maps, can play
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Challenges in the diagnosis and management of vitreoretinal lymphoma – Clinical and basic approaches Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2022-02-21 Hiroshi Takase, Ayako Arai, Yuko Iwasaki, Ayano Imai, Toshikage Nagao, Masahiko Kawagishi, Tomoka Ishida, Manabu Mochizuki
Vitreoretinal lymphoma (VRL) is a subtype of diffuse large B-cell lymphoma and is sight- and life-threatening in the vast majority of patients. Lymphoma cells infiltrate the vitreous body and/or subretinal space and exhibit clinical signs of vitreous opacities and creamy white subretinal lesions. Although the intraocular signs can serve as clues to suspect VRL, they are nonspecific and may be misdiagnosed
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It is time for a moonshot to find “Cures” for diabetic retinal disease Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2022-02-11 S. Robert Levine, Przemyslaw Sapieha, Sanjoy Dutta, Jennifer K. Sun, Thomas W. Gardner
Diabetic retinal disease (DRD), the most common complication of diabetes and a leading cause of blindness in working age individuals, is now understood to be a form of sensory neuropathy or neurovascular degeneration. Current treatments are focused on advanced vision-threatening disease and a single molecular target, vascular endothelial growth factor, has an approved therapy. We trace the evolution
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Retinal cadherins and the retinal cadherinopathies: Current concepts and future directions Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2022-01-21 Imran H. Yusuf, Andrew Garrett, Robert E. MacLaren, Peter Charbel Issa
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Metastatic uveal melanoma: The final frontier Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2022-01-06 Elina S. Rantala, Micaela M. Hernberg, Sophie Piperno-Neumann, Hans E. Grossniklaus, Tero T. Kivelä
Treatment of primary intraocular uveal melanoma has developed considerably, its driver genes are largely unraveled, and the ways to assess its risk for metastases are very precise, being based on an international staging system and genetic data. Unfortunately, the risk of distant metastases, which emerge in approximately one half of all patients, is unaltered. Metastases are the leading single cause
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The impact of sensory neuropathy and inflammation on epithelial wound healing in diabetic corneas Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2022-01-04 Fu-shin X. Yu, Patrick S.Y. Lee, Lingling Yang, Nan Gao, Yangyang Zhang, Alexander V. Ljubimov, Ellen Yang, Qingjun Zhou, Lixin Xie
Diabetic peripheral neuropathy (DPN) is the most common complication of diabetes, with several underlying pathophysiological mechanisms, some of which are still uncertain. The cornea is an avascular tissue and sensitive to hyperglycemia, resulting in several diabetic corneal complications including delayed epithelial wound healing, recurrent erosions, neuropathy, loss of sensitivity, and tear film
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Molecular basis of rod and cone differences Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2021-12-31 Satoru Kawamura, Shuji Tachibanaki
In the vertebrate retina, rods and cones both detect light, but they are different in functional aspects such as light sensitivity and time resolution, for example, and in some of cell biological aspects. For functional aspects, both photoreceptors are known to share a common mechanism, phototransduction cascade, consisting of a series of enzyme reactions to convert a photon-capture signal to an electrical
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Dynamic lipid turnover in photoreceptors and retinal pigment epithelium throughout life Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2021-12-29 Dominik Lewandowski, Christopher L. Sander, Aleksander Tworak, Fangyuan Gao, Qianlan Xu, Dorota Skowronska-Krawczyk
The retinal pigment epithelium-photoreceptor interphase is renewed each day in a stunning display of cellular interdependence. While photoreceptors use photosensitive pigments to convert light into electrical signals, the RPE supports photoreceptors in their function by phagocytizing shed photoreceptor tips, regulating the blood retina barrier, and modulating inflammatory responses, as well as regenerating
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Structure and function of ABCA4 and its role in the visual cycle and Stargardt macular degeneration Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2021-12-23 Robert S. Molday, Fabian A. Garces, Jessica Fernandes Scortecci, Laurie L. Molday
ABCA4 is a member of the superfamily of ATP-binding cassette (ABC) transporters that is preferentially localized along the rim region of rod and cone photoreceptor outer segment disc membranes. It uses the energy from ATP binding and hydrolysis to transport N-retinylidene-phosphatidylethanolamine (N-Ret-PE), the Schiff base adduct of retinal and phosphatidylethanolamine, from the lumen to the cytoplasmic
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Retinal ciliopathies through the lens of Bardet-Biedl Syndrome: Past, present and future Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2021-12-18 Bharatendu Chandra, Moon Ley Tung, Ying Hsu, Todd Scheetz, Val C. Sheffield
The primary cilium is a highly specialized and evolutionary conserved organelle in eukaryotes that plays a significant role in cell signaling and trafficking. Over the past few decades tremendous progress has been made in understanding the physiology of cilia and the underlying pathomechanisms of various ciliopathies. Syndromic ciliopathies consist of a group of disorders caused by ciliary dysfunction
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Bacterial keratitis: identifying the areas of clinical uncertainty Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2021-12-13 Stephen Tuft, Tobi F. Somerville, Ji-Peng Olivia Li, Timothy Neal, Surjo De, Malcolm J. Horsburgh, Joanne L. Fothergill, Daniel Foulkes, Stephen Kaye
Bacterial keratitis is a common corneal infection that is treated with topical antimicrobials. By the time of presentation there may already be severe visual loss from corneal ulceration and opacity, which may persist despite treatment. There are significant differences in the associated risk factors and the bacterial isolates between high income and low- or middle-income countries, so that general
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Diabetic macular ischaemia- a new therapeutic target? Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2021-12-11 Chui Ming Gemmy Cheung, Amani Fawzi, Kelvin YC. Teo, Hisashi Fukuyama, Sagnik Sen, Wei-Shan Tsai, Sobha Sivaprasad
Diabetic macular ischaemia (DMI) is traditionally defined and graded based on the angiographic evidence of an enlarged and irregular foveal avascular zone. However, these anatomical changes are not surrogate markers for visual impairment. We postulate that there are vascular phenotypes of DMI based on the relative perfusion deficits of various retinal capillary plexuses and choriocapillaris. This review
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Trustworthy AI: Closing the gap between development and integration of AI systems in ophthalmic practice Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2021-12-10 Cristina González-Gonzalo, Eric F. Thee, Caroline C.W. Klaver, Aaron Y. Lee, Reinier O. Schlingemann, Adnan Tufail, Frank Verbraak, Clara I. Sánchez
An increasing number of artificial intelligence (AI) systems are being proposed in ophthalmology, motivated by the variety and amount of clinical and imaging data, as well as their potential benefits at the different stages of patient care. Despite achieving close or even superior performance to that of experts, there is a critical gap between development and integration of AI systems in ophthalmic
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Clusterin, other extracellular chaperones, and eye disease Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2021-12-10 Mark R. Wilson, Sandeep Satapathy, Shinwu Jeong, M. Elizabeth Fini
Proteostasis refers to all the processes that maintain the correct expression level, location, folding and turnover of proteins, essential to organismal survival. Both inside cells and in body fluids, molecular chaperones play key roles in maintaining proteostasis. In this article, we focus on clusterin, the first-recognized extracellular mammalian chaperone, and its role in diseases of the eye. Clusterin
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Regulation of epigenetic homeostasis in uveal melanoma and retinoblastoma Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2021-12-01 Peiwei Chai, Ruobing Jia, Yongyun Li, Chuandi Zhou, Xiang Gu, Ludi Yang, Hanhan Shi, Hao Tian, Huimin Lin, Jie Yu, Ai Zhuang, Shengfang Ge, Renbing Jia, Xianqun Fan
Uveal melanoma (UM) and retinoblastoma (RB), which cause blindness and even death, are the most frequently observed primary intraocular malignancies in adults and children, respectively. Epigenetic studies have shown that changes in the epigenome contribute to the rapid progression of both UM and RB following classic genetic changes. The loss of epigenetic homeostasis plays an important role in oncogenesis
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Inherited retinal diseases: Linking genes, disease-causing variants, and relevant therapeutic modalities Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2021-11-25 Nina Schneider, Yogapriya Sundaresan, Prakadeeswari Gopalakrishnan, Avigail Beryozkin, Mor Hanany, Erez Y. Levanon, Eyal Banin, Shay Ben-Aroya, Dror Sharon
Inherited retinal diseases (IRDs) are a clinically complex and heterogenous group of visual impairment phenotypes caused by pathogenic variants in at least 277 nuclear and mitochondrial genes, affecting different retinal regions, and depleting the vision of affected individuals. Genes that cause IRDs when mutated are unique by possessing differing genotype-phenotype correlations, varying inheritance
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Paradox of complex diversity: Challenges in the diagnosis and management of bacterial keratitis Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2021-11-20 Rohan Bir Singh, Sujata Das, James Chodosh, Namrata Sharma, Michael E. Zegans, Regis P. Kowalski, Vishal Jhanji
Bacterial keratitis continues to be one of the leading causes of corneal blindness in the developed as well as the developing world, despite swift progress since the dawn of the “anti-biotic era”. Although, we have expeditiously developed our understanding about the different causative organisms and associated pathology leading to keratitis, extensive gaps in knowledge continue to dampen the efforts
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Corrigendum to “Splicing mutations in inherited retinal diseases” [Prog. Retin. Eye Res. 80 (2021) 100874] Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2021-11-19 Nicole Weisschuh, Elena Buena-Atienza, Bernd Wissinger
Abstract not available
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Age and intraocular pressure in murine experimental glaucoma Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2021-11-18 Johnny Di Pierdomenico, Delaney C.M. Henderson, Sara Giammaria, Victoria L. Smith, Aliénor J. Jamet, Corey A. Smith, Michele L. Hooper, Balwantray C. Chauhan
Age and intraocular pressure (IOP) are the two most important risk factors for the development and progression of open-angle glaucoma. While IOP is commonly considered in models of experimental glaucoma (EG), most studies use juvenile or adult animals and seldom older animals which are representative of the human disease. This paper provides a concise review of how retinal ganglion cell (RGC) loss
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Tilted disc syndrome (TDS): New hypotheses for posterior segment complications and their implications in other retinal diseases Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2021-11-17 Salomon Yves Cohen, Catherine Vignal-Clermont, Liem Trinh, Kyoko Ohno-Matsui
Tilted disc syndrome (TDS) is considered a congenital anomaly due to a delayed closure of the embryonic fissure. It is characterized by an oblique orientation of the axis of the optic disc, associated with other posterior pole anomalies such as inferior crescent, situs inversus and inferior staphyloma. The aim of this review was to summarize the data supporting the current hypotheses for the pathogenesis
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Assessment and management of retinopathy of prematurity in the era of anti-vascular endothelial growth factor (VEGF) Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2021-11-09 Andrew SH. Tsai, Hung-Da Chou, Xiao Chun Ling, Tala Al-Khaled, Nita Valikodath, Emily Cole, Vivien L. Yap, Michael F. Chiang, R.V. Paul Chan, Wei-Chi Wu
The incidence of retinopathy of prematurity (ROP) continues to rise due to the improved survival of very low birth weight infants in developed countries. This epidemic is also fueled by increased survival of preterm babies with variable use of oxygen and a lack of ROP awareness and screening services in resource-limited regions. Improvements in technology and a basic understanding of the disease pathophysiology
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Reticular pseudodrusen: A critical phenotype in age-related macular degeneration Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2021-11-06 Zhichao Wu, Erica L. Fletcher, Himeesh Kumar, Ursula Greferath, Robyn H. Guymer
Reticular pseudodrusen (RPD), or subretinal drusenoid deposits (SDD), refer to distinct lesions that occur in the subretinal space. Over the past three decades, their presence in association with age-related macular degeneration (AMD) has become increasingly recognized, especially as RPD have become more easily distinguished with newer clinical imaging modalities. There is also an increasing appreciation
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Anterior pituitary, sex hormones, and keratoconus: Beyond traditional targets Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2021-11-02 Dimitrios Karamichos, Paulina Escandon, Brenda Vasini, Sarah E. Nicholas, Lyly Van, Deanna H. Dang, Rebecca L. Cunningham, Kamran M. Riaz
"The Diseases of the Horny-coat of The Eye", known today as keratoconus, is a progressive, multifactorial, non-inflammatory ectatic corneal disorder that is characterized by steepening (bulging) and thinning of the cornea, irregular astigmatism, myopia, and scarring that can cause devastating vision loss. The significant socioeconomic impact of the disease is immeasurable, as patients with keratoconus
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The role of dark adaptation in understanding early AMD Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2021-10-06 Ian J. Murray, Elena Rodrigo-Diaz, Jeremiah M.F. Kelly, Tariq M. Aslam, Humza J. Tahir, David Carden, Laura Patryas, Neil R.A. Parry
The main aim of the paper is to discuss current knowledge on how Age Related Macular Degeneration (AMD) affects Dark Adaptation (DA). The paper is divided into three parts. Firstly, we outline some of the molecular mechanisms that control DA. Secondly, we review the psychophysical issues and the corresponding analytical techniques. Finally, we characterise the link between slowed DA and the morphological
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Recent understanding of binocular vision in the natural environment with clinical implications Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2021-10-06 T. Rowan Candy, Lawrence K. Cormack
Technological advances in recent decades have allowed us to measure both the information available to the visual system in the natural environment and the rich array of behaviors that the visual system supports. This review highlights the tasks undertaken by the binocular visual system in particular and how, for much of human activity, these tasks differ from those considered when an observer fixates
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Retinal pigment epithelium 65 kDa protein (RPE65): An update Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2021-10-02 Philip D. Kiser
Vertebrate vision critically depends on an 11-cis-retinoid renewal system known as the visual cycle. At the heart of this metabolic pathway is an enzyme known as retinal pigment epithelium 65 kDa protein (RPE65), which catalyzes an unusual, possibly biochemically unique, reaction consisting of a coupled all-trans-retinyl ester hydrolysis and alkene geometric isomerization to produce 11-cis-retinol
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Protection against corneal hyperosmolarity with soft-contact-lens wear Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2021-09-29 Young Hyun Kim, Thien Nguyen, Meng C. Lin, Cheng-Chun Peng, Clayton J. Radke
Hyperosmotic tear stimulates human corneal nerve endings, activates ocular immune response, and elicits dry-eye symptoms. A soft contact lens (SCL) covers the cornea preventing it from experiencing direct tear evaporation and the resulting blink-periodic salinity increases. For the cornea to experience hyperosmolarity due to tear evaporation, salt must transport across the SCL to the post-lens tear
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Regenerative therapy for the Cornea Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2021-09-14 Ajay Kumar, Hongmin Yun, Martha L. Funderburgh, Yiqin Du
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A reinterpretation of critical flicker-frequency (CFF) data reveals key details about light adaptation and normal and abnormal visual processing Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2021-09-08 Andrew T. Rider, G. Bruce Henning, Andrew Stockman
Our ability to see flicker has an upper frequency limit above which flicker is invisible, known as the “critical flicker frequency” (CFF), that typically grows with light intensity (I). The relation between CFF and I, the focus of nearly 200 years of research, is roughly logarithmic, i.e., CFF ∝ log(I)—a relation called the Ferry-Porter law. However, why this law should occur, and how it relates to
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Full-field stimulus testing: Role in the clinic and as an outcome measure in clinical trials of severe childhood retinal disease Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2021-08-28 Alejandro J. Roman, Artur V. Cideciyan, Vivian Wu, Alexandra V. Garafalo, Samuel G. Jacobson
Disease mechanisms have become better understood in previously incurable forms of early-onset severe retinal dystrophy, such as Leber congenital amaurosis (LCA). This has led to novel treatments and clinical trials that have shown some success. Standard methods to measure vision were difficult if not impossible to perform in severely affected patients with low vision and nystagmus. To meet the need
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Of men and mice: Human X-linked retinoschisis and fidelity in mouse modeling Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2021-08-11 Camasamudram Vijayasarathy, Sheik Pran Babu Sardar Pasha, Paul A. Sieving
X-linked Retinoschisis (XLRS) is an early-onset transretinal dystrophy, often with a prominent macular component, that affects males and generally spares heterozygous females because of X-linked recessive inheritance. It results from loss-of-function RS1 gene mutations on the X-chromosome. XLRS causes bilateral reduced acuities from young age, and on clinical exam and by ocular coherence tomography
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Molecular regulation of neuroinflammation in glaucoma: Current knowledge and the ongoing search for new treatment targets Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2021-08-01 Gülgün Tezel
Neuroinflammation relying on the inflammatory responses of glial cells has emerged as an impactful component of the multifactorial etiology of neurodegeneration in glaucoma. It has become increasingly evident that despite early adaptive and reparative features of glial responses, prolonged reactivity of the resident glia, along with the peripheral immune cells, create widespread toxicity to retinal
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Choriocapillaris: Fundamentals and advancements Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2021-07-19 Raphael Lejoyeux, Jennifer Benillouche, Joshua Ong, Marie-Hélène Errera, Ethan A. Rossi, Sumit R. Singh, Kunal K. Dansingani, Susana da Silva, Debasish Sinha, José-Alain Sahel, K. Bailey Freund, SriniVas R. Sadda, Gerard A. Lutty, Jay Chhablani
The choriocapillaris is the innermost structure of the choroid that directly nourishes the retinal pigment epithelium and photoreceptors. This article provides an overview of its hemovasculogenesis development to achieve its final architecture as a lobular vasculature, and also summarizes the current histological and molecular knowledge about choriocapillaris and its dysfunction. After describing the
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Exploring the choroidal vascular labyrinth and its molecular and structural roles in health and disease Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2021-07-17 J. Brinks, E.H.C. van Dijk, I. Klaassen, R.O. Schlingemann, S.M. Kielbasa, E. Emri, P.H.A. Quax, A.A. Bergen, O.C. Meijer, C.J.F. Boon
The choroid is a key player in maintaining ocular homeostasis and plays a role in a variety of chorioretinal diseases, many of which are poorly understood. Recent advances in the field of single-cell RNA sequencing have yielded valuable insights into the properties of choroidal endothelial cells (CECs). Here, we review the role of the choroid in various physiological and pathophysiological mechanisms
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Approaches for corneal endothelium regenerative medicine Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2021-07-06 Pere Català, Gilles Thuret, Heli Skottman, Jodhbir S. Mehta, Mohit Parekh, Sorcha Ní Dhubhghaill, Rob W.J. Collin, Rudy M.M.A. Nuijts, Stefano Ferrari, Vanessa L.S. LaPointe, Mor M. Dickman
The state of the art therapy for treating corneal endothelial disease is transplantation. Advances in the reproducibility and accessibility of surgical techniques are increasing the number of corneal transplants, thereby causing a global deficit of donor corneas and leaving 12.7 million patients with addressable visual impairment. Approaches to regenerate the corneal endothelium offer a solution to
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AI-based monitoring of retinal fluid in disease activity and under therapy Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2021-06-22 Ursula Schmidt-Erfurth, Gregor S. Reiter, Sophie Riedl, Philipp Seeböck, Wolf-Dieter Vogl, Barbara A. Blodi, Amitha Domalpally, Amani Fawzi, Yali Jia, David Sarraf, Hrvoje Bogunović
Retinal fluid as the major biomarker in exudative macular disease is accurately visualized by high-resolution three-dimensional optical coherence tomography (OCT), which is used world-wide as a diagnostic gold standard largely replacing clinical examination. Artificial intelligence (AI) with its capability to objectively identify, localize and quantify fluid introduces fully automated tools into OCT
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The two-faced effects of nerves and neuropeptides in corneal diseases Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2021-06-07 Romina Mayra Lasagni Vitar, Paolo Rama, Giulio Ferrari
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Detecting retinal cell stress and apoptosis with DARC: Progression from lab to clinic Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2021-06-05 Maria Francesca Cordeiro, Daniel Hill, Radhika Patel, Paolo Corazza, John Maddison, Saad Younis
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Müller cells and astrocytes in tractional macular disorders Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2021-06-05 Andreas Bringmann, Jan Darius Unterlauft, Thomas Barth, Renate Wiedemann, Matus Rehak, Peter Wiedemann
Tractional deformations of the fovea mainly arise from an anomalous posterior vitreous detachment and contraction of epiretinal membranes, and also occur in eyes with cystoid macular edema or high myopia. Traction to the fovea may cause partial- and full-thickness macular defects. Partial-thickness defects are foveal pseudocysts, macular pseudoholes, and tractional, degenerative, and outer lamellar
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Early and late stage gene therapy interventions for inherited retinal degenerations Prog. Retin. Eye. Res. (IF 19.704) Pub Date : 2021-05-29 Catherine Botto, Marco Rucli, Müge Defne Tekinsoy, Juliette Pulman, José-Alain Sahel, Deniz Dalkara