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Progress in Retinal and Eye Research

Dao-Yi Yu, Stephen J Cringle, Paula K Yu, Chandrakumar Balaratnasingam, Andrew Mehnert, Marinko V Sarunic, Dong An, Er-Ning Su
The central role of the cardiovascular system is to maintain adequate capillary perfusion. The spatially and temporally heterogeneous nature of capillary perfusion has been reported in some organs. However, such heterogeneous perfusion properties have not been sufficiently explored in the retina. Arguably, spatial and temporal heterogeneity of capillary perfusion could be more predominant in the retina than that in other organs. This is because the retina is one of the highest metabolic demand neural tissues yet it has a limited blood supply due to optical requirements...
February 12, 2019: Progress in Retinal and Eye Research
Wolfgang Baehr, Christin Hanke-Gogokhia, Ali Sharif, Michelle Reed, Tiffanie Dahl, Jeanne M Frederick, Guoxin Ying
Photoreceptors are polarized neurons, with very specific subcellular compartmentalization and unique requirements for protein expression and trafficking. Each photoreceptor contains an outer segment, the site of photon capture that initiates vision, an inner segment that houses the biosynthetic machinery and a synaptic terminal for signal transmission to downstream neurons. Outer segments and inner segments are connected by a connecting cilium (CC), the equivalent of a transition zone (TZ) of primary cilia...
December 25, 2018: Progress in Retinal and Eye Research
Arthur A Bergen, Swati Arya, Céline Koster, Matthew G Pilgrim, Dagmara Wiatrek-Moumoulidis, Peter van der Spek, Stefanie M Hauck, Camiel J F Boon, Eszter Emri, Alan J Stewart, Imre Lengyel
Retinal drusen formation is not only a clinical hallmark for the development of age-related macular degeneration (AMD) but also for other disorders, such as Alzheimer's disease and renal diseases. The initiation and growth of drusen is poorly understood. Attention has focused on lipids and minerals, but relatively little is known about the origin of drusen-associated proteins and how they are retained in the space between the basal lamina of the retinal pigment epithelium and the inner collagenous layer space (sub-RPE-BL space)...
December 17, 2018: Progress in Retinal and Eye Research
Paul G McMenamin, Daniel R Saban, Samantha J Dando
In the eye immune defenses must take place in a plethora of differing microenvironments ranging from the corneal and conjunctival epithelia facing the external environment to the pigmented connective tissue of the uveal tract containing smooth muscle, blood vessels and peripheral nerves to the innermost and highly protected neural retina. The extravascular environment of the neural retina, like the brain parenchyma, is stringently controlled to maintain conditions required for neural transmission. The unique physiological nature of the neural retina can be attributed to the blood retinal barriers (BRB) of the retinal vasculature and the retinal pigment epithelium, which both tightly regulate the transport of small molecules and restrict passage of cells and macromolecules from the circulation into the retina in a similar fashion to the blood brain barrier (BBB)...
December 12, 2018: Progress in Retinal and Eye Research
Kyoko Ohno-Matsui, Jost B Jonas
A posterior staphyloma is an outpouching of a circumscribed region of the posterior fundus and has been considered a hallmark of pathologic myopia. Occurring in highly myopic eyes, it is histologically characterized by a relatively abrupt scleral thinning starting at the staphyloma edge, a pronounced de-arrangement of scleral collagen fibrils and a marked choroidal thinning, which is the most marked at the staphyloma edge and which occurs in addition to the axial elongation-associated choroidal thinning. Besides in highly myopic eyes, a posterior staphyloma can be found in non-highly myopic eyes in association with retinitis pigmentosa or localized defects of Bruch's membrane in the cases of which it is not associated with a marked choroidal thinning...
December 8, 2018: Progress in Retinal and Eye Research
Zhongxiao Wang, Chi-Hsiu Liu, Shuo Huang, Jing Chen
The Wnt signaling pathway plays a pivotal role in vascular morphogenesis in various organs including the eye. Wnt ligands and receptors are key regulators of ocular angiogenesis both during the eye development and in vascular eye diseases. Wnt signaling participates in regulating multiple vascular beds in the eye including regression of the hyaloid vessels, and development of structured layers of vasculature in the retina. Loss-of-function mutations in Wnt signaling components cause rare genetic eye diseases in humans such as Norrie disease, and familial exudative vitreoretinopathy (FEVR) with defective ocular vasculature...
December 1, 2018: Progress in Retinal and Eye Research
James S Wolffsohn, Leon N Davies
Presbyopia is a global problem affecting over a billion people worldwide. The prevalence of unmanaged presbyopia is as high as 50% of those over 50 years of age in developing world populations, due to a lack of awareness and accessibility to affordable treatment, and is even as high as 34% in developed countries. Definitions of presbyopia are inconsistent and varied, so we propose a redefinition that states "presbyopia occurs when the physiologically normal age-related reduction in the eye's focusing range reaches a point, when optimally corrected for distance vision, that the clarity of vision at near is insufficient to satisfy an individual's requirements"...
January 2019: Progress in Retinal and Eye Research
Jia Hui Lee, Jiang-Hui Wang, Jinying Chen, Fan Li, Thomas L Edwards, Alex W Hewitt, Guei-Sheung Liu
Many clinical trials using gene therapy have shown significant therapeutic benefits and exceptional safety records. Increasing evidence is verifying the long sought-after promise that gene therapy will genetically 'cure' some severely disabling diseases. In particular, the first gene therapy bioproduct for RPE65-associated Leber's congenital amaurosis, which was approved by the US Food and Drug Administration in 2017, has provided tremendous encouragement to the field of gene therapy. Recent developments in genome editing technologies have significantly advanced our capability to precisely engineer genomes in eukaryotic cells...
January 2019: Progress in Retinal and Eye Research
Christophe Baudouin, Maurizio Rolando, Jose M Benitez Del Castillo, Elisabeth M Messmer, Francisco C Figueiredo, Murat Irkec, Gysbert Van Setten, Marc Labetoulle
Mucins are key actors in tear film quality and tear film stability. Alteration of membrane-bound mucin expression on corneal and conjunctival epithelial cells and/or gel-forming mucin secretion by goblet cells (GCs) promotes in ocular surface diseases and dry eye disease (DED). Changes in the mucin layer may lead to enhanced tear evaporation eventually contributing to tear hyperosmolarity which has been associated with ocular surface inflammation. Inflammatory mediators in turn may have a negative impact on GCs differentiation, proliferation, and mucin secretion...
November 22, 2018: Progress in Retinal and Eye Research
Martin Berry, Zubair Ahmed, Ann Logan
This review addresses issues relating to the survival and axon regeneration of both intrinsically photosensitive retinal ganglion cells (ipRGC) and αRGC, and possible ensuing patterns of functional recovery after optic nerve crush, all of which are broadly relevant to recovery from injury in the central nervous system (CNS) as whole. Although much needs to be clarified about the connectivity, function and patterns of myelination of regenerated CNS axons, the results of recent research on activity-induced αRGC axon regeneration associated with functional restitution have highlighted key focal obstacles to recovery including neurotrophic support, axon misguidance, target recognition failure and dysmyelination...
November 17, 2018: Progress in Retinal and Eye Research
Tina B McKay, Yashar Seyed-Razavi, Chiara E Ghezzi, Gabriela Dieckmann, Thomas J F Nieland, Dana M Cairns, Rachel E Pollard, Pedram Hamrah, David L Kaplan
The cornea is a valuable tissue for studying peripheral sensory nerve structure and regeneration due to its avascularity, transparency, and dense innervation. Somatosensory innervation of the cornea serves to identify changes in environmental stimuli at the ocular surface, thereby promoting barrier function to protect the eye against injury or infection. Due to regulatory demands to screen ocular safety of potential chemical exposure, a need remains to develop functional human tissue models to predict ocular damage and pain using in vitro-based systems to increase throughput and minimize animal use...
November 16, 2018: Progress in Retinal and Eye Research
Sylvia J Gasparini, Sílvia Llonch, Oliver Borsch, Marius Ader
The mammalian retina displays no intrinsic regenerative capacities, therefore retinal degenerative diseases such as age-related macular degeneration (AMD) or retinitis pigmentosa (RP) result in a permanent loss of the light-sensing photoreceptor cells. The degeneration of photoreceptors leads to vision impairment and, in later stages, complete blindness. Several therapeutic strategies have been developed to slow down or prevent further retinal degeneration, however a definitive cure i.e. replacement of the lost photoreceptors, has not yet been established...
November 13, 2018: Progress in Retinal and Eye Research
Kehao Wang, Barbara K Pierscionek
The ability of the human lens to accommodate is mediated by the ciliary muscle and zonule; the manifest optical power changes depend on the shape and material properties of the lens. The latter are difficult to measure with accuracy and, given the dynamic aspects of accommodation and the ageing of cells and tissues, the biomechanics of the lens is neither fixed nor constant. A range of techniques have been developed to measure both ageing trends and spatial variations in the mechanical properties and these have yielded a diverse array of findings and respective conclusions...
November 12, 2018: Progress in Retinal and Eye Research
Inês Laíns, Mari Gantner, Salome Murinello, Jessica A Lasky-Su, Joan W Miller, Martin Friedlander, Deeba Husain
Metabolomics is the qualitative and quantitative assessment of the metabolites (small molecules < 1.5 kDa) in body fluids. The metabolites are the downstream of the genetic transcription and translation processes and also downstream of the interactions with environmental exposures; thus, they are thought to closely relate to the phenotype, especially for multifactorial diseases. In the last decade, metabolomics has been increasingly used to identify biomarkers in disease, and it is currently recognized as a very powerful tool with great potential for clinical translation...
November 10, 2018: Progress in Retinal and Eye Research
Zi-Bing Jin, Mei-Ling Gao, Wen-Li Deng, Kun-Chao Wu, Sunao Sugita, Michiko Mandai, Masayo Takahashi
Cell replacement therapy is a promising treatment for irreversible retinal cell death in diverse diseases, such as age-related macular degeneration (AMD), Stargardt's disease, retinitis pigmentosa (RP) and glaucoma. These diseases are all characterized by the degeneration of one or two retinal cell types that cannot regenerate spontaneously in humans. Aberrant retinal pigment epithelial (RPE) cells can be observed through optical coherence tomography (OCT) in AMD patients. In RP patients, the morphological and functional abnormalities of RPE and photoreceptor layers are caused by a genetic abnormality...
November 9, 2018: Progress in Retinal and Eye Research
Jorge Ruiz-Medrano, Javier A Montero, Ignacio Flores-Moreno, Luis Arias, Alfredo García-Layana, José M Ruiz-Moreno
Myopia is a highly frequent ocular disorder worldwide and pathologic myopia is the 4th most common cause of irreversible blindness in developed countries. Pathologic myopia is especially common in East Asian countries. Ocular alterations associated with pathologic myopia, especially those involving the macular area-defined as myopic maculopathy-are the leading causes of vision loss in patients with pathologic myopia. High myopia is defined as the presence of a highly negative refractive error (>-6 to -8 diopters) in the context of eye elongation (26-26...
November 1, 2018: Progress in Retinal and Eye Research
Sylvia B Smith, Jing Wang, Xuezhi Cui, Barbara A Mysona, Jing Zhao, Kathryn E Bollinger
Retinal degenerative diseases are major causes of untreatable blindness worldwide and efficacious treatments for these diseases are sorely needed. A novel target for treatment of retinal disease is the transmembrane protein Sigma 1 Receptor (Sig1R). This enigmatic protein is an evolutionary isolate with no known homology to any other protein. Sig1R was originally thought to be an opioid receptor. That notion has been dispelled and more recent pharmacological and molecular studies suggest that it is a pluripotent modulator with a number of biological functions, many of which are relevant to retinal disease...
November 2018: Progress in Retinal and Eye Research
Enrico Borrelli, David Sarraf, K Bailey Freund, Srinivas R Sadda
The recent introduction of optical coherence tomography angiography (OCTA) has remarkably expanded our knowledge of the choroid through in vivo investigation of the anatomical and pathological features of this important vascular layer. New insights elucidating the morphological features of the choroid, in both physiological and pathological conditions, indicate that this vascular structure plays a crucial role in many chorioretinal disorders. In this article, a review of the salient histological and anatomical features of the choroid, essential for the proper interpretation of in vivo imaging, is followed by a discussion of the fundamental principles of OCTA and the application of this advanced imaging modality to study and understand the choroid...
November 2018: Progress in Retinal and Eye Research
Sophia Wienbar, Gregory W Schwartz
Retinal ganglion cells (RGCs) were one of the first classes of sensory neurons to be described in terms of a receptive field (RF). Over the last six decades, our understanding of the diversity of RGC types and the nuances of their response properties has grown exponentially. We will review the current understanding of RGC RFs mostly from studies in mammals, but including work from other vertebrates as well. We will argue for a new paradigm that embraces the fluidity of RGC RFs with an eye toward the neuroethology of vision...
November 2018: Progress in Retinal and Eye Research
Jonathan B Lin, Rajendra S Apte
Retinal degenerative diseases are a major cause of morbidity in modern society because visual impairment significantly decreases the quality of life of patients. A significant challenge in treating retinal degenerative diseases is their genetic and phenotypic heterogeneity. However, despite this diversity, many of these diseases share a common endpoint involving death of light-sensitive photoreceptors. Identifying common pathogenic mechanisms that contribute to photoreceptor death in these diverse diseases may lead to a unifying therapy for multiple retinal diseases that would be highly innovative and address a great clinical need...
November 2018: Progress in Retinal and Eye Research
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