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https://www.readbyqxmd.com/read/28534947/research-and-progress-on-clc%C3%A2-2-review
#1
Hongwei Wang, Minghui Xu, Qingjie Kong, Peng Sun, Fengyun Yan, Wenying Tian, Xin Wang
Chloride channel 2 (ClC-2) is one of the nine mammalian members of the ClC family. The present review discusses the molecular properties of ClC‑2, including CLCN2, ClC‑2 promoter and the structural properties of ClC‑2 protein; physiological properties; functional properties, including the regulation of cell volume. The effects of ClC‑2 on the digestive, respiratory, circulatory, nervous and optical systems are also discussed, in addition to the mechanisms involved in the regulation of ClC‑2. The review then discusses the diseases associated with ClC‑2, including degeneration of the retina, Sjögren's syndrome, age‑related cataracts, degeneration of the testes, azoospermia, lung cancer, constipation, repair of impaired intestinal mucosa barrier, leukemia, cystic fibrosis, leukoencephalopathy, epilepsy and diabetes mellitus...
May 18, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28534762/aiding-the-eye-watching-the-brain-james-weiland-ieee-fellow-explores-the-unique-challenges-of-retinal-prostheses
#2
Kristina Grifantini
The retina is a sophisticated neural network that provides humans with high-resolution vision. And for those who suffer from retinal disease or deterioration, particularly age-related macular degeneration (the leading cause of blindness among people over the age of 50 in the United States), a better understanding of how to stimulate the retina or completely override its path to the area of the brain that processes vision may offer hope to restore sight.
May 2017: IEEE Pulse
https://www.readbyqxmd.com/read/28532361/electrical-stimulation-of-the-retina-to-produce-artificial-vision
#3
James D Weiland, Steven T Walston, Mark S Humayun
Retinal prostheses aim to restore vision to blind individuals suffering from retinal diseases such as retinitis pigmentosa and age-related macular degeneration. These devices function by electrically stimulating surviving retinal neurons, whose activation is interpreted by the brain as a visual percept. Many prostheses are currently under development. They are categorized as epiretinal, subretinal, and suprachoroidal prostheses on the basis of the placement of the stimulating microelectrode array. Each can activate ganglion cells through direct or indirect stimulation...
October 14, 2016: Annual Review of Vision Science
https://www.readbyqxmd.com/read/28532005/non-viral-strategies-for-ocular-gene-delivery
#4
REVIEW
Ana V Oliveira, Ana M Rosa da Costa, Gabriela A Silva
The success of gene therapy relies on efficient gene transfer and stable transgene expression. The in vivo efficiency is determined by the delivery vector, route of administration, therapeutic gene, and target cells. While some requirements are common to several strategies, others depend on the target disease and transgene product. Consequently, it is unlikely that a single system is suitable for all applications. This review examines current gene therapy strategies, focusing on non-viral approaches and the use of natural polymers with the eye, and particularly the retina, as their gene delivery target...
August 1, 2017: Materials Science & Engineering. C, Materials for Biological Applications
https://www.readbyqxmd.com/read/28524846/concerted-regulation-of-retinal-pigment-epithelium-basement-membrane-and-barrier-function-by-angiocrine-factors
#5
Ignacio Benedicto, Guillermo L Lehmann, Michael Ginsberg, Daniel J Nolan, Rohan Bareja, Olivier Elemento, Zelda Salfati, Nazia M Alam, Glen T Prusky, Pierre Llanos, Sina Y Rabbany, Arvydas Maminishkis, Sheldon S Miller, Shahin Rafii, Enrique Rodriguez-Boulan
The outer blood-retina barrier is established through the coordinated terminal maturation of the retinal pigment epithelium (RPE), fenestrated choroid endothelial cells (ECs) and Bruch's membrane, a highly organized basement membrane that lies between both cell types. Here we study the contribution of choroid ECs to this process by comparing their gene expression profile before (P5) and after (P30) the critical postnatal period when mice acquire mature visual function. Transcriptome analyses show that expression of extracellular matrix-related genes changes dramatically over this period...
May 19, 2017: Nature Communications
https://www.readbyqxmd.com/read/28524150/-functional-examinations-of-visual-channels-clinical-aspects
#6
M V Zueva, I V Tsapenko, E P Lantukh, N M Maglakelidze
The review discusses literature data on the clinical relevance of functional assessment of magno- (M), parvo- (P), and koniocellular (K) pathways. It also covers the differential contribution of the M, P, and K channels to visual impairments and how it determines the prognosis, early diagnosis, and treatment choice in patients with neurodegenerative diseases of the retina and brain. Selective changes in the performance of the visual channels are investigated by the example of glaucoma and optic neuritis in multiple sclerosis (MS) patients...
2017: Vestnik Oftalmologii
https://www.readbyqxmd.com/read/28522986/retinal-ganglion-cells-and-circadian-rhythms-in-alzheimer-s-disease-parkinson-s-disease-and-beyond
#7
REVIEW
Chiara La Morgia, Fred N Ross-Cisneros, Alfredo A Sadun, Valerio Carelli
There is increasing awareness on the role played by circadian rhythm abnormalities in neurodegenerative disorders such as Alzheimer's disease (AD) and Parkinson's disease (PD). The characterization of the circadian dysfunction parallels the mounting evidence that the hallmarks of neurodegeneration also affect the retina and frequently lead to loss of retinal ganglion cells (RGCs) and to different degrees of optic neuropathy. In the RGC population, there is the subgroup of cells intrinsically photosensitive and expressing the photopigment melanopsin [melanopsin-containing retinal ganglion cells (mRGCs)], which are now well known to drive the entrainment of circadian rhythms to the light-dark cycles...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28522876/comparative-proteomic-analysis-of-the-mitochondria-associated-er-membrane-mam-in-a-long-term-type-2-diabetic-rodent-model
#8
Jacey Hongjie Ma, Shichen Shen, Joshua J Wang, Zhanwen He, Amanda Poon, Jun Li, Jun Qu, Sarah X Zhang
The mitochondria-associated ER membrane (MAM) plays a critical role in cellular energetics and calcium homeostasis; however, how MAM is affected under diabetic condition remains elusive. This study presented a comprehensive proteome profiling of isolated brain MAM from long-term type 2 diabetic mice vs. non-diabetic controls. MAM protein was extracted efficiently by a surfactant-aided precipitation/on-pellet digestion (SOD) method, and MAM proteome was quantified by an ion-current-based MS1 method combined with nanoLC-MS/MS...
May 18, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28521879/a-quick-simple-method-for-detecting-circulating-fluorescent-advanced-glycation-end-products-correlation-with-in-vitro-and-in-vivo-non-enzymatic-glycation
#9
Marika Villa, Mariacristina Parravano, Arianna Micheli, Lucia Gaddini, Andrea Matteucci, Cinzia Mallozzi, Francesco Facchiano, Fiorella Malchiodi-Albedi, Flavia Pricci
OBJECTIVE: Advanced glycation end-products (AGEs) constitute a highly heterogeneous family of compounds, relevant in the pathogenesis of diabetic complications, which could represent efficient biomarkers of disease progression and drug response. Unfortunately, due to their chemical heterogeneity, no method has been validated to faithfully monitor their levels in the course of the disease. In this study, we refine a procedure to quantitatively analyze fluorescent AGEs (fAGEs), a subset considered remarkably representative of the entire AGE family, and measure them in in vitro glycated BSA (gBSA) and in plasma and vitreous of diabetic rats, for testing its use to possibly quantify circulating AGEs in patients, as markers of metabolic control...
June 2017: Metabolism: Clinical and Experimental
https://www.readbyqxmd.com/read/28515229/unique-cell-biological-profiles-of-retinal-disease-causing-missense-mutations-in-the-polarity-protein-crumbs
#10
Milena Pellikka, Ulrich Tepass
Mutations in human CRB1 are a major cause of retinal disease that lead to blindness. CRB1 is a transmembrane protein found in the inner segment of photoreceptor cells (PRCs) and the apical membrane of Müller glia. The function of the extracellular region of CRB1 is poorly understood although more than 80 disease-causing missense mutations have been mapped to it. We have recreated four mutations in Drosophila Crumbs (Crb) that affect different extracellular domains. Crb regulates epithelial polarity and growth, and contributes to PRC differentiation and survival...
May 17, 2017: Journal of Cell Science
https://www.readbyqxmd.com/read/28515072/%C3%AF-3-and-%C3%AF-6-long-chain-pufas-and-their-enzymatic-metabolites-in-neovascular-eye-diseases
#11
REVIEW
Yan Gong, Zhongjie Fu, Raffael Liegl, Jing Chen, Ann Hellström, Lois Eh Smith
Neovascular eye diseases, including retinopathy of prematurity, diabetic retinopathy, and age-related macular degeneration, threaten the visual health of children and adults. Current treatment options, including anti-vascular endothelial growth factor therapy and laser retinal photocoagulation, have limitations and are associated with adverse effects; therefore, the identification of additional therapies is highly desirable. Both clinical and experimental studies show that dietary ω-3 (n-3) long-chain polyunsaturated fatty acids (LC-PUFAs) reduce retinal and choroidal angiogenesis...
May 17, 2017: American Journal of Clinical Nutrition
https://www.readbyqxmd.com/read/28513113/retina-and-nerve-fiber-layer-thickness-in-eyes-with-thyroid-associated-ophthalmopathy
#12
Sharon Blum Meirovitch, Igal Leibovitch, Anat Kesler, David Varssano, Amir Rosenblatt, Meira Neuderfer
BACKGROUND: Thyroid-associated ophthalmopathy (TAO) is an inflammatory disease that affects the thyroid gland and the eye orbit. Of patients with TAO, 3%-5% have severe sight-threatening disease due to optic neuropathy Optical coherence tomography (OCT), the non-invasive imaging technology that yields high-resolution cross-sectional images of the retina, provides qualitative and quantitative data on the retina. OBJECTIVES: To apply this technique to quantitatively assess retinal nerve fiber layer (RNFL) and macular ring thicknesses in healthy subjects and in patients with TAO to determine their relationship to the severity of the orbital disease...
May 2017: Israel Medical Association Journal: IMAJ
https://www.readbyqxmd.com/read/28510482/rod-outer-segment-development-influences-aav-mediated-photoreceptor-transduction-after-subretinal-injection
#13
Lolita Petit, Shan Ma, Shun-Yun Cheng, Guangping Gao, Claudio Punzo
Vectors based on the adeno-associated virus (AAV) are currently the preferred tools for delivering genes to photoreceptors (PR) in small and large animals. AAVs have been applied successfully in various models of PR dystrophies. However, unknown barriers still limit AAV's efficient application in several forms of severe PR degenerations, due to insufficient transgene expression and/or treated cells at the time of injection. Optimizations of PR gene therapy strategies will likely benefit from the identification of the cellular factors that influence PR transduction...
May 16, 2017: Human Gene Therapy
https://www.readbyqxmd.com/read/28509387/progranulin-increases-phagocytosis-by-retinal-pigment-epithelial-cells-in-culture
#14
Hiromi Murase, Kazuhiro Tsuruma, Yoshiki Kuse, Masamitsu Shimazawa, Hideaki Hara
Retinal pigment epithelium (RPE) cells take part in retinal preservation, such as phagocytizing the shed photoreceptor outer segments (POS), every day. The incomplete phagocytic function accelerates RPE degeneration and formation of the toxic by-product lipofuscin. Excessive lipofuscin accumulation is characteristic of various blinding diseases in the human eye. Progranulin is a cysteine-rich protein that has multiple biological activities, and it has a high presence in the retina. Progranulin has been recognized to be involved in macrophage phagocytosis in the brain...
May 16, 2017: Journal of Neuroscience Research
https://www.readbyqxmd.com/read/28507131/involvement-of-a-gut-retina-axis-in-protection-against-dietary-glycemia-induced-age-related-macular-degeneration
#15
Sheldon Rowan, Shuhong Jiang, Tal Korem, Jedrzej Szymanski, Min-Lee Chang, Jason Szelog, Christa Cassalman, Kalavathi Dasuri, Christina McGuire, Ryoji Nagai, Xue-Liang Du, Michael Brownlee, Naila Rabbani, Paul J Thornalley, James D Baleja, Amy A Deik, Kerry A Pierce, Justin M Scott, Clary B Clish, Donald E Smith, Adina Weinberger, Tali Avnit-Sagi, Maya Lotan-Pompan, Eran Segal, Allen Taylor
Age-related macular degeneration (AMD) is the major cause of blindness in developed nations. AMD is characterized by retinal pigmented epithelial (RPE) cell dysfunction and loss of photoreceptor cells. Epidemiologic studies indicate important contributions of dietary patterns to the risk for AMD, but the mechanisms relating diet to disease remain unclear. Here we investigate the effect on AMD of isocaloric diets that differ only in the type of dietary carbohydrate in a wild-type aged-mouse model. The consumption of a high-glycemia (HG) diet resulted in many AMD features (AMDf), including RPE hypopigmentation and atrophy, lipofuscin accumulation, and photoreceptor degeneration, whereas consumption of the lower-glycemia (LG) diet did not...
May 15, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28505063/retinal-vessel-metrics-normative-data-and-their-use-in-systemic-hypertension-results-from-the-gutenberg-health-study
#16
Katharina A Ponto, David J Werner, Linn Wiedemer, Dagmar Laubert-Reh, Alexander K Schuster, Stefan Nickels, René Höhn, Andreas Schulz, Harald Binder, Manfred Beutel, Karl J Lackner, Philipp S Wild, Norbert Pfeiffer, Alireza Mirshahi
PURPOSE OF REVIEW: In-vivo measurement of retinal vascular calibers may be used as a tool to study the pathophysiology and clinical status of the microvasculature of the retina. The aim of this study was to generate normative data for retinal vessel parameters, and to evaluate the clinical relevance in systemic hypertension. METHODS: Fundus photographs from 4309 participants of the Gutenberg Health Study were assessed using the 'retinal vessel analyzer' software (IMEDOS)...
May 12, 2017: Journal of Hypertension
https://www.readbyqxmd.com/read/28504201/cilia-the-sensory-antennae-in-the-eye
#17
REVIEW
Helen May-Simera, Kerstin Nagel-Wolfrum, Uwe Wolfrum
Cilia are hair-like projections found on almost all cells in the human body. Originally believed to function merely in motility, the function of solitary non-motile (primary) cilia was long overlooked. Recent research has demonstrated that primary cilia function as signalling hubs that sense environmental cues and are pivotal for organ development and function, tissue hoemoestasis, and maintenance of human health. Cilia share a common anatomy and their diverse functional features are achieved by evolutionarily conserved functional modules, organized into sub-compartments...
May 11, 2017: Progress in Retinal and Eye Research
https://www.readbyqxmd.com/read/28503176/impact-of-autoantibodies-against-glycolytic-enzymes-on-pathogenicity-of-autoimmune-retinopathy-and-other-autoimmune-disorders
#18
REVIEW
Grazyna Adamus
Autoantibodies (AAbs) against glycolytic enzymes: aldolase, α-enolase, glyceraldehyde-3-phosphate dehydrogenase, and pyruvate kinase are prevalent in sera of patients with blinding retinal diseases, such as paraneoplastic [cancer-associated retinopathy (CAR)] and non-paraneoplastic autoimmune retinopathies, as well as in many other autoimmune diseases. CAR is a degenerative disease of the retina characterized by sudden vision loss in patients with cancer and serum anti-retinal AAbs. In this review, we discuss the widespread serum presence of anti-glycolytic enzyme AAbs and their significance in autoimmune diseases...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28501829/genetic-causes-of-optic-nerve-hypoplasia
#19
REVIEW
Chun-An Chen, Jiani Yin, Richard Alan Lewis, Christian P Schaaf
Optic nerve hypoplasia (ONH) is the most common congenital optic nerve anomaly and a leading cause of blindness in the USA. Although most cases of ONH occur as isolated cases within their respective families, the advancement in molecular diagnostic technology has made us realise that a substantial fraction of cases has identifiable genetic causes, typically de novo mutations. An increasing number of genes has been reported, mutations of which can cause ONH. Many of the genes involved serve as transcription factors, participating in an intricate multistep process critical to eye development and neurogenesis in the neural retina...
May 13, 2017: Journal of Medical Genetics
https://www.readbyqxmd.com/read/28500274/combined-hmg-coa-reductase-and-prenylation-inhibition-in-treatment-of-ccm
#20
Sayoko Nishimura, Ketu Mishra-Gorur, JinSeok Park, Yulia V Surovtseva, Said M Sebti, Andre Levchenko, Angeliki Louvi, Murat Gunel
Cerebral cavernous malformations (CCMs) are common vascular anomalies that develop in the central nervous system and, more rarely, the retina. The lesions can cause headache, seizures, focal neurological deficits, and hemorrhagic stroke. Symptomatic lesions are treated according to their presentation; however, targeted pharmacological therapies that improve the outcome of CCM disease are currently lacking. We performed a high-throughput screen to identify Food and Drug Administration-approved drugs or other bioactive compounds that could effectively suppress hyperproliferation of mouse brain primary astrocytes deficient for CCM3...
May 12, 2017: Proceedings of the National Academy of Sciences of the United States of America
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