AnatomyOrganismsDiseasesChemicals and DrugsAnalytical, Diagnostic and Therapeutic Techniques and EquipmentPsychiatry and PsychologyPhenomena and ProcessesDisciplines and OccupationsInformation ScienceHealth Care
Retinal DegenerationPhotoreceptor Cells, VertebrateRetinitis PigmentosaRetinal DiseasesRetinaRetinal Pigment Epitheliumcis-trans-IsomerasesEye ProteinsRhodopsinElectroretinographyRetinal Rod Photoreceptor CellsPhotoreceptor CellsDisease Models, AnimalSpinal DiseasesPigment Epithelium of EyeLeber Congenital AmaurosisMacular DegenerationLipofuscinRetinal Cone Photoreceptor CellsCyclic Nucleotide Phosphodiesterases, Type 6Night BlindnessPeripherinsInjections, IntraocularSpinal OsteophytosisFundus OculiEye Diseases, HereditaryRetinal VesselsLumbar VertebraeIntervertebral Disc DegenerationOphthalmoscopesNeurodegenerative DiseasesMutationSpondylosisRetinal DystrophiesGyrate AtrophyOxidative StressAgingMice, Inbred C57BLStem Cell TransplantationUsher SyndromesVision, OcularTomography, Optical CoherenceOphthalmoscopyRetinal NeovascularizationVitreoretinopathy, ProliferativeEpendymoglial CellsIntervertebral Disc DisplacementRetinal Ganglion CellsSpinal FusionBlindnessCells, CulturedCell SurvivalFluorescein AngiographyNerve Tissue ProteinsDark AdaptationOsteoarthritisCervical VertebraeDiabetic RetinopathyEpiretinal MembraneSpinal StenosisRetinitisVisual AcuityAntioxidantsGenetic TherapyLightRetinal NeuronsEye Infections, ViralMolecular Sequence DataRetinal TelangiectasisGenetic VectorsVitreous BodyBlotting, WesternVisual ProsthesisFovea CentralisReverse Transcriptase Polymerase Chain ReactionPhenotypeGene Expression RegulationMice, KnockoutRetinoidsCell DeathColor Vision DefectsMice, TransgenicApoptosisCell LineDNA, MitochondrialGreen Fluorescent ProteinsTime FactorsRod OpsinsDecompression, SurgicalVisual Field TestsMitochondriaAmyloidAmino Acid SequenceRadiculopathyImmunohistochemistryGene ExpressionCell DifferentiationDog DiseasesMuscular Atrophy, SpinalSignal Transduction
DegenerationRetinitisPhotoreceptorsMacularBlindnessPigment epithelialPathogenesisNeuronsRetinaInherited retinal degenerationsModels of retinalGeneticVitroMechanismsDegenerationsTherapiesCuresDetachmentAbstractGene therapyPluripotent stemNeurodegenerationNeuralPathologyProsthesesTissueGlaucomaOphthalmologyClinicalDiabetic retinopathyDementiaBipolar cellsCellsImplantsRetinoschisisMolecularBiologyAlterationsDifferentiationResearchTreatmentTherapeuticVivoMiceHumanRestoreFoveal
Degeneration28
Retinitis7
Photoreceptors12
  • It is a soluble, cell-surface protein that plays an important role in the maintenance of the retina where it is expressed and secreted by retinal bipolar cells and photoreceptors, as well as in the pineal gland. (wikipedia.org)
  • However, despite this diversity, many of these diseases share a common endpoint involving death of light-sensitive photoreceptors. (wustl.edu)
  • Because the retina and photoreceptors, in particular, have immense metabolic and energetic requirements, many investigators have hypothesized that metabolic dysfunction may be a common link unifying various retinal degenerative diseases. (wustl.edu)
  • Both atrophic and hypertrophic changes occur in the RPE underlying the central macula and can lead to the loss of retinal photoreceptors. (medscape.com)
  • Retinal neuroprotection by hypoxic preconditioning is independent of hypoxia-inducible factor-1α expression in photoreceptors. (hslu.ch)
  • The light-dependent fluctuation of a disease-related substance in the photoreceptors should prompt further study of the potential role of light as a modulator of the progression of RPGR XLRP. (molvis.org)
  • This project seeks to elucidate transcriptional and post-transcriptional regulatory networks that determine cell fate and guide the development of photoreceptors from retinal progenitor cells. (nih.gov)
  • We are now focused on delineating the transcription factors and signaling pathways that are responsible for generating photoreceptors from retinal progenitor cells. (nih.gov)
  • Many forms of blindness result from the dysfunction or loss of retinal photoreceptors. (nature.com)
  • Moreover, the photoreceptors in our hiPSC-derived retinal tissue achieve advanced maturation, showing the beginning of outer-segment disc formation and photosensitivity. (nature.com)
  • Retinal degenerative diseases destroy photoreceptors-the retina's rods and cones-but other parts of the eye usually remain healthy. (medicalxpress.com)
  • These cells process the photoreceptors' inputs before they reach ganglion cells, which send retinal signals to the brain. (medicalxpress.com)
Macular4
  • Retinal and macular diseases are a major cause of visual impairment and affect the quality of life of millions worldwide. (nih.gov)
  • Best disease, also termed vitelliform macular dystrophy, is typically an autosomal dominant disorder, which classically presents in childhood with the striking appearance of a yellow or orange yolklike lesion in the macula. (medscape.com)
  • Adult vitelliform macular dystrophy resembles Best disease, but it can be differentiated by its later age of onset, smaller lesion, and normal electro-oculogram testing. (medscape.com)
  • The EOG in Best's disease and dominant cystoid macular dystrophy (DCMD). (medscape.com)
Blindness10
  • A new treatment for patients with a form of congenital retinal blindness has shown success in improving vision, according to results published today in Nature Medicine led by researchers at the Scheie Eye Institute in the Perelman School of Medicine at the University of Pennsylvania. (news-medical.net)
  • Finding genes for retinal degenerations has immediate benefits for people living with blindness and vision loss, their families, and their physicians. (news-medical.net)
  • Examples of such altered fundus reflexes include the golden fundus reflex observed in X-linked retinoschisis and Oguchi disease [ 1 - 3 ], a form of congenital stationary night blindness. (molvis.org)
  • Because of their limited potential for regeneration, the loss of retinal neurons leads to permanent blindness. (labmanager.com)
  • The Los Angeles Chapter of the Foundation Fighting Blindness will be hosting our 17th Annual Los Angeles VisionWalk, a 5k walk fundraiser to benefit the Foundation's mission to find treatments and cures for blinding retinal degenerative diseases. (volunteermatch.org)
  • Abstract: The retinal degenerative diseases, which together constitute a leading cause of hereditary blindness worldwide, are largely untreatable. (utah.edu)
  • The Foundation Fighting Blindness is dedicated to funding innovative research to find preventions, treatments, and cures for inherited retinal degenerative diseases. (volunteerinfo.org)
  • Furthermore, accumulated light-induced damage may contribute to AMD, a globally important retinal degenerative disease that causes blindness. (arvojournals.org)
  • For early-stage treatment of these degenerative retinal diseases, the rationale is to stop or delay the death of the retinal cells, which is challenging, with many patients ultimately progressing to severe visual impairment and eventually complete blindness. (lu.se)
  • Rods and cones are frequent targets of heritable neurodegenerative diseases that cause visual impairment, including blindness, in millions of people worldwide. (bvsalud.org)
Pigment epithelial2
  • LCN2 was increased by SOX9 and SIRT1 deacetylated SOX9 to promote its nuclear translocation, which further inhibited the viability of human retinal pigment epithelial cells and promoted cell apoptosis and angiogenesis as well as CNV-induced AMD formation. (hindawi.com)
  • The present study investigated the effects of GSK2606414 on proliferation, apoptosis, and the expression of activating transcription factor 4 (ATF4), CCAAT/enhancer‑binding protein homologous protein (CHOP) and vascular endothelial growth factor (VEGF) in human retinal pigment epithelial (RPE) cells under endoplasmic reticulum (ER) stress. (spandidos-publications.com)
Pathogenesis4
  • Here, we discuss a new area of research examining the role of NAD + and sirtuins in regulating retinal metabolism and in the pathogenesis of retinal degenerative diseases. (wustl.edu)
  • LCN2 is a member of secreted adipokines which engaged in the pathogenesis of several diseases including cancer, diabetes, obesity, and AMD [ 13 ]. (hindawi.com)
  • 1 Although the pathogenesis of light-induced retinal damage remains unclear, oxidative stress is likely involved. (arvojournals.org)
  • Overall, the application of spatial transcriptomics to rd1 mice can help to elucidate the important pathogenesis of RP involving photoreceptor apoptosis and retinal remodeling. (bvsalud.org)
Neurons5
  • Any damage to retinal neurons can have devastating consequences, including loss of vision. (nih.gov)
  • This involves placing stem cell clusters into a special medium that induces the spontaneous formation of undeveloped neurons, followed by their differentiation into retinal cells. (labmanager.com)
  • That process produces actual retinal neurons organized as complex tissue, without any external stimulation of development pathways during specialization. (labmanager.com)
  • Numbers matter: The automation empowered us to produce trillions of retinal neurons for transplantation and we are excited to see the translation of our approach into routine cell manufacture," commented Petr Baranov from the Schepens Eye Research Institute of Massachusetts Eye and Ear. (labmanager.com)
  • The implant capitalizes on the electrical excitability of retinal neurons known as bipolar cells. (medicalxpress.com)
Retina6
  • Therefore, the number of RPE cells invading the retina in retinal degenerative disease may be underappreciated by funduscopy. (bmj.com)
  • Retinoschisin is an extracellular protein that plays a crucial role in the cellular organization of the retina: it binds the plasma membranes of various retinal cells tightly to maintain the structure of the retina. (wikipedia.org)
  • AMD has two types: dry AMD with the presence of drusen and atrophy and wet AMD with the features of edema and hemorrhage within or below the retina or retinal pigment epithelium besides drusen and atrophy [ 3 ]. (hindawi.com)
  • Our investigations utilize in vivo mouse retina and human retinal organoids derived from pluripotent stem cells as study systems. (nih.gov)
  • Scientists also need to figure out how to electrically stimulate the retina in a way that the brain can interpret with high spatial resolution, says Joseph Rizzo , an ophthalmologist at the Massachusetts Eye and Ear Infirmary and codirector of the Boston Retinal Implant Project. (technologyreview.com)
  • The advanced outer segment-like structures reported here support the notion that 3D retina cups could serve as a model for studying mature photoreceptor development and allow for more robust modeling of retinal degenerative disease in vitro. (utah.edu)
Inherited retinal degenerations1
  • This review summarizes the diverse genetic causes of inherited retinal degenerations (IRDs) and their convergence onto common pathogenic mechanisms of vision loss. (bvsalud.org)
Models of retinal1
  • Getting tissues from human subjects with RP is rare, so transgenic models of retinal degenerative diseases are desirable. (utah.edu)
Genetic4
  • A significant challenge in treating retinal degenerative diseases is their genetic and phenotypic heterogeneity. (wustl.edu)
  • Successful gene therapy is based on the effective delivery and maintained expression of healthy copies of a gene into tissues of individuals with a disease-associated genetic mutation. (news-medical.net)
  • The research will focus on a single genetic disease, then spread out to additional conditions that are affected by modulation of the UPR pathway. (biospace.com)
  • A number of genetic diseases, neurodegenerative and retinal degenerative diseases are related to unfolded proteins in the endoplasmic reticulum (ER). (biospace.com)
Vitro2
  • It is possible to grow retinal tissue in vitro . (labmanager.com)
  • In vitro use of degenerative tenocytes is essential when investigating the efficacy of novel treatment on tendinopathy. (jove.com)
Mechanisms4
  • The understanding of the underlying mechanisms is complicated by the heterogeneity of cases within a particular retinopathy and by the interplay of multiple cellular signaling involved in each disease model. (molvis.org)
  • 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. (wustl.edu)
  • What are intrinsic control mechanisms that lead to photoreceptor cell fate from retinal progenitors? (nih.gov)
  • While these approaches target different diseases and employ different mechanisms and methodologies, one thing is universally true: Their development requires massive amounts of retinal cells for research purposes. (labmanager.com)
Degenerations1
  • While more than 200 genes for retinal degenerations have been identified, approximately 40-50% of cases remain a mystery. (news-medical.net)
Therapies2
  • This important proof-of-concept finding supports the development of personalized iPSC-based transplantation therapies for retinal disease. (nature.com)
  • This success brings us one step closer to the anticipated use of hiPSC for disease modelling and open possibilities for future therapies. (nature.com)
Cures1
  • Scientists can identify the origins of diseases by studying how RNA turns genes on and off, potentially leading to innovative disease treatments and possible cures. (albany.edu)
Detachment1
Abstract1
  • abstract = " Retinal degenerative diseases are a major cause of morbidity in modern society because visual impairment significantly decreases the quality of life of patients. (wustl.edu)
Gene therapy5
  • Additionally, adeno-associated virus-mediated gene delivery is a safe and effective method for modulating gene expression, and thus is a useful research tool for manipulating individual UPR markers in affected retinas and a promising delivery vector for gene therapy in retinal degenerative disorders. (molvis.org)
  • A new study presented at the 121st Annual Meeting of the American Academy of Ophthalmology (AAO) 2017, for the first time found a novel gene therapy that can improve the eyesight of patients with inherited retinal disease. (news-medical.net)
  • Patients who had lost their sight to an inherited retinal disease could see well enough to navigate a maze after being treated with a new gene therapy, according to research presented today at AAO 2017, the 121st Annual Meeting of the American Academy of Ophthalmology. (news-medical.net)
  • The relatively fast disease progression makes the TgP347L rabbit an excellent model for gene therapy, cell biological intervention, progenitor cell transplantation, surgical interventions and bionic prosthetic studies. (utah.edu)
  • However, delivery of genes which prevent apoptotic cell death may have broad application to gene therapy of retinal degenerative diseases. (nih.gov)
Pluripotent stem2
  • Induced pluripotent stem cells (iPSCs) generated from patient fibroblasts could potentially be used as a source of autologous cells for transplantation in retinal disease. (nature.com)
  • Induced pluripotent stem cells (iPSCs) hold great potential for the modelling of these diseases or as potential therapeutic agents. (nature.com)
Neurodegeneration1
  • He applies genetics and genomics approaches to study age-related neurodegeneration associated with Alzheimer's disease, dementia and glaucoma. (jax.org)
Neural3
  • Retinal implants can take over for these lost cells, converting light into neural signals that are then interpreted by the brain. (technologyreview.com)
  • BioTime's majority owned subsidiary Cell Cure Neurosciences Ltd. is developing therapeutic products derived from stem cells for the treatment of retinal and neural degenerative diseases. (aol.com)
  • By stimulating bipolar cells, the implant takes advantage of important natural properties of the retinal neural network, which produces more refined images than the devices that skip these cells. (medicalxpress.com)
Pathology1
  • Despite recent advances in our understanding of the mechanism of these retinopathies, much work remains to get a clear picture of the molecular pathology of each disease. (molvis.org)
Prostheses1
  • Existing retinal prostheses are powered by extraocular devices wired to the retinal electrode array, which require complex surgeries, and provide visual acuity up to about 20/1,200. (medicalxpress.com)
Tissue7
  • Dr Tamara Jamaspishvili is a research pathologist, translational research scientist focusing on developing, evaluating, validating, and applying tissue-based prognostic and predictive biomarkers and models for improved disease prognostication and management of cancer patients. (upstate.edu)
  • The researchers compared the quality of the robot- and human-grown cells by producing several thousand retinal tissue samples for automatic processing and the same amount for manual handling. (labmanager.com)
  • Since this protein is only produced in developing retinal cells, the high fluorescence intensity points to the parts of the sample with the right tissue. (labmanager.com)
  • Here we have succeeded in inducing human iPSC to recapitulate the main steps of retinal development and to form fully laminated 3D retinal tissue by exploiting the intrinsic cues of the system to guide differentiation ( Supplementary Fig. 1 ). (nature.com)
  • The main advantage of this technique is that we can obtain human degenerative tenocyte without additional patient injury because the degenerative tissue is acquired during standard epicondylitis surgery. (jove.com)
  • To acquire a degenerative tendocyte tissue sample, use a number 15 surgical scalpel to make a three to five centimeter skin incision just anterior medial to the lateral epicondyle, and proximal to the level of the joint to expose the lateral elbow and visually locate the extensor carpi radialis longus in the extensor aponeurosis interface. (jove.com)
  • After anterior retraction of the extensor carpi radialis longus, locate the pathologic degenerative tissue by its characteristic dull grayish color and typically edematous and friable tissue, and use the scalpel and mini blades to carefully, but sharply, resect all of the degenerative tissue. (jove.com)
Glaucoma2
  • We, as a family, know firsthand the importance of finding a cure for glaucoma and other debilitating neurodegenerative eye diseases. (jax.org)
  • Without these amazing donations, we would not have been able to get going so quickly on our research to understand how aging contributes to increased risk for glaucoma and other diseases of the eye," says Howell. (jax.org)
Ophthalmology2
  • In contrast, the rabbit possesses a number of desirable qualities for a model of retinal disease including a large eye and an existing and substantial knowledge base in retinal circuitry, anatomy and ophthalmology. (utah.edu)
  • In June 2002, Fox and his colleagues published a study showing that 7- to 10-year-old children whose mothers had elevated levels of lead in their blood during the first trimester of pregnancy developed retinal abnormalities, specifically in their rods (Investigative Ophthalmology & Visual Science, June 2002, Vol. 43. (sciencedaily.com)
Clinical3
  • Recent clinical trials evaluating allogeneic retinal grafts derived from human embryonic stem cells (hESCs) show the procedure to be safe and potentially effective 1 . (nature.com)
  • As one of the first of its kind on a global scale, IOB will bridge basic and clinical science for eye diseases within one organization. (unibas.ch)
  • Molecular hydrogen has been used as an antioxidant to treat many diseases in clinical and animal studies. (arvojournals.org)
Diabetic retinopathy1
Dementia2
  • Alzheimer disease (AD) is the most common progressive degenerative form of dementia, strongly associated with advancing age. (medscape.com)
  • Artificial intelligence for biomarker discovery in Alzheimer's disease and dementia. (cdc.gov)
Bipolar cells1
  • The wireless retinal implants convert light transmitted from special glasses into electrical current, which stimulates the retina's bipolar cells. (medicalxpress.com)
Cells9
  • These intraretinal pigmented cells may be derived from the retinal pigment epithelium (RPE), but differences and similarities between intraretinal pigmented cells and RPE have so far not been well characterised. (bmj.com)
  • This structure allows it to adhere to the plasma membrane of retinal cells such as bipolar and photoreceptor cells, joining them together. (wikipedia.org)
  • Ciliopathies are diseases that affect the cilia, sensory organelles that most mammalian cells possess and which play a critical role in many biological functions. (news-medical.net)
  • The array delivers electrical signals to the nerve cells in the eye, mimicking the role of light-sensitive cells lost in degenerative retinal disease. (technologyreview.com)
  • A ray of light, for example, stimulates retinal cells in a more precise and refined way than does the electric current coming from an electrode. (technologyreview.com)
  • BioTime's subsidiary OrthoCyte Corporation is developing therapeutic applications of stem cells to treat orthopedic diseases and injuries. (aol.com)
  • ReCyte Therapeutics, Inc. is developing applications of BioTime's proprietary induced pluripotent stem cell technology to reverse the developmental aging of human cells to treat cardiovascular and blood cell diseases. (aol.com)
  • The RPE appears to have degenerative changes in some cases, and secondary loss of photoreceptor cells has been noted. (medscape.com)
  • The retinal pigment epithelium (RPE) is a monolayer of hexagonal cells located at the back of the eye. (bvsalud.org)
Implants2
  • Retinal implants available today already provide patients with some level of vision. (lu.se)
  • Another important aspect relates to the long-term functionality of retinal implants, which is still limited. (lu.se)
Retinoschisis1
  • RS1 structure-function relationships: roles in retinal adhesion and X-linked retinoschisis (Ph.D. thesis). (wikipedia.org)
Molecular1
  • Hartzell HC, Qu Z, Yu K, Xiao Q, Chien LT. Molecular physiology of bestrophins: multifunctional membrane proteins linked to best disease and other retinopathies. (medscape.com)
Biology3
  • Mitochondrial biology, stress signaling and aging-related degenerative diseases. (upstate.edu)
  • Part of the Diana Davis Spencer Foundation's gift provides graduate students and postdoctoral fellows the opportunity to be mentored by world-class experts in ocular biology, genetics and genomics, disease modeling and computational biology. (jax.org)
  • This method can help answer key questions about human degenerative tenocyte biology as only few studies have described human degenerative tenocyte isolation and culture in detail. (jove.com)
Alterations2
  • They may have visual system deficits that eventually could lead to permanent retinal alterations and learning problems," Fox says. (sciencedaily.com)
  • Retinal functional alterations in mice lacking intermediate filament proteins glial fibrillary acidic protein and vimentin. (lu.se)
Differentiation1
  • We implemented automated liquid change during retinal differentiation and showed it had no negative effect on cell specialization," commented Evgenii Kegeles of the Genome Engineering Lab at MIPT. (labmanager.com)
Research6
  • Although further research is necessary to translate these findings to the bedside, they have strong potential to truly transform the standard of care for patients with retinal degenerative diseases. (wustl.edu)
  • This research has been extended to include how aging affects retinal and photoreceptor function. (nih.gov)
  • Despite decades of research, there is currently no cure for these diseases. (nature.com)
  • JAX researchers discuss how philanthropy is furthering scientific research and training activities in the field of neurodegenerative diseases of the eye. (jax.org)
  • The gifts fund the Laboratory's scientific research and training activities in the field of neurodegenerative diseases of the eye, including graduate and postdoctoral fellowship training. (jax.org)
  • Proteostasis Therapeutics, Inc. , located in Cambridge, Mass., announced today an R&D and commercialization agreement with Tokyo-based Astellas Pharma Inc . The focus of the agreement will be to research and develop compounds that modulate the Unfolded Protein Response (UPR) through the use of Proteostasis 's proprietary "Disease Relevant Translation" and " Proteostasis Network" platform. (biospace.com)
Treatment5
  • Its researchers have established Ireland's most successful start-up companies, including the biomedical company, Opsona Therapeutics, for the treatment of autoimmune diseases and cancers, and the games industry software company, Havok, that was sold to Intel for €76 million. (tcd.ie)
  • Currently, we have limited treatment options for all of our patients with blinding eye diseases", says Dr. Werner Kübler, Director of the University Hospital Basel. (unibas.ch)
  • 3 - 5 Therefore, antioxidants capable of easily passing the blood-retinal barrier could be a promising treatment for light-induced damage. (arvojournals.org)
  • Therefore, it is necessary to understand how the disease develops and determine whether the treatment is effective. (bvsalud.org)
  • The prognosis is poor as no treatment is available for the primary disease. (medscape.com)
Therapeutic1
  • BioTime's therapeutic product development strategy is pursued through subsidiaries that focus on specific organ systems and related diseases for which there is a high unmet medical need. (aol.com)
Vivo1
  • Here we report that hiPSC can, in a highly autonomous manner, recapitulate spatiotemporally each of the main steps of retinal development observed in vivo and form three-dimensional retinal cups that contain all major retinal cell types arranged in their proper layers. (nature.com)
Mice2
  • The new technique for the powerful gene editing tool CRISPR to restore retinal function in mice afflicted by a degenerative retinal disease. (asianage.com)
  • In this study, spatial transcriptomic analysis is used to study the changes in different retinal layers of rd1 mice at different ages. (bvsalud.org)
Human2
  • We propose the following protocol to isolate human degenerative tenocytes during surgery. (jove.com)
  • This manuscript shows that disease progression in the TgP347L rabbit closely mimics human cone-sparing RP, including the cone-associated preservation of bipolar cell signaling and triggering of reprogramming. (utah.edu)
Restore2
  • A team led by Stanford University researchers has developed a wireless retinal implant that they say could restore vision five times better than existing devices. (medicalxpress.com)
  • At advanced stages of a disease, one option is to restore visual function with the help of electronic devices. (lu.se)
Foveal1
  • In young males who carry a gene mutation, the disease presents itself as retinal cavities, splitting of inner retinal layers (also known as foveal schisis), and defective synapse activity. (wikipedia.org)