Abstract
Best vitelliform macular dystrophy (BVMD, also called Best's disease) is a dominantly inherited, juvenile-onset form of macular degeneration, which is characterized by abnormal accumulation of yellow pigment in the outer retina and a depressed electro-oculogram light peak (LP). Over 100 disease-caus...
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PMID: 20349192
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Abstract
We studied the role of MT3 in oxidative stress responses. H(2)O(2) induced a large increase in labile zinc in wild-type (WT) astrocytes, but stimulated only a modest rise in MT3-null astrocytes. In addition, H(2)O(2)-induced lysosomal membrane permeabilization (LMP) and cell death were comparably at...
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PMID: 20544854
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Abstract
We analyzed the phenotype of spinal curvature and expression of genes related to the anti-oxidant defense system and myogenesis in muscle of 8 and 30 months old GH-transgenic males. Gene expression analyses revealed that both superoxide dismutase isoforms were down-regulated only in 30 months old an...
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PMID: 20338232
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Abstract
We have identified two biomarkers for non-enzymatic nitration in aged human Bruch's membrane, indicative of inflammation, that include 3-nitrotyrosine identified in Bruch's membrane preparations and nitrated A2E from the lipid soluble extract of the Bruch's membrane preparation. Approximately 30-40...
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PMID: 20153746
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Abstract
Six novel missense mutations (Thr2Asn, Leu75Phe, Ser144Asn, Arg255Trp, Pro297Thr, and Asp301Gly) and 1 previously reported mutation (Arg218Cys) were identified. Each family was found to have a unique BEST1 mutation that segregated with the disease. Two of the six novel mutations are located within t...
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PMID: 20057343
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Abstract
The pigment epithelium cell structure and therapeutic effect of antioxidant SkQ1, selectively penetrating into mitochondria from eye drops, were studied upon development in OXYS rats of age-related retinopathy as a model of macular degeneration. The characteristic dynamics and ultrastructural peculi...
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PMID: 20367599
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Abstract
We take a look at different methods of detection and quantification of lipofuscin in single cells by using its physical or chemical features....
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PMID: 20072918
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Abstract
We examined insulin signaling in adipocytes from T2D patients, and contrary to findings in animal studies, we observed attenuation of insulin activation of mammalian target of rapamycin (mTOR) in complex with raptor (mTORC1). As a consequence, mTORC1 downstream effects were also affected in T2D: fee...
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PMID: 20386866
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Abstract
Bisretinoid lipofuscin compounds that accumulate in retinal pigment epithelial (RPE) cells are implicated in the pathogenesis of some forms of macular degeneration. In the development of approaches to the amelioration of retinal disorders characterized by enhanced RPE lipofuscin formation, attention...
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PMID: 20552437
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Abstract
We use the protein RPE65 as an example to describe generally applicable methods for determining the stability and enzymatic activity of proteins and their mutants involved in retinoid metabolism. Additionally, we introduce a range of analytical techniques involving high-performance liquid chromatogr...
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PMID: 20552432
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Abstract
We present the quantitative use of two-photon excited fluorescence (TPEF) and second harmonic generation (SHG) as a noninvasive means to monitor the differentiation of human mesenchymal stem cells (hMSCs) using entirely endogenous sources of contrast. We demonstrate that the individual fluorescence...
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PMID: 20419124
PDF is available here.
Abstract
That reciprocity held is consistent with a photochemical origin, which may or may not cause retinal degeneration. The implementation of safe methods for delivering light to the retina requires a better understanding of the mechanism causing autofluorescence reduction. Finally, RPE imaging was demons...
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PMID: 19628734
PDF is available here.
Abstract
The kinetics of lipofuscin growth in diseased retinal pigment epithelium cells is investigated using Monte Carlo simulations and scaling theory on a cluster aggregation model. The model captures the essential physics of lipofuscin growth in the cells. A remarkable feature is that small particles may...
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PMID: 20365007
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Abstract
We hypothesize that signal transduction within the neurons of ARH is dependent on oxidative stress phenomena....
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PMID: 19950070
PDF is available here.
Abstract
We report a case of brown bowel syndrome complicating jejunoileal bypass, the first case reported in the literature to the best of our knowledge....
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PMID: 19506985
PDF is available here.
Abstract
We have identified two related but previously unknown RPE lipofuscin compounds. By high performance liquid chromatography-electrospray ionization-tandem mass spectrometry, we determined that the first of these compounds is a phosphatidyl-dihydropyridine bisretinoid; to indicate this structure and it...
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PMID: 19478335
PDF is available here.
Abstract
The accumulation of lysosome- and mitochondria-derived reactive oxygen species (ROS) are the most important causative factors for aging. Autophagic dysfunction and mitochondrial DNA damage in the central nervous system (CNS) are prominently found in microglia, the resident mononuclear phagocyte popu...
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PMID: 19428609
PDF is available here.
Abstract
We solve the question of how and why do cells sense oxygen. In other words, to better understand aging we need to know what O(2) species are being sensed by cells. In conclusion, hypoxia and hyperoxia represent an experimental model adequate for studying aging processes....
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PMID: 20021403
PDF is available here.
Abstract
Glycoxidized particles phagocytosed might stay in RPE cells without any acute biological reaction. Our rabbit model of AMD simulated abnormal FAF patterns observed in aging human eyes with or without AMD. Glycoxidized particles phagocytosed by RPE cells could be deposited on Bruch's membrane in rabb...
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PMID: 19330346
PDF is available here.
Abstract
We reported several brain alterations including a strong decrease in hippocampal proliferation and survival in both spontaneous and streptozotocin-induced models of experimental T1D. The aim of this study was to explore in streptozotocin-treated mice and other parameters associated to mild neurodege...
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PMID: 19041902
PDF is available here.
Abstract
SW-AF and NIR-AF imaging confirmed granular retinal pigment epithelium atrophy which corresponds to FFA findings. SW-AF and NIR-AF imaging methods are noninvasive and useful techniques for documentation of fundus changes in VKH disease....
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PMID: 19882556
PDF is available here.
Abstract
The integrated fluorescence of murine eyes is collected as a function of age. This fluorescence is attributed to pigments generally referred to as lipofuscin and is observed to increase with age. No difference in fluorescence intensity is observed between the eyes of males or females. This work prov...
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PMID: 18764899
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Abstract
Although short-lived vertebrates can serve as model animals for understanding the mechanism of aging, whether the annual fish Nothobranchius rachovii is suitable for studying aging remains an open question. In this study, histochemical, biochemical, and genetic techniques were used to determine the...
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PMID: 19126839
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Abstract
We concluded that Ohrid trout presented a pigment composition in liver macrophages that differed from other fish, including salmonids, where most liver phagocytes essentially display melanin. Our quantitative data support interspecies differences in the amount of liver macrophages and also that afte...
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PMID: 18767051
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Abstract
We examined patients with retinal dystrophies such as Stargardt disease, Best vitelliform macular dystrophy, and retinal dystrophies associated with mutations in the peripherin/RDS gene. All retinal dystrophies were confirmed by molecular genetic analysis. A broad range of characteristic FAF pattern...
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PMID: 18289629
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Abstract
Retinal phototoxicity significantly increased with age; however, the retinal protection by IOLs was independent of age. The interaction between the transmission curve of IOLs and light sources required inclusion of widely used lamps besides sunlight for evaluation of IOLs. For the 420 to 480 nm wave...
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PMID: 18812134
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Abstract
Retinal epithelial cells overlying drusen have less lipofuscin than neighboring epithelial cells. The loss of lipofuscin seems due to a loss of cytoplasm containing lipofuscin that contributes to drusen formation. Macrophages in Bruch's membrane may be responsible for removing this lipofuscin debris...
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PMID: 18696097
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Abstract
Immersion microscopy was used to analyse patterns of lipofuscin accumulation during ageing making possible to distinguish diffuse type (lipofuscin granules were irregularly distributed and non-confluent), unipolar type (lipofuscin granules were grouped at the end of the cell), bipolar type (lipofusc...
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PMID: 19024118
PDF is available here.
Abstract
We report that mice lacking both the ATP-binding cassette transporter 4 (Abca4) and enzyme retinol dehydrogenase 8 (Rdh8), proteins critical for all-trans-retinal clearance from photoreceptors, developed severe RPE/photoreceptor dystrophy at an early age. This phenotype includes lipofuscin, drusen,...
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PMID: 18658157
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Abstract
We quantified the ATP-dependent phagocytic function of RPE cells loaded or not with the lipofuscin component A2E and inhibiting or not mitochondrial ATP synthesis either pharmacologically or genetically. We found that physiological levels of lysosomal A2E reduced mitochondrial membrane potential and...
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PMID: 18621729
PDF is available here.
Abstract
Vitamin A supplementation should be avoided in patients with ABCA4 mutations or other retinal or macular dystrophies associated with lipofuscin accumulation in the retinal pigment epithelium....
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PMID: 18515570
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Abstract
The RPE-AF of exudative AMD lesions varies greatly. The AF differences probably represent different kinds of metabolism disorders in the RPE. Furthermore, they apparently have a great influence on the chances of anti- vascular endothelial growth factor (VEGF) therapy success; in particular the devel...
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PMID: 18509668
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Abstract
The removal of central nervous system (CNS) tissues as part of bovine spongiform encephalopathy (BSE) risk material is one of the highest priority tasks to avoid contamination of the human food chain with BSE. No currently available method enables the real-time detection of possible CNS tissue conta...
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PMID: 18620407
PDF is available here.
Abstract
Dehydroepiandrosterone (DHEA), one of the major steroid hormones, and its ester have recently received attention with regard to aging and age-related diseases like Alzheimer and others. DHEA is synthesized de novo in the brain and its substantial fall with age has been shown to be associated with ne...
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PMID: 18307051
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Abstract
The study revealed a novel change in RPE autofluorescence induced by 568-nm light exposure. Retinal damage occurred as a direct result of total average power, independent of the light-delivery METHOD: Because the exposures were near or below permissible levels in laser safety standards, these result...
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PMID: 18408191
PDF is available here.
Abstract
Well-defined increased FAF at the new macula after MT360 suggests that FAF reflects not only fluorophores in the RPE but also in the neurosensory retina. These fluorophores may result from interactions between the retina and CNV/pathologic RPE....
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PMID: 18458934
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Abstract
The most striking morphologic change in neurons during normal aging is the accumulation of autophagic vacuoles filled with lipofuscin or neuromelanin pigments. These organelles are similar to those containing the ceroid pigments associated with neurologic disorders, particularly in diseases caused b...
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PMID: 18384642
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Abstract
Porcine ocular fundi were successfully preserved in an organotypic perfusion culture for 7 days, and exhibited remarkable autofluorescence after light and free radical exposure, making the model suitable for investigations of lipofuscinogenesis....
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PMID: 18351374
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Abstract
Neuromelanin and lipofuscin are two pigments produced within the human brain that, until recently, were considered inert cellular waste products of little interest to neuroscience. Recent research has increased our understanding of the nature and interactions of these pigments with their cellular en...
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PMID: 18278576
PDF is available here.
Abstract
An intact retinal pigment epithelium (RPE) represents an essential condition for the visual process. This post-mitotic RPE monolayer combines different functions such as degradation of photoreceptor outer segments, vitamin A cycle, support of retinal metabolism and maintenance of the outer blood-ret...
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PMID: 18516775
PDF is available here.
Abstract
The canine TPP1 mutation results in progressive vision loss and retinal degeneration similar to that which occurs in human late infantile NCL. With the canine model, the natural history of disease progression in the retina provides a better understanding of the pathologic course of the disease and p...
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PMID: 18344450
PDF is available here.
Abstract
AOFVD can be associated with different FAF patterns. A functional correlate with visual acuity and retinal sensitivity, measured by means of microperimetry, can be found....
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PMID: 18536595
PDF is available here.
Abstract
Endocrine disrupting compounds (EDCs), including both natural estrogens and estrogenic chemicals, are almost ubiquitous in the aquatic environment. In the marine bivalve Mytilus galloprovincialis different estrogenic compounds, both individually and in mixtures, were shown to affect the immune funct...
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PMID: 18374427
PDF is available here.
Abstract
We think that the use of this Ponto-Caspian non-indigenous species in biomonitoring surveys deserves further investigation, owing to its current position in freshwater and brackish European ecosystems, considering both biomass and functioning. Cellular responses could be studied in parallel with end...
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PMID: 18499217
PDF is available here.
Abstract
We have shown that platinum nanoparticles (nano-Pt) are a superoxide dismutase (SOD)/catalase mimetic. Various data have shown extension of the Caenorhabditis elegans lifespan by antioxidant treatment. The present study was designed to elucidate the survival benefit conferred by nano-Pt, as compared...
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PMID: 18400258
PDF is available here.
Abstract
We report that a major lipofuscin component, A2E, activates the retinoic acid receptor (RAR). In vitro experiments using luciferase reporter assay, competitional binding assay, analysis of target genes, and chromatin immunoprecipitation (ChIP) assay strongly suggest that A2E is a bona fide ligand fo...
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PMID: 18326047
PDF is available here.
Abstract
Autofluorescence photography demonstrated the RPE atrophy, which was hard to see by other means of investigation. The areas of RPE atrophy did not necessarily correspond to the hypopigmented lesions, which suggested that both the choroid and the RPE can be affected independently. Retinal pigment epi...
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PMID: 18378316
PDF is available here.
Abstract
AcrySof Natural filter significantly reduced blue light-induced apoptosis. This was most likely due to its filtering effect on blue wavelength light, which reduces the energy that reaches the cells. In patients with cataract who are at a high risk for AMD, the implantation of a blue light-filtering...
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PMID: 18299878
PDF is available here.
Eun-Joo EJ Shin,
Ji Hoon JH Jeong,
Guoying G Bing,
Eon Sub ES Park,
Jong Seok JS Chae,
Tran Phi Hoang TP Yen,
Won-Ki WK Kim,
Myung-Bok MB Wie,
Bae-Dong BD Jung,
Hyun Ji HJ Kim,
Sung-Youl SY Lee and
Hyoung-Chun HC Kim
Abstract
We have demonstrated that kainate (KA) induces a reduction in mitochondrial Mn-superoxide dismutase (Mn-SOD) expression in the rat hippocampus and that KA-induced oxidative damage is more prominent in senile-prone (SAM-P8) than senile-resistant (SAM-R1) mice. To extend this, we examined whether KA s...
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PMID: 18248956
PDF is available here.
Abstract
We have investigated whether administration of an antioxidant, epigallocatechin-3-gallate (EGCG), a component of green tea, reduces their oxidative stress and pathophysiology in mdx mice, a mild phenotype model of human Duchenne-type muscular dystrophy. EGCG (5 mg/kg body weight in saline) was injec...
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PMID: 18264714
PDF is available here.
Abstract
We have evaluated the possible role of glutathione monoester (GME), when administered intraperitoneally (12mg/kg body weight) for 20 days on age-associated changes in the levels of lipofuscin, Na+K+, Mg2+, Ca2+ ATPase activities and intracellular calcium levels in discrete brain regions of young and...
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PMID: 18242929
PDF is available here.
Abstract
In the foveola, FAF and NIA were relatively normal. Parafoveal loss of retinal pigment epithelium (RPE) was indicated by absent FAF and NIA. An area of reduced FAF and NIA surrounded the parafoveal region of RPE loss. In the adjacent area, FAF was increased and increased NIA marked the peripheral bo...
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PMID: 18080820
PDF is available here.
Abstract
Fundus autofluorescence (FAF) imaging is a novel imaging method that allows topographic mapping of lipofuscin distribution in the retinal pigment epithelium cell monolayer as well as of other fluorophores that may occur with disease in the outer retina and the subneurosensory space. Excessive accumu...
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PMID: 18327131
PDF is available here.
Abstract
An ecotoxicological protocol with caged mussels, Mytilus galloprovincialis, was developed to evaluate the potential impact of an offshore gas platform in the central Adriatic Sea. Reference organisms were collected on a seasonal basis from an unpolluted site and transplanted for four weeks in both t...
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PMID: 17897709
PDF is available here.
Abstract
A total of 40 urine samples from the 40 patients were cytologically analyzed, and RRTCs were found in 25 samples (25 of 40, 62.5%). These RRTCs showed clear or vacuolated cytoplasm, intracytoplasmic pigmented granules (hemosiderin or lipofuscin) and large nuclei with round to irregular nuclear conto...
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PMID: 18540293
PDF is available here.
Abstract
We report 27 cases of adults (23 females/4 males, average age 53 years old) presenting yellow-orange pigmented symmetric spots in the internal part of their superior eyelids, different from xanthelasma. Most of them were not aware of their orange palpebral spots (OPS). A systematic observation of ad...
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PMID: 18216481
PDF is available here.
Abstract
We have long promulgated the idea that microglial cells serve an entirely beneficial role in the central nervous system (CNS), not only as immunological sentinels to fend off potentially dangerous infections, but also as constitutively neuroprotective glia that help sustain neuronal function in the...
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PMID: 18508444
PDF is available here.