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Lipofuscin

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https://www.readbyqxmd.com/read/28024876/neuronal-ceroid-lipofuscinosis-ncl-is-caused-by-the-entire-deletion-of-cln8-in-the-alpenl%C3%A3-ndische-dachsbracke-dog
#1
M Hirz, M Drögemüller, A Schänzer, V Jagannathan, E Dietschi, H H Goebel, W Hecht, S Laubner, M J Schmidt, F Steffen, M Hilbe, K Köhler, C Drögemüller, C Herden
Neuronal ceroid lipofuscinoses (NCLs) are inherited lysosomal storage diseases that have been described in a variety of dog breeds, where they are caused by different mutations in different genes. However, the causative gene defect in the breed Alpenländische Dachsbracke remained unknown so far. Here we present two confirmed cases of NCL in Alpenländische Dachsbracke dogs from different litters of the same sire with a different dam harboring the same underlying novel mutation in the CLN8 gene. Case 1, a 2-year-old male Alpenländische Dachsbracke was presented with neurological signs including disorientation, character changes including anxiety states and aggressiveness, sudden blindness and reduction of food intake...
December 19, 2016: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/28005672/discordance-between-blue-light-autofluorescence-and-near-infrared-autofluorescence-in-age-related-macular-degeneration
#2
Michael J Heiferman, Amani A Fawzi
PURPOSE: To identify the origin and significance of discordance between blue-light autofluorescence (BL-AF; 488 nm) and near-infrared autofluorescence (NI-AF; 787 nm) in patients with age-related macular degeneration (AMD). METHODS: A total of 86 eyes of 59 patients with a diagnosis of AMD were included in this cross-sectional study conducted between March 9, 2015 and May 1, 2015. A masked observer examined the BL-AF, NI-AF, and spectral-domain optical coherence tomography images...
December 2016: Retina
https://www.readbyqxmd.com/read/28005671/hyperspectral-autofluorescence-imaging-of-drusen-and-retinal-pigment-epithelium-in-donor-eyes-with-age-related-macular-degeneration
#3
Yuehong Tong, Tal Ben Ami, Sungmin Hong, Rainer Heintzmann, Guido Gerig, Zsolt Ablonczy, Christine A Curcio, Thomas Ach, R Theodore Smith
PURPOSE: To elucidate the molecular pathogenesis of age-related macular degeneration (AMD) and interpretation of fundus autofluorescence imaging, the authors identified spectral autofluorescence characteristics of drusen and retinal pigment epithelium (RPE) in donor eyes with AMD. METHODS: Macular RPE/Bruch membrane flat mounts were prepared from 5 donor eyes with AMD. In 12 locations (1-3 per eye), hyperspectral autofluorescence images in 10-nm-wavelength steps were acquired at 2 excitation wavelengths (λex 436, 480 nm)...
December 2016: Retina
https://www.readbyqxmd.com/read/27999666/beclin-1-mediated-autophagy-may-be-involved-in-the-elderly-cognitive-and-affective-disorders-in-streptozotocin-induced-diabetic-mice
#4
Zhu-Fei Guan, Xiu-Ling Zhou, Xiao-Ming Zhang, Yu Zhang, Yan-Mei Wang, Qi-Lin Guo, Gang Ji, Guo-Feng Wu, Na-Na Wang, Hao Yang, Zhong-Yu Yu, Hou-Guang Zhou, Jing-Chun Guo, Ying-Chao Liu
BACKGROUND: Diabetes is the most common metabolic disease with many chronic complications, and cognitive disorders are one of the common complications in patients with diabetes. Previous studies have showed that autophagy played important roles in the progression of metabolic syndrome, diabetes and other diseases. So we investigated whether aged diabetic mice are prone to be associated with the cognitive and affective disorders and whether Beclin-1-mediated autophagy might be involved in thepahological process...
2016: Translational Neurodegeneration
https://www.readbyqxmd.com/read/27992857/tfeb-ameliorates-the-impairment-of-the-autophagy-lysosome-pathway-in-neurons-induced-by-doxorubicin
#5
Jose Felix Moruno-Manchon, Ndidi-Ese Uzor, Shelli R Kesler, Jeffrey S Wefel, Debra M Townley, Archana Sidalaghatta Nagaraja, Sunila Pradeep, Lingegowda S Mangala, Anil K Sood, Andrey S Tsvetkov
Doxorubicin, a commonly used chemotherapy agent, induces severe cardio- and neurotoxicity. Molecular mechanisms of cardiotoxicity have been extensively studied, but mechanisms by which doxorubicin exhibits its neurotoxic properties remain unclear. Here, we show that doxorubicin impairs neuronal autophagy, leading to the accumulation of an autophagy substrate p62. Neurons treated with doxorubicin contained autophagosomes, damaged mitochondria, and lipid droplets. The brains from mice treated with pegylated liposomal doxorubicin exhibited autophagosomes, often with mitochondria, lipofuscin, and lipid droplets...
December 16, 2016: Aging
https://www.readbyqxmd.com/read/27978557/understanding-rpe-lipofuscin
#6
R Theodore Smith, Sina Farsiu, Michael Allingham
No abstract text is available yet for this article.
December 1, 2016: Investigative Ophthalmology & Visual Science
https://www.readbyqxmd.com/read/27931218/scutellaria-barbata-flavonoids-alleviate-memory-deficits-and-neuronal-injuries-induced-by-composited-a%C3%AE-in-rats
#7
Xiao G Wu, Shu S Wang, Hong Miao, Jian J Cheng, Shu F Zhang, Ya Z Shang
BACKGROUND: The aim of the present study was to investigate the effects of Scutellaria barbata flavonoids (SBF) on memory impairment and neuronal injury induced by amyloid beta protein 25-35 in combination with aluminum trichloride (AlCl3) and recombinant human transforming growth factor-β1 (RHTGF-β1) (composited Aβ) in rats. METHODS: The composited Aβ-treated model of Alzheimer's disease (AD)-like memory impairment and neuronal injury was established in male rats by right intracerebroventricular injection of composited Aβ, and the effects of SBF were assessed using this rat model...
December 8, 2016: Behavioral and Brain Functions: BBF
https://www.readbyqxmd.com/read/27911422/-diagnostic-potential-of-optical-coherence-tomography-for-small-choroidal-melanomas
#8
A F Brovkina, A S Stoyukhina, I V Musatkina
AIM: To investigate the sequence of OCT manifestations in choroidal melanoma (CM) with account to its growth. MATERIAL AND METHODS: A total of 28 CM patients were examined. Of them, 18 patients with early CM, 10 - with medium-sized CM. The mean patient age at diagnosis was 60.54±5.31 years. Tumor location was macular (15 eyes) or perimacular (13 eyes), its prominence varied from 0.98 to 4.9 mm and basal diameter - from 5.08 to 18.9 mm. Additionally to OCT, tumor autofluorescence was studied in all cases...
2016: Vestnik Oftalmologii
https://www.readbyqxmd.com/read/27909686/lipofuscins-prepared-by-modification-of-photoreceptor-cells-via-glycation-or-lipid-peroxidation-show-the-similar-phototoxicity
#9
Alexander Dontsov, Anna Koromyslova, Mikhail Ostrovsky, Natalia Sakina
AIM: To investigate the effect of two ways of lipofuscin production (lipid peroxidation and glycation) on lipofuscin fluorescence characteristics and phototoxicity and to compare them with the properties of natural lipofuscin. METHODS: Model lipofuscins were prepared on the basis of bovine photoreceptor outer segments (POS) with bisretinoid A2E addition. One set of samples was prepared from POS modified by lipid peroxidation, while another set from POS modified by glycation with fructose...
November 20, 2016: World Journal of Experimental Medicine
https://www.readbyqxmd.com/read/27881166/using-the-social-amoeba-dictyostelium-to-study-the-functions-of-proteins-linked-to-neuronal-ceroid-lipofuscinosis
#10
REVIEW
Robert J Huber
Neuronal ceroid lipofuscinosis (NCL), also known as Batten disease, is a debilitating neurological disorder that affects both children and adults. Thirteen genetically distinct genes have been identified that when mutated, result in abnormal lysosomal function and an excessive accumulation of ceroid lipofuscin in neurons, as well as other cell types outside of the central nervous system. The NCL family of proteins is comprised of lysosomal enzymes (PPT1/CLN1, TPP1/CLN2, CTSD/CLN10, CTSF/CLN13), proteins that peripherally associate with membranes (DNAJC5/CLN4, KCTD7/CLN14), a soluble lysosomal protein (CLN5), a protein present in the secretory pathway (PGRN/CLN11), and several proteins that display different subcellular localizations (CLN3, CLN6, MFSD8/CLN7, CLN8, ATP13A2/CLN12)...
November 24, 2016: Journal of Biomedical Science
https://www.readbyqxmd.com/read/27853905/an-antidiabetic-polyherbal-phytomedicine-confers-stress-resistance-and-extends-lifespan-in-caenorhabditis-elegans
#11
Laxmi Rathor, Aakanksha Pant, Harshika Awasthi, Dayanandan Mani, Rakesh Pandey
An Ayurvedic polyherbal extract (PHE) comprising six herbs viz. Berberis aristata, Cyperus rotundus, Cedrus deodara, Emblica officinalis, Terminalia chebula and Terminalia bellirica is mentioned as an effective anti-hyperglycemic agent in 'Charaka Samhita', the classical text of Ayurveda. Previously, antidiabetic drug metformin was found to elicit antiaging effects and PHE was also found to exhibit antidiabetic effects in humans. Therefore, we screened it for its in vivo antioxidant antiaging effect on stress and lifespan using human homologous Caenorhabditis elegans model system...
November 16, 2016: Biogerontology
https://www.readbyqxmd.com/read/27847630/clinical-applications-of-fundus-autofluorescence-in-retinal-disease
#12
REVIEW
Madeline Yung, Michael A Klufas, David Sarraf
Fundus autofluorescence (FAF) is a non-invasive retinal imaging modality used in clinical practice to provide a density map of lipofuscin, the predominant ocular fluorophore, in the retinal pigment epithelium. Multiple commercially available imaging systems, including the fundus camera, the confocal scanning laser ophthalmoscope, and the ultra-widefield imaging device, are available to the clinician. Each offers unique advantages for evaluating various retinal diseases. The clinical applications of FAF continue to expand...
2016: International Journal of Retina and Vitreous
https://www.readbyqxmd.com/read/27843235/flat-choroidal-melanoma-masquerading-as-central-serous-chorioretinopathy
#13
Timothy Patrick Higgins, Chloe T L Khoo, George Magrath, Carol L Shields
There are several mimickers of choroidal melanoma. We report a patient with recent family stress who developed blurred vision to 20/50 OD and was found to have unilateral central serous chorioretinopathy and a coincidental choroidal nevus. After 1 year without resolution of the subretinal fluid, the patient was referred for our opinion. On examination, visual acuity was 20/50 in the right eye and 20/20 in the left eye. The left eye was normal. Evaluation of the right eye showed a small, pigmented submacular choroidal lesion measuring 4 mm × 3 mm...
September 2016: Oman Journal of Ophthalmology
https://www.readbyqxmd.com/read/27826111/effect-of-ranching-time-on-melanomacrophage-centres-in-anterior-kidney-and-spleen-of-southern-bluefin-tuna-thunnus-maccoyii
#14
Daryl Evans, Barbara Nowak
Melanomacrophage centres (MMCs) are common in the haemopoietic tissue of fish and are an important component of the humoral immune response. Increasingly they are being studied as a biomarker of environmental stress in both wild and aquaculture fish stocks. Computer image analysis of histological sections of the head kidney and spleen of ranched Southern bluefin tuna (SBT) was used to investigate changes in MMCs over various stages of the ranching cycle. Histochemical pigment differentiation indicated haemosiderin, lipofuscin and melanin were present in MMCs of both the kidney and spleen, with kidney MMCs being highly positive for lipofuscin and melanin and spleen having high levels of haemosiderin and a lower level of melanin than kidney...
December 2016: Fish & Shellfish Immunology
https://www.readbyqxmd.com/read/27824490/cost-effective-elimination-of-lipofuscin-fluorescence-from-formalin-fixed-brain-tissue-by-white-phosphor-light-emitting-diode-array
#15
Yulong Sun, Avi Chakrabartty
Autofluorescence of aldehyde-fixed tissues greatly hinders fluorescence microscopy. In particular, lipofuscin, an autofluorescent component of aged brain tissue, complicates fluorescence imaging of tissue in neurodegenerative diseases. Background and lipofuscin fluorescence can be reduced by greater than 90% through photobleaching using white phosphor light emitting diode arrays prior to treatment with fluorescent probes. We compared the effect of photobleaching versus established chemical quenchers on the quality of fluorescent staining in formalin-fixed brain tissue of frontotemporal dementia with tau-positive inclusions...
December 2016: Biochemistry and Cell Biology, Biochimie et Biologie Cellulaire
https://www.readbyqxmd.com/read/27824154/manganese-mediated-acceleration-of-age-related-hearing-loss-in-mice
#16
Nobutaka Ohgami, Ichiro Yajima, Machiko Iida, Xiang Li, Reina Oshino, Mayuko Y Kumasaka, Masashi Kato
Despite the fact that manganese (Mn) is known to be a neurotoxic element relevant to age-related disorders, the risk of oral exposure to Mn for age-related hearing loss remains unclear. In this study, we orally exposed wild-type young adult mice to Mn (Mn-exposed WT-mice) at 1.65 and 16.50 mg/L for 4 weeks. Mn-exposed WT-mice showed acceleration of age-related hearing loss. Mn-exposed WT-mice had neurodegeneration of spiral ganglion neurons (SGNs) with increased number of lipofuscin granules. Mn-exposed WT-mice also had increased hypoxia-inducible factor-1 alpha (Hif-1α) protein with less hydroxylation at proline 564 and decreased c-Ret protein in SGNs...
November 8, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27812872/sudan-black-b-the-specific-histochemical-stain-for-lipofuscin-a-novel-method-to-detect-senescent-cells
#17
Konstantinos Evangelou, Vassilis G Gorgoulis
The Sudan-Black-B (SBB) histochemical stain is well known to specifically react against lipofuscin, an aggregate of oxidized proteins, lipids, and metals. Lipofuscin is related to many ageing processes. It is also known to accumulate in senescent cells. We recently proved that lipofuscin detection, when applying the SBB staining, is highly specific for the visualization of senescent cells. Here, we present in detail this SBB method that can detect senescent cells in any material, irrespective of its preparation...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/27796896/the-cutaneous-telocytes
#18
Catalin G Manole, Olga Simionescu
Telocytes (TCs) are interstitial cells found in stroma of many organs, including the skin dermis. Ultrastructurally, normal skin TCs recapitulates all the previously documented features in interstitum of other organs. Their (ultra)structural hallmark is the presence of particular shaped cellular prolongations (termed telopodes), along other features as cellular organelles representation and their distribution within cell body and its prolongations. Transmission electron microscopy (TEM) or high magnification light microscopy indicated that the particular shape of telopodes alternate characteristically thin segments (termed podomeres) and dilated segments (called podoms)...
2016: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/27778018/neuronal-ceroid-lipofuscinosis-in-an-adult-american-staffordshire-terrier
#19
Anna Nolte, Aimara Bello, Michaela Drögemüller, Tosso Leeb, Eva Brockhaus, Wolfgang Baumgärtner, Peter Wohlsein
A female, 5-year-old American Staffordshire Terrier with severe progressive neurological deficits, particularly in terms of ataxia and keeping balance, was examined pathomorphologically and a genetic analysis was performed. In neurons of various localizations of the central nervous system an accumulation of a finely granular pale eosinophilic or light brown material was found. In addition, the cerebellum revealed marked degeneration and loss of Purkinje and inner granule cells. The accumulated PAS-positive, argyrophilic, autofluorescent material showed ultrastructurally a lamellar appearance suggestive of lipofuscin...
December 5, 2016: Tierärztliche Praxis. Ausgabe K, Kleintiere/Heimtiere
https://www.readbyqxmd.com/read/27773704/membrane-damage-by-betulinic-acid-provides-insights-into-cellular-aging
#20
Waleska K Martins, Andreza B Gomide, Érico T Costa, Helena C Junqueira, Beatriz S Stolf, Rosangela Itri, Maurício S Baptista
BACKGROUND: Cell senescence is a process of central importance to the understanding of aging as well as to the development of new drugs. It is related with genomic instability, which has been shown to occur in the presence of autophagy deficiency. Yet, the mechanism that triggers genomic instability and senescence from a condition of autophagy deficiency remains unknown. By analyzing the consequences of treating human keratinocytes (HaCaT) with the pentacyclic triterpenoid Betulinic Acid (BA) we were able to propose that cell senescence can develop as a response to parallel damage in the membranes of mitochondria and lysosome...
January 2017: Biochimica et Biophysica Acta
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