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https://www.readbyqxmd.com/read/28430167/induced-pluripotent-stem-cell-modeling-of-gaucher-s-disease-what-have-we-learned
#1
REVIEW
Dino Matias Santos, Gustavo Tiscornia
Gaucher's disease (GD) is the most frequently inherited lysosomal storage disease, presenting both visceral and neurologic symptoms. Mutations in acid β-glucocerebrosidase disrupt the sphingolipid catabolic pathway promoting glucosylceramide (GlcCer) accumulation in lysosomes. Current treatment options are enzyme replacement therapy (ERT) and substrate reduction therapy (SRT). However, neither of these approaches is effective in treating the neurological aspect of the disease. The use of small pharmacological compounds that act as molecular chaperones is a promising approach that is still experimental...
April 21, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28429928/a-dual-enzyme-hydrogen-peroxide-generation-machinery-in-hydrogels-supports-antimicrobial-wound-treatment
#2
Daniela Huber, Gregor Tegl, Anna Mensah, Bianca Beer, Martina Baumann, Nicole Borth, Christoph Sygmund, Roland Ludwig, Georg M Guebitz
The aging population and accompanying diseases like diabetes resulted in an increased occurrence of chronic wounds. Topical wound treatment with antimicrobial agents to inhibit bacterial invasion and promote wound healing is often associated with difficulties. Here, we investigated the potential of succinyl chitosan (SC) - carboxymethyl cellulose (CMC) hydrogels which constantly release clinically relevant levels of hydrogen peroxide (H2O2). CMC hydrogel matrix was in-situ converted by limited hydrolysis by a cellulase into substrates accepted by cellobiose dehydrogenase (CDH) for continuous production of H2O2 (30 µM over 24 h)...
April 21, 2017: ACS Applied Materials & Interfaces
https://www.readbyqxmd.com/read/28428634/lipid-droplets-and-liver-disease-from-basic-biology-to-clinical-implications
#3
REVIEW
Nina L Gluchowski, Michel Becuwe, Tobias C Walther, Robert V Farese
Lipid droplets are dynamic organelles that store neutral lipids during times of energy excess and serve as an energy reservoir during deprivation. Many prevalent metabolic diseases, such as the metabolic syndrome or obesity, often result in abnormal lipid accumulation in lipid droplets in the liver, also called hepatic steatosis. Obesity-related steatosis, or NAFLD in particular, is a major public health concern worldwide and is frequently associated with insulin resistance and type 2 diabetes mellitus. Here, we review the latest insights into the biology of lipid droplets and their role in maintaining lipid homeostasis in the liver...
April 21, 2017: Nature Reviews. Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28428354/multiplex-tandem-mass-spectrometry-enzymatic-activity-assay-for-newborn-screening-of-the-mucopolysaccharidoses-and-type-2-neuronal-ceroid-lipofuscinosis
#4
Yang Liu, Fan Yi, Arun Babu Kumar, Naveen Kumar Chennamaneni, Xinying Hong, C Ronald Scott, Michael H Gelb, Frantisek Turecek
BACKGROUND: We expanded the use of tandem mass spectrometry combined with liquid chromatography (LC-MS/MS) for multiplex newborn screening of seven lysosomal enzymes in dried blood spots (DBS). The new assays are for enzymes responsible for the mucopolysaccharidoses (MPS-I, -II, -IIIB, -IVA, -VI, and -VII) and type 2 neuronal ceroid lipofuscinosis (LINCL). METHODS: New substrates were prepared and char-acterized for tripeptidyl peptidase 1 (TPP1), α-N-acetylglucosaminidase (NAGLU), and lysosomal β-glucuronidase (GUSB)...
April 20, 2017: Clinical Chemistry
https://www.readbyqxmd.com/read/28427536/health-risks-of-food-oxidation
#5
Mario Estévez, Zhuqing Li, Olugbenga P Soladoye, Thomas Van-Hecke
The impact of dietary habits on our health is indisputable. Consumer's concern on aging and age-related diseases challenges scientists to underline the potential role of food on the extension and guarantee of lifespan and healthspan. While some dietary components and habits are generally regarded as beneficial for our health, some others are being found to exert potential toxic effects and hence, contribute to the onset of particular health disorders. Among the latter, lipid and protein oxidation products formed during food production, storage, processing, and culinary preparation have been recently identified as potentially harmful to humans...
2017: Advances in Food and Nutrition Research
https://www.readbyqxmd.com/read/28427477/plasma-chitotriosidase-activity-versus-ccl18-level-for-assessing-type-i-gaucher-disease-severity-protocol-for-a-systematic-review-with-meta-analysis-of-individual-participant-data
#6
Tatiana Raskovalova, Patrick B Deegan, Ruby Yang, Elena Pavlova, Jérome Stirnemann, José Labarère, Ari Zimran, Pramod K Mistry, Marc Berger
BACKGROUND: Gaucher disease (GD) is an autosomal recessive lysosomal storage disorder caused by deficiency in acid beta-glucosidase. GD exhibits a wide clinical spectrum of disease severity with an unpredictable natural course. Plasma chitotriosidase activity and CC chemokine ligand 18 (CCL18) have been exchangeably used for monitoring GD activity and response to enzyme replacement therapy in conjunction with clinical assessment. Yet, a large-scale head-to-head comparison of these two biomarkers is currently lacking...
April 20, 2017: Systematic Reviews
https://www.readbyqxmd.com/read/28426266/physicochemical-characteristics-of-the-seed-and-seed-oil-of-the-potentially-medicinal-plant-ziziphus-oenoplia
#7
Hosakatte Niranjana Murthy, Kadanthottu Sebastian Joseph, Abhishek Madiwal, Dinesh Rajen Gerald, Mahananda Badiger, Lakshmi Kolkar, Reshma Hiremath
The proximate composition of seeds, physicochemical characteristics, and fatty acid profiles of Ziziphus oenoplia seed oil were determined in this study. The seeds possessed low moisture (4.54%) and high carbohydrate (42.96%) and protein content (40%), making the seed oil suitable for storage and consumption. The saponification value (197.80) of the seed oil makes it a promising source for the soap and shampoo industry. The iodine and saponification values are comparable to those of major edible seed oils such as groundnut and soybean...
April 20, 2017: Journal of Dietary Supplements
https://www.readbyqxmd.com/read/28423886/adiponectin-induced-ampk-impairment-mediates-insulin-resistance-in-bama-mini-pig-fed-high-fat-and-high-sucrose-diet
#8
Miaomiao Niu, Lei Xiang, Yaqian Liu, Yuqiong Zhao, Jifang Yuan, Xin Dai, Hua Chen
Objective: Adipose tissue is no longer considered as an inert storage organ for lipid, but instead is thought to play an active role in regulating insulin effects via secretion adipokines. However, conflicting reports have emerged regarding the effects of adipokines. In this study, we investigated the role of adipokines in glucose metabolism and insulin sensitivity in obese Bama mini-pigs. Methods: An obesity model was established in Bama mini-pigs, by feeding with high-fat and high-sucrose diet for 30 weeks...
March 25, 2017: Asian-Australasian Journal of Animal Sciences
https://www.readbyqxmd.com/read/28422289/dietary-tuna-dark-muscle-protein-attenuates-hepatic-steatosis-and-increases-serum-high-density-lipoprotein-cholesterol-in-obese-type-2-diabetic-obese-kk-a-y-mice
#9
Hayato Maeda, Ryota Hosomi, Mari Fukuda, Yuki Ikeda, Munehiro Yoshida, Kenji Fukunaga
Tuna muscle consists of light and dark muscle in approximately equal proportions. However, besides for the light muscle of tuna, cod, sardine, and salmon, few researches have assessed the health-promoting functions of fish protein. Therefore, we evaluated the mechanisms underlying the alteration of lipid storage and cholesterol metabolism following the intake of tuna dark muscle protein (TDMP) by obese type-2 diabetic/obese mice. Four-week-old male KK-A(y) mice were separated into 2 dietary groups, with one group receiving a casein-based diet and the other receiving a diet with the substitution of part of the protein (50%, w/w) by TDMP (TDMP diet) for 4 wk...
April 19, 2017: Journal of Food Science
https://www.readbyqxmd.com/read/28421605/cholinergic-glutamatergic-co-transmission-in-striatal-cholinergic-interneurons-new-mechanisms-regulating-striatal-computation
#10
REVIEW
Ornela Kljakic, Helena Janickova, Vania F Prado, Marco A M Prado
It is well established that neurons secrete neuropeptides and ATP with classical neurotransmitters; however, certain neuronal populations are also capable of releasing two classical neurotransmitters by a process named co-transmission. Although there has been progress in our understanding of the molecular mechanism underlying co-transmission, the individual regulation of neurotransmitter secretion and the functional significance of this neuronal 'bilingualism' is still unknown. Striatal cholinergic interneurons (CINs) have been shown to secrete glutamate (Glu) in addition to acetylcholine (ACh) and are recognized for their role in the regulation of striatal circuits and behavior...
April 18, 2017: Journal of Neurochemistry
https://www.readbyqxmd.com/read/28421568/prions-prionoid-complexes-and-amyloids-the-bad-the-good-and-something-in-between
#11
Iva Hafner Bratkovič
Prions are infectious agents causing transmissible spongiform encephalopathies in humans and animals. These protein-based particles template conformational changes in a host-encoded prion protein to an insoluble self-like conformation. Prions are also present in yeast, where they support protein-based epigenetic inheritance. There is emerging evidence that prion-like (prionoid) particles can support a variety of pathological and beneficial functions. The recent data on the prionoid spread of other pathological amyloids are discussed in light of differences between prions and prion-like aggregates...
April 19, 2017: Swiss Medical Weekly
https://www.readbyqxmd.com/read/28421508/appendix-d-irvine-scientific-%C3%A2-vitrification-system
#12
Matthew VerMilyea, Amber Brewer
This chapter will describe the use of the Irvine Scientific(®) vitrification and warming solutions (Vit Kits(®)) along with a detailed protocol for the correct use of the CryoTip(®) vitrification device for human embryos and oocytes. Successful pregnancies have been reported after carrying out rapid vitrification methods of oocytes, cleavage stage embryos, and blastocysts using the CryoTip(®) (Popwell et al. Fertil Steril, 101:e20, 2014; Kuwayama et al. Reprod Biomed 11:608-614, 2005; Kuwayama et al. Fertil Steril 84:S187, 2005; Kuwayama et al...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28421368/recombinant-foot-and-mouth-disease-virus-fmdv-non-structural-protein-3a-fused-to-enhanced-green-fluorescent-protein-egfp-as-a-candidate-probe-to-identify-fmdv-infected-cattle-in-serosurveys
#13
Cecilia M Lotufo, Ingrid E Bergmann, Nora M Mattion, Maximiliano Wilda, Pablo R Grigera
Recombinant protein 3A-EGFP, a fusion construct between foot-and-mouth disease virus (FMDV) non-structural protein 3A and the enhanced green fluorescent protein (EGFP) was expressed in BL21-DE3 cells. The identity of the partially purified protein 3A-EGFP was confirmed by its reactivity with sera from cattle infected with FMDV and with a monoclonal antibody specific for FMDV-3ABC (MAb3H7) in Western blot assays. No reactivity was observed with sera from uninfected vaccinated animals. The performance of 3A-EGFP as an antigen in an indirect enzyme-linked immunosorbent assay (ELISA) was assessed and compared with that of a previously developed and validated capture ELISA that uses a 3ABC recombinant antigen (3ABC ELISA) and has been widely applied for serological surveys in Argentina...
April 18, 2017: Archives of Virology
https://www.readbyqxmd.com/read/28421048/functional-information-stored-in-the-conserved-structural-rna-domains-of-flavivirus-genomes
#14
REVIEW
Alba Fernández-Sanlés, Pablo Ríos-Marco, Cristina Romero-López, Alfredo Berzal-Herranz
The genus Flavivirus comprises a large number of small, positive-sense single-stranded, RNA viruses able to replicate in the cytoplasm of certain arthropod and/or vertebrate host cells. The genus, which has some 70 member species, includes a number of emerging and re-emerging pathogens responsible for outbreaks of human disease around the world, such as the West Nile, dengue, Zika, yellow fever, Japanese encephalitis, St. Louis encephalitis, and tick-borne encephalitis viruses. Like other RNA viruses, flaviviruses have a compact RNA genome that efficiently stores all the information required for the completion of the infectious cycle...
2017: Frontiers in Microbiology
https://www.readbyqxmd.com/read/28420123/stability-of-bioactive-compounds-in-broccoli-as-affected-by-cutting-styles-and-storage-time
#15
Ana Mariel Torres-Contreras, Vimal Nair, Luis Cisneros-Zevallos, Daniel A Jacobo-Velázquez
Broccoli contains bioactive molecules and thus its consumption is related with the prevention of chronic and degenerative diseases. The application of wounding stress to horticultural crops is a common practice, since it is the basis for the fresh-cut produce industry. In this study, the effect of four different cutting styles (CSs) (florets (CS1), florets cut into two even pieces (CS2), florets cut into four even pieces (CS3), and florets processed into chops (CS4)) and storage time (0 and 24 h at 20 °C) on the content of bioactive compounds in broccoli was evaluated...
April 15, 2017: Molecules: a Journal of Synthetic Chemistry and Natural Product Chemistry
https://www.readbyqxmd.com/read/28418743/stockpile-model-of-personal-protective-equipment-in-taiwan
#16
Yu-Ju Chen, Po-Jung Chiang, Yu-Hsin Cheng, Chun-Wei Huang, Hui-Yun Kao, Chih-Kai Chang, Hsun-Miao Huang, Pei-Yin Liu, Jen-Hsin Wang, Yi-Chien Chih, Shu-Mei Chou, Chin-Hui Yang, Chang-Hsun Chen
The Taiwan Centers for Disease Control (Taiwan CDC) has established a 3-tier personal protective equipment (PPE) stockpiling framework that could maintain a minimum stockpile for the surge demand of PPE in the early stage of a pandemic. However, PPE stockpiling efforts must contend with increasing storage fees and expiration problems. In 2011, the Taiwan CDC initiated a stockpile replacement model in order to optimize the PPE stockpiling efficiency, ensure a minimum stockpile, use the government's limited funds more effectively, and achieve the goal of sustainable management...
March 2017: Health Security
https://www.readbyqxmd.com/read/28418018/neurodevelopmental-changes-in-excitatory-synaptic-structure-and-function-in-the-cerebral-cortex-of-sanfilippo-syndrome-iiia-mice
#17
Chrissa A Dwyer, Samantha L Scudder, Ying Lin, Lara E Dozier, Dustin Phan, Nicola J Allen, Gentry N Patrick, Jeffrey D Esko
Sanfilippo syndrome, MPS IIIA-D, results from deficits in lysosomal enzymes that specifically degrade heparan sulfate, a sulfated glycosaminoglycan. The accumulation of heparan sulfate results in neurological symptoms, culminating in extensive neurodegeneration and early death. To study the impact of storage in postnatal neurodevelopment, we examined murine models of MPS IIIA, which lack the enzyme sulfamidase. We show that changes occur in excitatory postsynaptic structure and function in the somatosensory cortex prior to signs of neurodegeneration...
April 18, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28417336/treatment-of-depression-in-adults-with-fabry-disease
#18
Nadia Ali, Scott Gillespie, Dawn Laney
Fabry disease (FD) is a genetic X-linked, multisystemic, progressive lysosomal storage disorder (LSD). Depression has emerged as a disease complication, with prevalence estimates ranging from 15 to 62%. This is a pilot study examining the effects of psychological counseling for depression in FD on depression, adaptive functioning (AF), quality of life (QOL), and subjective pain experience. Telecounseling was also piloted, as it has beneficial effects in other chronic diseases which make in-person counseling problematic...
April 18, 2017: JIMD Reports
https://www.readbyqxmd.com/read/28416815/in-situ-programming-of-leukaemia-specific-t-cells-using-synthetic-dna-nanocarriers
#19
Tyrel T Smith, Sirkka B Stephan, Howell F Moffett, Laura E McKnight, Weihang Ji, Diana Reiman, Emmy Bonagofski, Martin E Wohlfahrt, Smitha P S Pillai, Matthias T Stephan
An emerging approach for treating cancer involves programming patient-derived T cells with genes encoding disease-specific chimeric antigen receptors (CARs), so that they can combat tumour cells once they are reinfused. Although trials of this therapy have produced impressive results, the in vitro methods they require to generate large numbers of tumour-specific T cells are too elaborate for widespread application to treat cancer patients. Here, we describe a method to quickly program circulating T cells with tumour-recognizing capabilities, thus avoiding these complications...
April 17, 2017: Nature Nanotechnology
https://www.readbyqxmd.com/read/28415883/electrosteric-stealth-rivastigmine-loaded-liposomes-for-brain-targeting-preparation-characterization-ex-vivo-bio-distribution-and-in-vivo-pharmacokinetic-studies
#20
Sara Nageeb El-Helaly, Ahmed Abd Elbary, Mohamed A Kassem, Mohamed A El-Nabarawi
Being one of the highly effective drugs in treatment of Alzheimer's disease, Rivastigmine brain targeting is highly demandable, therefore liposomal dispersion of Rivastigmine was prepared containing 2 mol% PEG-DSPE added to Lecithin, Didecyldimethyl ammonium bromide (DDAB), Tween 80 in 1:0.02:0.25 molar ratio. A major challenge during the preparation of liposomes is maintaining a stable formulation, therefore the aim of our study was to increase liposomal stability by addition of DDAB to give an electrostatic stability and PEG-DSPE to increase stability by steric hindrance, yielding what we called an electrosteric stealth (ESS) liposomes...
November 2017: Drug Delivery
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