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https://www.readbyqxmd.com/read/29683947/encephalocraniocutaneous-lipomatosis
#1
Abhishek Bavle, Rikin Shah, Naina Gross, Theresa Gavula, Alejandro Ruiz-Elizalde, Klaas Wierenga, Rene McNall-Knapp
A 5-year-old boy presented with worsening headaches for 3 months. On examination, he was found to have a hairless fatty tissue nevus of the scalp (nevus psiloliparus), subcutaneous soft tissue masses on the right side of his face, neck, mandible and right buttock and epibulbar dermoid of the right eye (choristoma) (Figs. 1A, B). Magnetic resonance imaging revealed a large suprasellar mass, which was debulked and found to be a pilocytic astrocytoma. Testing was not performed for the BRAF/KIAA1549 fusion or BRAFV600E mutation...
April 20, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29681767/evaluation-of-lymphocyte-response-to-the-induced-oxidative-stress-in-a-cohort-of-ageing-subjects-including-semisupercentenarians-and-their-offspring
#2
Federico Sizzano, Sebastiano Collino, Ornella Cominetti, Daniela Monti, Paolo Garagnani, Rita Ostan, Chiara Pirazzini, Maria Giulia Bacalini, Daniela Mari, Giuseppe Passarino, Claudio Franceschi, Alessio Palini
The production of reactive oxygen species (ROS) may promote immunosenescence if not counterbalanced by the antioxidant systems. Cell membranes, proteins, and nucleic acids become the target of ROS and progressively lose their structure and functions. This process could lead to an impairment of the immune response. However, little is known about the capability of the immune cells of elderly individuals to dynamically counteract the oxidative stress. Here, the response of the main lymphocyte subsets to the induced oxidative stress in semisupercentenarians (CENT), their offspring (OFF), elderly controls (CTRL), and young individuals (YO) was analyzed using flow cytometry...
2018: Mediators of Inflammation
https://www.readbyqxmd.com/read/29681142/modeling-the-present-and-future-incidence-of-pediatric-hand-foot-and-mouth-disease-associated-with-ambient-temperature-in-mainland-china
#3
Qi Zhao, Shanshan Li, Wei Cao, De-Li Liu, Quan Qian, Hongyan Ren, Fan Ding, Gail Williams, Rachel Huxley, Wenyi Zhang, Yuming Guo
BACKGROUND: There is limited evidence about the association between ambient temperature and the incidence of pediatric hand, foot, and mouth disease (HFMD) nationwide in China. OBJECTIVES: We examined the childhood temperature-HFMD associations across mainland China, and we projected the change in HFMD cases due to projected temperature change by the 2090s. METHODS: Data on daily HFMD (children 0-14 y old) counts and weather were collected from 362 sites during 2009-2014...
April 20, 2018: Environmental Health Perspectives
https://www.readbyqxmd.com/read/29677359/essential-infantile-esotropia-potential-pathogenetic-role-of-extended-subcortical-neuroplasticity
#4
Michael C Brodsky
Essential infantile esotropia is generated by prenuclear visual pathways that increase esotonus and gradually drive the eyes into a convergent position. Contrary to the prevailing notion that infantile esotropia reflects a primary disturbance within the visual cortex, accumulating evidence suggests that infantile esotropia is generated by lower subcortical centers that subserve nasalward optokinesis. These phylogenetically older visuo-vestibular pathways include the nucleus of the optic tract, accessory optic system, inferior olive, cerebellar flocculus, and vestibular nucleus...
April 1, 2018: Investigative Ophthalmology & Visual Science
https://www.readbyqxmd.com/read/29675426/a-novel-method-for-drug-screen-to-regulate-g-protein-coupled-receptors-in-the-metabolic-network-of-alzheimer-s-disease
#5
Yang Li, Wei Zheng, Wuyun Qiqige, Shujuan Cao, Jishou Ruan, Yanping Zhang
Alzheimer's disease (AD) is a chronic and progressive neurodegenerative disorder and the pathogenesis of AD is poorly understood. G protein-coupled receptors (GPCRs) are involved in numerous key AD pathways and play a key role in the pathology of AD. To fully understand the pathogenesis of AD and design novel drug therapeutics, analyzing the connection between AD and GPCRs is of great importance. In this paper, we firstly build and analyze the AD-related pathway by consulting the KEGG pathway of AD and a mass of literature and collect 25 AD-related GPCRs for drug discovery...
2018: BioMed Research International
https://www.readbyqxmd.com/read/29673784/visual-texture-agnosia-in-dementia-with-lewy-bodies-and-alzheimer-s-disease
#6
Yuka Oishi, Toru Imamura, Tatsuo Shimomura, Kyoko Suzuki
BACKGROUND: Neuroimaging and some clinical studies have reported that the ventral visual pathway is relevant for visual texture recognition. Although a variety of visual deficits have been reported in patients with Alzheimer's disease (AD) and dementia with Lewy bodies (DLB), visual material identification and texture recognition have not been thoroughly examined. OBJECTIVES: To investigate visual texture recognition in patients with AD and DLB and to clarify the relationship between visual texture recognition and various visuoperceptual functions...
March 27, 2018: Cortex; a Journal Devoted to the Study of the Nervous System and Behavior
https://www.readbyqxmd.com/read/29673536/fluorescent-anesthetics
#7
Daniel J Emerson, Ivan J Dmochowski
Methods for using exogenous fluorophore and general anesthetic 1-aminoanthracene (1-AMA) and its photoactive derivative 1-azidoanthracene (1-AZA) are provided. 1-AMA potentiates GABAA chloride currents and immobilizes Xenopus laevis tadpoles. Cellular and tissue anesthetic distribution can be imaged for quantifying "on-pathway" and "off-pathway" targets. 1-AZA shares targets with 1-AMA and offers further optoanesthetic spatial and temporal control upon near-UV laser irradiation. Furthermore, 1-AZA adduction provides screening of possible relevant anesthetic protein targets and binding site characterization...
2018: Methods in Enzymology
https://www.readbyqxmd.com/read/29672888/a-novel-fibroblast-growth-factor-15-dependent-and-bile-acid-independent-promotion-of-liver-regeneration-in-mice
#8
Bo Kong, Runbin Sun, Mingxing Huang, Monica D Chow, Xiao-Bo Zhong, Wen Xie, Yi-Horng Lee, Grace L Guo
The role of intestine-derived factors in promoting liver regeneration after partial hepatectomy (PHx) are not entirely known, but bile acids (BAs) and fibroblast growth factor 15 (Fgf15) that is highly expressed in the mouse ileum could promote hepatocyte proliferation. Fgf15 strongly suppresses the synthesis of BAs and emerging evidence indicates that Fgf15 is important for liver regeneration. The mechanisms by which Fgf15 promotes liver regeneration are unclear, but Fgf15 may do so indirectly by reducing BA levels and/or directly by promoting cell proliferation...
April 19, 2018: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/29672668/distinct-effects-of-apathy-and-dopamine-on-effort-based-decision-making-in-parkinson-s-disease
#9
Campbell Le Heron, Olivia Plant, Sanjay Manohar, Yuen-Siang Ang, Matthew Jackson, Graham Lennox, Michele T Hu, Masud Husain
Effort-based decision-making is a cognitive process crucial to normal motivated behaviour. Apathy is a common and disabling complication of Parkinson's disease, but its aetiology remains unclear. Intriguingly, the neural substrates associated with apathy also subserve effort-based decision-making in animal models and humans. Furthermore, the dopaminergic system plays a core role in motivating effortful behaviour for reward, and its dysfunction has been proposed to play a crucial role in the aetiology of apathy in Parkinson's disease...
April 17, 2018: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/29670180/manipulating-nanostructure-to-simultaneously-improve-the-electrical-conductivity-and-strength-in-microalloyed-al-zr-conductors
#10
S Y Jiang, R H Wang
To elude the strength-electrical conductivity trade-off dilemma, a nanostructuring strategy was achieved in microalloyed Al-0.1wt.% Zr conductor by optimizing the processing route, leading to enhanced strength and simultaneously improved electrical conductivity. The nanostructural design involved ultrafine grains with coherent Al3 Zr nanoprecipitates dispersed within the grain interior. The key is to create intragranular coherent Al3 Zr nanoprecipitates with size of ~6 nm, which not only produce the highest precipitate hardening but also minimize the local strain field to reduce the scattering of electron motion...
April 18, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29668002/a-sharp-edge-based-acoustofluidic-chemical-signal-generator
#11
Po-Hsun Huang, Chung Yu Chan, Peng Li, Yuqi Wang, Nitesh Nama, Hunter Bachman, Tony Jun Huang
Resolving the temporal dynamics of cell signaling pathways is essential for regulating numerous downstream functions, from gene expression to cellular responses. Mapping these signaling pathways requires the exposure of cells to time-varying chemical signals; these are difficult to generate and control over a wide temporal range. Herein, we present an acoustofluidic chemical signal generator based on a sharp-edge-based micromixing strategy. The device, simply by modulating the driving signals of an acoustic transducer including the ON/OFF switching frequency, actuation time and duty cycle, is capable of generating both single-pulse and periodic chemical signals that are temporally controllable in terms of stimulation period, stimulation duration and duty cycle...
April 18, 2018: Lab on a Chip
https://www.readbyqxmd.com/read/29667812/turning-on-off-the-anti-tumor-effect-of-au-cluster-via-atomically-controlling-its-molecular-size
#12
Jiao Zhai, Yanwei Jia, Lina Zhao, Qing Yuan, Fuping Gao, Xiangchun Zhang, Pengju Cai, Liang Gao, Juanjuan Guo, Shuhong Yi, Zhifang Chai, Yuliang Zhao, Xueyun Gao
We reported two Au clusters with precisely controlled molecular size (Au5Peptide3 and Au22Peptide10) showing different anti-tumor effects. In vitro, both Au5Peptide3 and Au22Peptide10 were well taken up by human nasopharyngeal cancer cells (CNE1 cells). However, only Au5Peptide3 significantly induced CNE1 cell apoptosis. Further studies showed that CNE1 cells took up Au5Peptide3 (1.98*10-15 mol/cell) and 9% of them entered mitochondria (0.186*10-15 mol/cell). As a comparison, the uptake of Au22Peptide10 was only half the amount of Au5Peptide3 (1...
April 18, 2018: ACS Nano
https://www.readbyqxmd.com/read/29666248/study-of-protein-folding-under-native-conditions-by-rapidly-switching-the-hydrostatic-pressure-inside-an-nmr-sample-cell
#13
Cyril Charlier, T Reid Alderson, Joseph M Courtney, Jinfa Ying, Philip Anfinrud, Adriaan Bax
In general, small proteins rapidly fold on the timescale of milliseconds or less. For proteins with a substantial volume difference between the folded and unfolded states, their thermodynamic equilibrium can be altered by varying the hydrostatic pressure. Using a pressure-sensitized mutant of ubiquitin, we demonstrate that rapidly switching the pressure within an NMR sample cell enables study of the unfolded protein under native conditions and, vice versa, study of the native protein under denaturing conditions...
April 16, 2018: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/29661000/regulation-of-clathrin-mediated-endocytosis
#14
Marcel Mettlen, Ping-Hung Chen, Saipraveen Srinivasan, Gaudenz Danuser, Sandra L Schmid
Clathrin-mediated endocytosis (CME) is the major endocytic pathway in mammalian cells. It is responsible for the uptake of transmembrane receptors and transporters, for remodeling plasma membrane composition in response to environmental changes, and for regulating cell surface signaling. CME occurs via the assembly and maturation of clathrin-coated pits that concentrate cargo as they invaginate and pinch off to form clathrin-coated vesicles. In addition to the major coat proteins, clathrin triskelia and adaptor protein complexes, CME requires a myriad of endocytic accessory proteins and phosphatidylinositol lipids...
April 16, 2018: Annual Review of Biochemistry
https://www.readbyqxmd.com/read/29660314/nrf2-as-a-therapeutic-target-for-rheumatic-diseases
#15
REVIEW
María Luisa Ferrándiz, Josep Nacher-Juan, Maria José Alcaraz
Nuclear factor (erythroid-derived 2)-like 2 (Nrf2) is a master regulator of cellular protective processes. Rheumatic diseases are chronic conditions characterized by inflammation, pain, tissue damage and limitations in function. Main examples are rheumatoid arthritis, systemic lupus erythematosus, osteoarthritis and osteoporosis. Their high prevalence constitutes a major health problem with an important social and economic impact. A wide range of evidence indicates that Nrf2 may control different mechanisms involved in the physiopathology of rheumatic conditions...
April 13, 2018: Biochemical Pharmacology
https://www.readbyqxmd.com/read/29657092/the-critical-chemical-and-mechanical-regulation-of-folic-acid-on-neural-engineering
#16
Gloria B Kim, Yongjie Chen, Weibo Kang, Jinshan Guo, Russell Payne, Hui Li, Qiong Wei, Julianne Baker, Cheng Dong, Sulin Zhang, Pak Kin Wong, Elias B Rizk, Jiazhi Yan, Jian Yang
The mandate of folic acid supplementation in grained products has reduced the occurrence of neural tube defects by one third in the U.S since its introduction by the Food and Drug Administration in 1998. However, the advantages and possible mechanisms of action of using folic acid for peripheral nerve engineering and neurological diseases still remain largely elusive. Herein, folic acid is described as an inexpensive and multifunctional niche component that modulates behaviors in different cells in the nervous system...
April 3, 2018: Biomaterials
https://www.readbyqxmd.com/read/29649553/the-interplay-between-dose-and-immune-system-activation-determines-fungal-infection-outcome-in-the-african-malaria-mosquito-anopheles-gambiae
#17
Victoria L Rhodes, Matthew B Thomas, Kristin Michel
The Toll pathway is a central regulator of antifungal immunity in insects. In mosquitoes, the Toll pathway affects infections with the fungal entomopathogen, Beauveria bassiana, which is considered a potential mosquito biopesticide. We report here the use of B. bassiana strain I93-825 in Anopheles gambiae to analyze the impact of Toll pathway modulation on mosquito survival. Exposure to a narrow dose range of conidia by direct contact decreased mosquito longevity and median survival. In addition, fungal exposure dose correlated positively and linearly with hazard ratio...
April 9, 2018: Developmental and Comparative Immunology
https://www.readbyqxmd.com/read/29629106/influences-of-the-heme-lysine-crosslink-in-cytochrome-p460-over-redox-catalysis-and-nitric-oxide-sensitivity
#18
Avery C Vilbert, Jonathan D Caranto, Kyle M Lancaster
Ammonia (NH3 )-oxidizing bacteria (AOB) derive total energy for life from the multi-electron oxidation of NH3 to nitrite (NO2 - ). One obligate intermediate of this metabolism is hydroxylamine (NH2 OH), which can be oxidized to the potent greenhouse agent nitrous oxide (N2 O) by the AOB enzyme cytochrome (cyt) P460. We have now spectroscopically characterized a 6-coordinate (6c) {FeNO}7 intermediate on the NH2 OH oxidation pathway of cyt P460. This species has two fates: it can either be oxidized to the {FeNO}6 that then undergoes attack by NH2 OH to ultimately generate N2 O, or it can lose its axial His ligand, thus generating a stable, off-pathway 5-coordinate (5c) {FeNO}7 species...
January 14, 2018: Chemical Science
https://www.readbyqxmd.com/read/29626161/extracellular-atp-activates-hyaluronan-synthase-2-has2-in-epidermal-keratinocytes-via-p2y-2-ca-2-signaling-and-mapk-pathways
#19
Leena Rauhala, Tiina Jokela, Riikka Kärnä, Geneviève Bart, Piia Takabe, Sanna Oikari, Markku I Tammi, Sanna Pasonen-Seppänen, Raija H Tammi
Extracellular nucleotides are used as signaling molecules by several cell types. In epidermis, their release is triggered by insults such as ultraviolet radiation, barrier disruption and wounding and by specific nerve terminals firing. Increased synthesis of hyaluronan, a ubiquitous extracellular matrix glycosaminoglycan, also occurs in response to stress, leading to the attractive hypothesis that nucleotide signaling and hyaluronan synthesis could also be linked. In HaCaT keratinocytes ATP caused a rapid and strong but transient activation of hyaluronan synthase 2 ( HAS2 ) expression via PKC-, CaMKII-, MAPK- and CREB-dependent pathways by activating the purinergic P2Y2 receptor...
April 6, 2018: Biochemical Journal
https://www.readbyqxmd.com/read/29624490/identifying-potential-provider-and-environmental-contamination-on-a-clinical-biocontainment-unit-using-aerosolized-pathogen-simulants
#20
David G Drewry, Lauren M Sauer, Kathryn Shaw-Saliba, Jennifer Therkorn, Kaitlin Rainwater-Lovett, Thomas Pilholski, Brian T Garibaldi
The Johns Hopkins Hospital created a biocontainment unit (BCU) to care for patients with highly infectious diseases while assuring healthcare worker safety. Research to date for BCU protocols and practices are based on case reports and lessons learned from patient care and exercises. This study seeks to be the first to explore the influences of healthcare worker movement and personal protective equipment (PPE) doffing on the transport of simulant pathogen particles in a BCU. A cough device released 1 μm fluorescent polystyrene beads (PSLs) in the patient room...
March 2018: Health Security
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