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https://www.readbyqxmd.com/read/27910599/synthetic-plasma-edge-diagnostics-for-emc3-eirene-highlighted-for-wendelstein-7-x
#1
H Frerichs, F Effenberg, O Schmitz, C Biedermann, Y Feng, M Jakubowski, R König, M Krychowiak, J Lore, H Niemann, T S Pedersen, L Stephey, G A Wurden
Interpretation of spectroscopic measurements in the edge region of high-temperature plasmas can be a challenge since line of sight integration effects make direct interpretation in terms of quantitative, local emission strengths often impossible. The EMC3-EIRENE code-a 3D fluid edge plasma and kinetic neutral gas transport code-is a suitable tool for full 3D reconstruction of such signals. A versatile synthetic diagnostic module has been developed recently which allows the realistic 3D setup of various plasma edge diagnostics to be captured...
November 2016: Review of Scientific Instruments
https://www.readbyqxmd.com/read/27910524/spatially-resolved-thomson-scattering-measurements-of-the-transition-from-the-collective-to-the-non-collective-regime-in-a-laser-produced-plasma
#2
D B Schaeffer, C G Constantin, A S Bondarenko, E T Everson, C Niemann
We present optical Thomson scattering results that image for the first time in a single measurement the spatial transition from collective to non-collective scattering. Data were taken in the Phoenix laser laboratory at the University of California, Los Angeles. The Raptor laser was used to ablate a carbon plasma, which was diagnosed with the frequency-doubled Phoenix laser serving as a Thomson scattering probe. Scattered light was collected from the laser plasma up to 10 cm from the target surface and up to 10 us after ablation, and imaged with high spatial and spectral resolutions...
November 2016: Review of Scientific Instruments
https://www.readbyqxmd.com/read/27910389/overview-of-diagnostic-performance-and-results-for-the-first-operation-phase-in-wendelstein-7-x-invited
#3
M Krychowiak, A Adnan, A Alonso, T Andreeva, J Baldzuhn, T Barbui, M Beurskens, W Biel, C Biedermann, B D Blackwell, H S Bosch, S Bozhenkov, R Brakel, T Bräuer, B Brotas de Carvalho, R Burhenn, B Buttenschön, A Cappa, G Cseh, A Czarnecka, A Dinklage, P Drews, A Dzikowicka, F Effenberg, M Endler, V Erckmann, T Estrada, O Ford, T Fornal, H Frerichs, G Fuchert, J Geiger, O Grulke, J H Harris, H J Hartfuß, D Hartmann, D Hathiramani, M Hirsch, U Höfel, S Jabłoński, M W Jakubowski, J Kaczmarczyk, T Klinger, S Klose, J Knauer, G Kocsis, R König, P Kornejew, A Krämer-Flecken, N Krawczyk, T Kremeyer, I Książek, M Kubkowska, A Langenberg, H P Laqua, M Laux, S Lazerson, Y Liang, S C Liu, A Lorenz, A O Marchuk, S Marsen, V Moncada, D Naujoks, H Neilson, O Neubauer, U Neuner, H Niemann, J W Oosterbeek, M Otte, N Pablant, E Pasch, T Sunn Pedersen, F Pisano, K Rahbarnia, L Ryć, O Schmitz, S Schmuck, W Schneider, T Schröder, H Schuhmacher, B Schweer, B Standley, T Stange, L Stephey, J Svensson, T Szabolics, T Szepesi, H Thomsen, J-M Travere, H Trimino Mora, H Tsuchiya, G M Weir, U Wenzel, A Werner, B Wiegel, T Windisch, R Wolf, G A Wurden, D Zhang, A Zimbal, S Zoletnik
Wendelstein 7-X, a superconducting optimized stellarator built in Greifswald/Germany, started its first plasmas with the last closed flux surface (LCFS) defined by 5 uncooled graphite limiters in December 2015. At the end of the 10 weeks long experimental campaign (OP1.1) more than 20 independent diagnostic systems were in operation, allowing detailed studies of many interesting plasma phenomena. For example, fast neutral gas manometers supported by video cameras (including one fast-frame camera with frame rates of tens of kHz) as well as visible cameras with different interference filters, with field of views covering all ten half-modules of the stellarator, discovered a MARFE-like radiation zone on the inboard side of machine module 4...
November 2016: Review of Scientific Instruments
https://www.readbyqxmd.com/read/27906091/impact-of-prophylactic-administration-of-levosimendan-on-short-term-and-long-term-outcome-in-high-risk-patients-with-severely-reduced-left-ventricular-ejection-fraction-undergoing-cardiac-surgery-a-retrospective-analysis
#4
Philippe Grieshaber, Stella Lipp, Andreas Arnold, Gerold Görlach, Matthias Wollbrück, Peter Roth, Bernd Niemann, Jochen Wilhelm, Andreas Böning
BACKGROUND: Patients with severely reduced left-ventricular ejection fraction carry a high risk of morbidity and mortality after cardiac surgery. Levosimendan can be used prophylactically in these patients having shown positive effects on short-term outcome. However, effects on long-term outcome and patient subgroups benefiting the most are unknown. We aim to address these topics with real-life data from our clinical practice. METHODS: Two hundred eigthy eight patients with preoperative LVEF ≤ 35% underwent cardiac surgery with cardiopulmonary bypass between 2009 and 2013...
December 1, 2016: Journal of Cardiothoracic Surgery
https://www.readbyqxmd.com/read/27903679/the-bruton-s-tyrosine-kinase-btk-inhibitor-acalabrutinib-demonstrates-potent-on-target-effects-and-efficacy-in-two-mouse-models-of-chronic-lymphocytic-leukemia
#5
Sarah E M Herman, Arnau Montraveta, Carsten U Niemann, Helena Mora-Jensen, Michael Gulrajani, Fanny Krantz, Rose Mantel, Lisa L Smith, Fabienne McClanahan, Bonnie Harrington, Dolors Colomer, Todd Covey, John C Byrd, Raquel Izumi, Allard Kaptein, Roger Ulrich, Amy Johnson, Brian J Lannutti, Adrian Wiestner, Jennifer A Woyach
PURPOSE: Acalabrutinib (ACP-196) is a novel, potent, and highly selective BTK inhibitor, which binds covalently to Cys481 in the ATP-binding pocket of BTK. We sought to evaluate the anti-tumor effects of acalabrutinib treatment in two established mouse models of chronic lymphocytic leukemia (CLL). EXPERIMENTAL DESIGN: Two distinct mouse models were used, the TCL1 adoptive transfer model where leukemic cells from Eμ-TCL1 transgenic mice are transplanted into C57BL/6 mice, and the human NSG primary CLL xenograft model...
November 30, 2016: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
https://www.readbyqxmd.com/read/27902765/loss-of-cathepsin-b-and-l-leads-to-lysosomal-dysfunction-npc-like-cholesterol-sequestration-and-accumulation-of-the-key-alzheimer-s-proteins
#6
Stjepko Cermak, Marko Kosicek, Aleksandra Mladenovic-Djordjevic, Kosara Smiljanic, Selma Kanazir, Silva Hecimovic
Proper function of lysosomes is particularly important in neurons, as they cannot dilute accumulated toxic molecules and aggregates by cell division. Thus, impairment of lysosomal function plays an important role in neuronal degeneration and in the pathogenesis of numerous neurodegenerative diseases. In this work we analyzed how inhibition and/or loss of the major lysosomal proteases, the cysteine cathepsins B and L (CtsB/L), affects lysosomal function, cholesterol metabolism and degradation of the key Alzheimer's disease (AD) proteins...
2016: PloS One
https://www.readbyqxmd.com/read/27900365/genome-sequencing-in-a-case-of-niemann-pick-type-c
#7
Max Dougherty, John Lazar, Jason C Klein, Karina Diaz, Theodore Gobillot, Eli Grunblatt, Nicholas Hasle, Daniel Lawrence, Megan Maurano, Maria Nelson, Gregory Olson, Sanjay Srivatsan, Jay Shendure, C Dirk Keene, Thomas Bird, Marshall S Horwitz, Desiree A Marshall
Adult-onset Niemann-Pick disease type C (NPC) is an infrequent presentation of a rare neurovisceral lysosomal lipid storage disorder caused by autosomal recessive mutations in NPC1 (∼95%) or NPC2 (∼5%). Our patient was diagnosed at age 33 when he presented with a 10-yr history of difficulties in judgment, concentration, speech, and coordination. A history of transient neonatal jaundice and splenomegaly with bone marrow biopsy suggesting a lipid storage disorder pointed to NPC; biochemical ("variant" level cholesterol esterification) and ultrastructural studies in adulthood confirmed the diagnosis...
November 2016: Cold Spring Harbor Molecular Case Studies
https://www.readbyqxmd.com/read/27900236/identification-of-two-sulfated-cholesterol-metabolites-found-in-the-urine-of-a-patient-with-niemann-pick-disease-type-c-as-novel-candidate-diagnostic-markers
#8
Masamitsu Maekawa, Kaoru Omura, Shoutaro Sekiguchi, Takashi Iida, Daisuke Saigusa, Hiroaki Yamaguchi, Nariyasu Mano
In the urine of a Niemann-Pick disease type C (NPC) patient, we have identified three characteristic intense peaks that have not been observed in the urine of a 3β-hydroxysteroid-Δ(5)-C27-steroid dehydrogenase deficiency patient or a healthy infant and adult. Based on accurate masses of the protonated molecules, we focused on two of them as candidate NPC diagnostic markers. Two synthesized authentic preparations agreed with the two compounds found in NPC patient urine in regard to both chromatographic behavior and accurate masses of the deprotonated molecules...
2016: Mass Spectrometry
https://www.readbyqxmd.com/read/27898547/bilateral-congenital-iris-sphincter-agenesis-diagnosed-after-massive-bleeding-episode-during-repair-of-aneurysmal-dilation-of-patent-ductus-arteriosus
#9
Tomohiro Yamamoto, Martin Schmidt-Niemann, Ehrenfried Schindler
We report a rare case of an infant with both an aneurysmal dilation of the patent ductus arteriosus (PDA) and bilateral congenital iris sphincter agenesis. Her mydriasis without pupillary light reflex was first noted after a massive intraoperative bleeding episode during the PDA ligation. The assumption that the mydriasis was a sign of cerebral ischemia led to additional examinations and intensive medical therapies that in retrospect were unnecessary. This is the first reported case of combined aneurysmal dilation of a PDA and congenital iris sphincter agenesis in the anesthesia literature...
November 28, 2016: A & A Case Reports
https://www.readbyqxmd.com/read/27895719/drabal-novel-method-to-mine-large-high-throughput-screening-assays-using-bayesian-active-learning
#10
Othman Soufan, Wail Ba-Alawi, Moataz Afeef, Magbubah Essack, Panos Kalnis, Vladimir B Bajic
BACKGROUND: Mining high-throughput screening (HTS) assays is key for enhancing decisions in the area of drug repositioning and drug discovery. However, many challenges are encountered in the process of developing suitable and accurate methods for extracting useful information from these assays. Virtual screening and a wide variety of databases, methods and solutions proposed to-date, did not completely overcome these challenges. This study is based on a multi-label classification (MLC) technique for modeling correlations between several HTS assays, meaning that a single prediction represents a subset of assigned correlated labels instead of one label...
2016: Journal of Cheminformatics
https://www.readbyqxmd.com/read/27890675/synergistic-drug-combination-effectively-blocks-ebola-virus-infection
#11
Wei Sun, Shihua He, Carles Martínez-Romero, Jennifer Kouznetsova, Gregory Tawa, Miao Xu, Paul Shinn, Ethan Fisher, Yan Long, Omid Motabar, Shu Yang, Philip E Sanderson, Peter R Williamson, Adolfo García-Sastre, Xiangguo Qiu, Wei Zheng
Although a group of FDA-approved drugs were previously identified with activity against Ebola virus, most of them are not clinically useful because their human blood concentrations are not high enough to inhibit EBOV infection. We screened 795 unique three-drug combinations in an EBOV entry assay. Two sets of three-drug combinations, toremifene-mefloquine-posaconazole and toremifene-clarithromycin-posaconazole, were identified that effectively blocked EBOV entry and were further validated for inhibition of live EBOV infection...
November 24, 2016: Antiviral Research
https://www.readbyqxmd.com/read/27890640/an-mtdna-mutation-accelerates-liver-aging-by-interfering-with-the-ros-response-and-mitochondrial-life-cycle
#12
Jan Niemann, Cindy Johne, Susanne Schröder, Franziska Koch, Saleh M Ibrahim, Julia Schultz, Markus Tiedge, Simone Baltrusch
Mitochondrial dysfunction affects liver metabolism, but it remains unclear whether this interferes with normal liver aging. We investigated several mitochondrial pathways in hepatocytes and liver tissue from a conplastic mouse strain compared with the control C57BL/6NTac strain over 18 months of life. The C57BL/6NTac-mtNODLtJ mice differed from C57BL/6NTac mice by a point mutation in mitochondrial-encoded subunit 3 of cytochrome c oxidase. Young C57BL/6NTac-mtNODLtJ mice showed reduced mitochondrial metabolism but similar reactive oxygen species (ROS) production to C57BL/6NTac mice...
November 24, 2016: Free Radical Biology & Medicine
https://www.readbyqxmd.com/read/27888692/mitochondrial-gsh-replenishment-as-a-potential-therapeutic-approach-for-niemann-pick-type-c-disease
#13
Sandra Torres, Nuria Matías, Anna Baulies, Susana Nuñez, Cristina Alarcon-Vila, Laura Martinez, Natalia Nuño, Anna Fernandez, Joan Caballeria, Thierry Levade, Alba Gonzalez-Franquesa, Pablo Garcia-Rovés, Elisa Balboa, Silvana Zanlungo, Gemma Fabrías, Josefina Casas, Carlos Enrich, Carmen Garcia-Ruiz, José C Fernández-Checa
Niemann Pick type C (NPC) disease is a progressive lysosomal storage disorder caused by mutations in genes encoding NPC1/NPC2 proteins, characterized by neurological defects, hepatosplenomegaly and premature death. While the primary biochemical feature of NPC disease is the intracellular accumulation of cholesterol and gangliosides, predominantly in endolysosomes, mitochondrial cholesterol accumulation has also been reported. As accumulation of cholesterol in mitochondria is known to impair the transport of GSH into mitochondria, resulting in mitochondrial GSH (mGSH) depletion, we investigated the impact of mGSH recovery in NPC disease...
November 20, 2016: Redox Biology
https://www.readbyqxmd.com/read/27884455/-acid-sphingomyelinase-deficiency-niemann-pick-disease-type-b-in-adulthood-a-retrospective-multicentric-study-of-28%C3%A2-adult-cases
#14
O Lidove, N Belmatoug, R Froissart, C Lavigne, I Durieu, K Mazodier, C Serratrice, C Douillard, C Goizet, P Cathebras, G Besson, Z Amoura, A Tazi, M Gatfossé, S Rivière, T Sené, M T Vanier, J-M Ziza
INTRODUCTION: Acid sphingomyelinase deficiency (ASMD) is an autosomal recessive disease with a clinical spectrum ranging from a neurovisceral infantile form (Niemann-Pick disease type A) to a chronic visceral form also encountered in adults (Niemann-Pick disease type B, NP-B). METHODS: Retrospective multicentric analysis of French adult patients with ASMD over the period 1985-March 2015. Clinical, biological, and imaging data were analyzed. RESULTS: Twenty-eight patients (19 males, 9 females) were analyzed...
November 22, 2016: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/27877888/lysosomal-ph-inducible-supramolecular-dissociation-of-polyrotaxanes-possessing-acid-labile-n-triphenylmethyl-end-groups-and-their-therapeutic-potential-for-niemann-pick-type-c-disease
#15
Atsushi Tamura, Kei Nishida, Nobuhiko Yui
Niemann-Pick type C (NPC) disease is characterized by the accumulation of cholesterol in lysosomes. We have previously reported that biocleavable polyrotaxanes (PRXs) composed of β-cyclodextrins (β-CDs) threaded onto a linear polymer capped with bulky stopper molecules via intracellularly cleavable linkers show remarkable cholesterol reducing effects in NPC disease patient-derived fibroblasts owing to the stimuli-responsive intracellular dissociation of PRXs and subsequent β-CD release from the PRXs. Herein, we describe a series of novel acid-labile 2-(2-hydroxyethoxy)ethyl group-modified PRXs (HEE-PRXs) bearing terminal N-triphenylmethyl (N-Trt) groups as a cleavable component for the treatment of NPC disease...
2016: Science and Technology of Advanced Materials
https://www.readbyqxmd.com/read/27876313/lysosomal-acid-lipase-deficiency-expanding-differential-diagnosis
#16
Vassili Valayannopoulos, Eugen Mengel, Anais Brassier, Gregory Grabowski
The differential diagnoses for metabolic liver diseases may be challenging in clinical settings, which represents a critical issue for disorders such as lysosomal acid lipase deficiency (LAL-D). LAL-D is caused by deficient activity of the LAL enzyme, resulting in the accumulation of cholesteryl esters and triglycerides throughout the body, predominately in the liver, spleen, gastrointestinal tract, and blood vessel walls. LAL-D is a progressive, multi-organ disease with early mortality and significant morbidity characterized by a combination of hepatic dysfunction and dyslipidemia...
November 10, 2016: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/27871250/drug-resistance-and-population-structure-of-m-tuberculosis-isolates-from-prisons-and-communities-in-ethiopia
#17
Solomon Ali, Patrick Beckert, Abraham Haileamlak, Andreas Wieser, Michael Pritsch, Norbert Heinrich, Thomas Löscher, Michael Hoelscher, Stefan Niemann, Andrea Rachow
BACKGROUND: The population structure and drug resistance pattern of Mycobacterium tuberculosis complex (MTBC) isolates in Ethiopian prisons and some communities is still unknown. METHODS: A comparative cross sectional study was conducted on 126 MTBC strains isolated from prisons and communities in southwestern, southern and eastern Ethiopia. Phenotypic drug susceptibility testing was performed with the MGIT960 system. Combined 24-loci Mycobacterium interspersed repetitive unit-variable number tandem repeat and spacer oligonucleotide typing methods were used to study the MTBC population structure...
November 21, 2016: BMC Infectious Diseases
https://www.readbyqxmd.com/read/27865842/decreasing-smpd1-activity-in-beas-2b-bronchial-airway-epithelial-cells-results-in-increased-nrf2-activity-cytokine-synthesis-and-neutrophil-recruitment
#18
Elyse MacFadden-Murphy, Lucie Roussel, Guy Martel, Julie Bérubé, Simon Rousseau
Niemann-Pick disease (NPD) type B is a rare autosomal recessive disease characterized by variable levels of impairment in sphingomyelin phosphodiesterase 1 (SMPD1) activity. Lung involvement is the most important prognostic factor in NPD-B, with recurrent respiratory infections starting in infancy being the major cause of morbidity and mortality. We hypothesized that decreased SMPD1 activity impaired airway epithelium host defense response. SMPD1 activity was reduced using inducible shRNA. Surprisingly, decreasing SMPD1 activity by 50%, resulted in increased neutrophil recruitment, both at baseline and in response to bacterial stimulation...
November 16, 2016: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/27860245/telocytes-a-potential-defender-in-the-spleen-of-npc1-mutant-mice
#19
Bichao Zhang, Ciqing Yang, Liang Qiao, Qiuling Li, Congrui Wang, Xin Yan, Juntang Lin
Niemann-Pick disease, type C1 (Npc1), is an atypical lysosomal storage disorder caused by autosomal recessive inheritance of mutations in Npc1 gene. In the Npc1 mutant mice (Npc1(-/-) ), the initial manifestation is enlarged spleen, concomitant with free cholesterol accumulation. Telocytes (TCs), a novel type of interstitial cell, exist in a variety of tissues including spleen, presumably thought to be involved in many biological processes such as nursing stem cells and recruiting inflammatory cells. In this study, we found that the spleen is significantly enlarged in Npc1(-/-) mice, and the results from transmission electron microscopy examination and immunostaining using three different TCs markers, c-Kit, CD34 and Vimentin revealed significantly increased splenic TCs in Npc1(-/-) mice...
November 18, 2016: Journal of Cellular and Molecular Medicine
https://www.readbyqxmd.com/read/27858506/causes-of-prehospital-misinterpretations-of-st-elevation-myocardial-infarction
#20
Nichole Bosson, Stephen Sanko, Ronald E Stickney, James Niemann, William J French, James G Jollis, Michael C Kontos, Tyson G Taylor, Peter W Macfarlane, Richard Tadeo, William Koenig, Marc Eckstein
OBJECTIVES: To determine the causes of software misinterpretation of ST elevation myocardial infarction (STEMI) compared to clinically identified STEMI to identify opportunities to improve prehospital STEMI identification. METHODS: We compared ECGs acquired from July 2011 through June 2012 using the LIFEPAK 15 on adult patients transported by the Los Angeles Fire Department. Cases included patients ≥18 years who received a prehospital ECG. Software interpretation of the ECG (STEMI or not) was compared with data in the regional EMS registry to classify the interpretation as true positive (TP), true negative (TN), false positive (FP), or false negative (FN)...
November 18, 2016: Prehospital Emergency Care
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