12 research outputs found

    Human MCTS1-dependent translation of JAK2 is essential for IFN-γ immunity to mycobacteria.

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    Human inherited disorders of interferon-gamma (IFN-γ) immunity underlie severe mycobacterial diseases. We report X-linked recessive MCTS1 deficiency in men with mycobacterial disease from kindreds of different ancestries (from China, Finland, Iran, and Saudi Arabia). Complete deficiency of this translation re-initiation factor impairs the translation of a subset of proteins, including the kinase JAK2 in all cell types tested, including T lymphocytes and phagocytes. JAK2 expression is sufficiently low to impair cellular responses to interleukin-23 (IL-23) and partially IL-12, but not other JAK2-dependent cytokines. Defective responses to IL-23 preferentially impair the production of IFN-γ by innate-like adaptive mucosal-associated invariant T cells (MAIT) and γδ T lymphocytes upon mycobacterial challenge. Surprisingly, the lack of MCTS1-dependent translation re-initiation and ribosome recycling seems to be otherwise physiologically redundant in these patients. These findings suggest that X-linked recessive human MCTS1 deficiency underlies isolated mycobacterial disease by impairing JAK2 translation in innate-like adaptive T lymphocytes, thereby impairing the IL-23-dependent induction of IFN-γ

    Waste management optimization based on research in the manufacturing company Gumres

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    Celem artykułu jest przedstawienie problematyki związanej z gospodarowaniem odpadami oraz próby optymalizacji gospodarki odpadami w przedsiębiorstwie produkcyjnym Gumres. Aby zrealizować to zamierzenie, wykorzystano dane z dokumentów przedsiębiorstwa, obejmujących zakres ewidencji odpadów, oraz z informacji uzyskanych podczas wywiadu pogłębionego. Ponadto autorzy dokonali przeglądu literatury dotyczącej rozważanego tematu. Przedstawiono zagadnienie odpadów w ujęciu teoretycznym, a także prawne aspekty gospodarowania odpadami. W szczególności opisano organizację gospodarki odpadami. Zasadnicza część artykułu poświęcona jest badaniom własnym dotyczącym procesu modernizacji gospodarki odpadami w wybranym przedsiębiorstwie. Zaprezentowano profil firmy, wskazano identyfikację powstających odpadów oraz propozycję modernizacji gospodarki odpadami. W ostatniej części opracowania wyszczególniono działania naprawcze o charakterze optymalizacyjnym i wnioski końcowe.The aim of this article is to present the issues related to waste management and the attempts of the manufacturing company Gumres to optimize waste management. In order to achieve this goal, data were used from the company’s documents covering waste records and from information obtained during an in-depth interview. In addition, the authors reviewed the literature on waste management. Waste was presented in theoretical terms and the legal aspects of waste management were also considered. The organization of waste management is described. The main part of the article is devoted to the authors’ own research on the process of modernization of waste management in a selected company. The profile of the selected company is presented, the identification of the waste generated and the proposal for modernization of waste management are indicated. In the last part of the paper, corrective measures to optimize waste management and final conclusions are detailed

    Search for light sterile neutrinos with the STEREO experiment

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    The stereo experiment is searching for a non-standard oscillation in the propagation of anti-neutrinos produced by a nuclear reactor which could be the sign for the existence of a sterile state of eV mass and the origin of the reactor anti-neutrino anomaly. In this paper, results from 66 days of reactor turned on and 138 days of reactor turned off are reported excluding large amplitude oscillations. A special focus is put on the data analysis and studies of correlated backgrounds. In particular the origin of the correlated background measured in reactor turned off periods is discussed. This background mainly originates from neutrons produced by cosmic radiation

    Search for light sterile neutrinos with the STEREO experiment

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    International audienceThe stereo experiment is searching for a non-standard oscillation in the propagation of anti-neutrinos produced by a nuclear reactor which could be the sign for the existence of a sterile state of eV mass and the origin of the reactor anti-neutrino anomaly. In this paper, results from 66 days of reactor turned on and 138 days of reactor turned off are reported excluding large amplitude oscillations. A special focus is put on the data analysis and studies of correlated backgrounds. In particular the origin of the correlated background measured in reactor turned off periods is discussed. This background mainly originates from neutrons produced by cosmic radiation

    Improved STEREO simulation with a new gamma ray spectrum of excited gadolinium isotopes using FIFRELIN

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    International audienceThe STEREO experiment measures the electron antineutrino spectrum emitted in a research reactor using the inverse beta decay reaction on H nuclei in a gadolinium loaded liquid scintillator. The detection is based on a signal coincidence of a prompt positron and a delayed neutron capture event. The simulated response of the neutron capture on gadolinium is crucial for the comparison with data, in particular in the case of the detection efficiency. Among all stable isotopes,155^{155}Gd and157^{157}Gd have the highest cross sections for thermal neutron capture. The excited nuclei after the neutron capture emit gamma rays with a total energy of about 8MeV. The complex level schemes of156^{156}Gd and158^{158}Gd are a challenge for the modeling and prediction of the deexcitation spectrum, especially for compact detectors where gamma rays can escape the active volume. With a new description of the Gd (n, γ \gamma ) cascades obtained using the FIFRELIN code, the agreement between simulation and measurements with a neutron calibration source was significantly improved in the STEREO experiment. A database of ten millions of deexcitation cascades for each isotope has been generated and is now available for the user

    First antineutrino energy spectrum from 235^{235}U fissions with the STEREO detector at ILL

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    International audienceThis article reports the measurement of the 235U-induced antineutrino spectrum shape by the Stereo experiment. 43 000 antineutrinos have been detected at about 10 m from the highly enriched core of the ILL reactor during 118 full days equivalent at nominal power. The measured inverse beta decay spectrum is unfolded to provide a pure 235U spectrum in antineutrino energy. A careful study of the unfolding procedure, including a cross-validation by an independent framework, has shown that no major biases are introduced by the method. A significant local distortion is found with respect to predictions around E ν ≃ 5.3 MeV. A Gaussian fit of this local excess leads to an amplitude of A = 12.1 ± 3.4% (3.5σ)

    Improved Sterile Neutrino Constraints from the STEREO Experiment with 179 Days of Reactor-On Data

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    The STEREO experiment is a very short baseline reactor antineutrino experiment. It is designed to test the hypothesis of light sterile neutrinos being the cause of a deficit of the observed antineutrino interaction rate at short baselines with respect to the predicted rate, known as the Reactor Antineutrino Anomaly. The STEREO experiment measures the antineutrino energy spectrum in six identical detector cells covering baselines between 9 and 11 m from the compact core of the ILL research reactor. In this article, results from 179 days of reactor turned on and 235 days of reactor turned off are reported in unprecedented detail. The current results include improvements in the description of the optical model of the detector, the gamma-cascade after neutron captures by gadolinium, the treatment of backgrounds, and the statistical method of the oscillation analysis. Using a direct comparison between antineutrino interaction rates of all cells, independent of any flux prediction, we find the data compatible with the null oscillation hypothesis. The best-fit point of the Reactor Antineutrino Anomaly is rejected at more than 99.9% C.L

    The genome of the sea urchin Strongylocentrotus purpuratus.

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    International audienceWe report the sequence and analysis of the 814-megabase genome of the sea urchin Strongylocentrotus purpuratus, a model for developmental and systems biology. The sequencing strategy combined whole-genome shotgun and bacterial artificial chromosome (BAC) sequences. This use of BAC clones, aided by a pooling strategy, overcame difficulties associated with high heterozygosity of the genome. The genome encodes about 23,300 genes, including many previously thought to be vertebrate innovations or known only outside the deuterostomes. This echinoderm genome provides an evolutionary outgroup for the chordates and yields insights into the evolution of deuterostomes
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