2,285 research outputs found

    Converging Role for REEP1/SPG31 in Oxidative Stress

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    Mutations in the receptor expression-enhancing protein 1 gene (REEP1) are associated with hereditary spastic paraplegia type 31 (SPG31), a neurological disorder characterized by lengthdependent degeneration of upper motor neuron axons. Mitochondrial dysfunctions have been observed in patients harboring pathogenic variants in REEP1, suggesting a key role of bioenergetics in disease-related manifestations. Nevertheless, the regulation of mitochondrial function in SPG31 remains unclear. To elucidate the pathophysiology underlying REEP1 deficiency, we analyzed in vitro the impact of two different mutations on mitochondrial metabolism. Together with mitochondrial morphology abnormalities, loss-of-REEP1 expression highlighted a reduced ATP production with increased susceptibility to oxidative stress. Furthermore, to translate these findings from in vitro to preclinical models, we knocked down REEP1 in zebrafish. Zebrafish larvae showed a significant defect in motor axon outgrowth leading to motor impairment, mitochondrial dysfunction, and reactive oxygen species accumulation. Protective antioxidant agents such as resveratrol rescued free radical overproduction and ameliorated the SPG31 phenotype both in vitro and in vivo. Together, our findings offer new opportunities to counteract neurodegeneration in SPG31

    Validation tests of the CMS TIB/TID structures

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    Tracker Inner Barrel half-cylinders and Tracker Inner Disks of the CMS tracker have been integrated in three INFN sites. Integrated structures are submitted to an extensive set of tests whose main aim is to validate the functioning of the structures in CMS-like conditions. The tests have furthermore proven to be a great opportunity to study several aspects of the performance in detail. In this note the tests are described in some detail and an overview of the results is presented

    Environmental awareness gained during a citizen science project in touristic resorts is maintained after 3 years since participation

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    The research leading to these results has received funding from Project AWARE Foundation, ASTOI Association, Milano, Ministry of Tourism of the Arab Republic of Egypt, Settemari S. p. A Tour Operator, Scuba Nitrox Safety International, Viaggio nel Blu Diving Center.Tourism is one of the largest economic sectors in the world. It has a positive effect on the economy of many countries, but it can also lead to negative impacts on local ecosystems. Informal environmental education through Citizen Science (CS) projects can be effective in increasing citizen environmental knowledge and awareness in the short-term. A change of awareness could bring to a behavioral change in the long-term, making tourism more sustainable. However, the long-term effects of participating in CS projects are still unknown. This is the first follow-up study concerning the effects of participating in a CS project on cognitive and psychological aspects at the basis of pro-environmental behavior. An environmental education program was developed, between 2012 and 2013, in a resort in Marsa Alam, Egypt. The study directly evaluated, through paper questionnaires, the short-term (after 1 week or 10 days) retention of knowledge and awareness of volunteers that had participated in the activities proposed by the program. After three years, participants were re-contacted via email to fill in the same questionnaire as in the short-term study, plus a new section with psychological variables. 40.5% of the re-contacted participants completed the follow-up questionnaires with a final sample size of fifty-five people for this study. Notwithstanding the limited sample size, positive trends in volunteer awareness, personal satisfaction regarding the CS project, and motivation to engage in pro-environmental behavior in the long-term were observed.Publisher PDFPeer reviewe

    Tracker Operation and Performance at the Magnet Test and Cosmic Challenge

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    During summer 2006 a fraction of the CMS silicon strip tracker was operated in a comprehensive slice test called the Magnet Test and Cosmic Challenge (MTCC). At the MTCC, cosmic rays detected in the muon chambers were used to trigger the readout of all CMS sub-detectors in the general data acquisition system and in the presence of the 4 T magnetic field produced by the CMS superconducting solenoid. This document describes the operation of the Tracker hardware and software prior, during and after data taking. The performance of the detector as resulting from the MTCC data analysis is also presented

    Measurement of hadronic shower punchthrough in magnetic field

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    The total punchthrough probability of showers produced by negative pions, positive pions, positive kaons and protons, has been measured as a function of depth in an absorber in a magnetic field ranging from 0 to 3 Tesla. The incident particle momentum varied from 10 to 300 GeV/c. The lateral shower development and particle multiplicity at several absorber depths have been determined. The measurements are compared with the predictions of Monte Carlo simulation programs

    Status report of the RD5 experiment

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    Differential cross section measurements for the production of a W boson in association with jets in proton–proton collisions at √s = 7 TeV

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    Measurements are reported of differential cross sections for the production of a W boson, which decays into a muon and a neutrino, in association with jets, as a function of several variables, including the transverse momenta (pT) and pseudorapidities of the four leading jets, the scalar sum of jet transverse momenta (HT), and the difference in azimuthal angle between the directions of each jet and the muon. The data sample of pp collisions at a centre-of-mass energy of 7 TeV was collected with the CMS detector at the LHC and corresponds to an integrated luminosity of 5.0 fb[superscript −1]. The measured cross sections are compared to predictions from Monte Carlo generators, MadGraph + pythia and sherpa, and to next-to-leading-order calculations from BlackHat + sherpa. The differential cross sections are found to be in agreement with the predictions, apart from the pT distributions of the leading jets at high pT values, the distributions of the HT at high-HT and low jet multiplicity, and the distribution of the difference in azimuthal angle between the leading jet and the muon at low values.United States. Dept. of EnergyNational Science Foundation (U.S.)Alfred P. Sloan Foundatio

    Penilaian Kinerja Keuangan Koperasi di Kabupaten Pelalawan

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    This paper describe development and financial performance of cooperative in District Pelalawan among 2007 - 2008. Studies on primary and secondary cooperative in 12 sub-districts. Method in this stady use performance measuring of productivity, efficiency, growth, liquidity, and solvability of cooperative. Productivity of cooperative in Pelalawan was highly but efficiency still low. Profit and income were highly, even liquidity of cooperative very high, and solvability was good
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