316 research outputs found

    Scholarly publishing: digital dreams or nightmares?

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    The world of scholarly publishing is a complex one. At one end of the scholarly publishing spectrum are the annual multi-million dollar profits of STM global publishers such as Reed Elsevier; at the other the plight of university presses in Australia

    Students Creating Their Own Thinking-Learning Contexts

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    This paper focuses on two high school students and their thoughts and feelings as they engaged in a topic of their choosing during a two-month summer action research program. Their high school astronomy teacher monitored their choice of topic and progress. The students engaged in authentic tasks and materials couched in problem-oriented formats within meaningful learning contexts designed to foster thinking and learning. These students worked as a team, but pursued individual paths of inquiry using critical and imaginative thinking, and engaged in social and solitary contexts that involved them in writing, intervening, and reflecting on ideas gleaned from conversations and readings (electronic and conventional) with a university educator and an astronomer/educator during their self-directed case-based research. The process engaged students in formal skills such as written communication, literacy, logic, and calculation using an innovative electronic interactive network. Evaluations of timed writings, concept maps, notebook entries, and vee diagrams are presented and discussed

    Pathogenic Variants in Fucokinase Cause a Congenital Disorder of Glycosylation

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    FUK encodes fucokinase, the only enzyme capable of converting L-fucose to fucose-1-phosphate, which will ultimately be used for synthesizing GDP-fucose, the donor substrate for all fucosyltransferases. Although it is essential for fucose salvage, this pathway is thought to make only a minor contribution to the total amount of GDP-fucose. A second pathway, the major de novo pathway, involves conversion of GDP-mannose to GDP-fucose. Here we describe two unrelated individuals who have pathogenic variants in FUK and who presented with severe developmental delays, encephalopathy, intractable seizures, and hypotonia. The first individual was compound heterozygous for c.667T>C (p.Ser223Pro) and c.2047C>T (p.Arg683Cys), and the second individual was homozygous for c.2980A>C (p.Lys994Gln). Skin fibroblasts from the first individual confirmed the variants as loss of function and showed significant decreases in total GDP-[3H] fucose and [3H] fucose-1-phosphate. There was also a decrease in the incorporation of [5,6-3H]-fucose into fucosylated glycoproteins. Lys994 has previously been shown to be an important site for ubiquitin conjugation. Here, we show that loss-of-function variants in FUK cause a congenital glycosylation disorder characterized by a defective fucose-salvage pathway

    Thymus transplantation for complete DiGeorge syndrome: European experience

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    Background: Thymus transplantation is a promising strategy for the treatment of athymic complete DiGeorge syndrome (cDGS). Methods: Twelve patients with cDGS were transplanted with allogeneic cultured thymus. Objective: To confirm and extend the results previously obtained in a single centre. Results: Two patients died of pre-existing viral infections without developing thymopoeisis and one late death occurred from autoimmune thrombocytopaenia. One infant suffered septic shock shortly after transplant resulting in graft loss and the need for a second transplant. Evidence of thymopoeisis developed from 5-6 months after transplantation in ten patients. The median (range) of circulating naïve CD4 counts (x10663 /L) were 44(11-440) and 200(5-310) at twelve and twenty-four months post-transplant and T-cell receptor excision circles were 2238 (320-8807) and 4184 (1582 -24596) per106 65 T-cells. Counts did not usually reach normal levels for age but patients were able to clear pre-existing and later acquired infections. At a median of 49 months (22-80), eight have ceased prophylactic antimicrobials and five immunoglobulin replacement. Histological confirmation of thymopoeisis was seen in seven of eleven patients undergoing biopsy of transplanted tissue including five showing full maturation through to the terminal stage of Hassall body formation. Autoimmune regulator (AIRE) expression was also demonstrated. Autoimmune complications were seen in 7/12 patients. In two, early transient autoimmune haemolysis settled after treatment and did not recur. The other five suffered ongoing autoimmune problems including: thyroiditis (3); haemolysis (1), thrombocytopaenia (4) and neutropenia (1). Conclusions: This study confirms the previous reports that thymus transplantation can reconstitute T cells in cDGS but with frequent autoimmune complications in survivors

    LunaH-Map: Revealing Lunar Water with a New Radiation Sensor Array

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    A new type of neutron and gamma-ray spectrometer called the Miniature Neutron Spectrometer (Mini-NS) has been developed, assembled, qualified and delivered as part of the Lunar Polar Hydrogen Mapper (LunaH-Map) cubesat mission. The LunaH-Map spacecraft is currently manifested as a secondary payload on the Space Launch System (SLS) Artemis-1 rocket. LunaH-Map will deploy from Artemis-1 and enter a low altitude perilune elliptical orbit around the Moon. The Mini-NS will measure the lunar epithermal neutron albedo, and measurements around perilune will be used to produce maps of hydrogen enrichments and depletions across the lunar South Pole region including both within and outside of permanently shadowed regions (PSRs). The Min-NS was designed to achieve twice the epithermal neutron count rate of the Lunar Prospector Neutron Spectrometer (LP-NS). The instrument response was characterized through the collection of pre-flight neutron counting data with a Cf-252 neutron source at Arizona State University across hundreds of power cycles, as well as across the expected temperature range. The instrument spatial response was characterized at the Los Alamos National Laboratories (LANL) Neutron Free In-Air Facility. The LunaH-Map Mini-NS was designed to fit within the cubesat form-factor and uses two detectors with eight sensor heads that can be operated independently. For future missions with different science goals that can be achieved with epithermal neutron detection, the number of Mini-NS sensor heads can easily be modified without requiring a complete re-design and re-qualification

    A randomised-controlled trial investigating potential underlying mechanisms of a functionality-based approach to improving women’s body image

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    Focusing on body functionality is a promising technique for improving women's body image. This study replicates prior research in a large novel sample, tests longer-term follow-up effects, and investigates underlying mechanisms of these effects (body complexity and body-self integration). British women (N = 261) aged 18–30 who wanted to improve their body image were randomised to Expand Your Horizon (three online body functionality writing exercises) or an active control. Trait body image was assessed at Pretest, Posttest, 1-week, and 1-month Follow-Up. To explore whether changes in body complexity and body-self integration ‘buffer’ the impact of negative body-related experiences, participants also completed beauty-ideal media exposure. Relative to the control, intervention participants experienced improved appearance satisfaction, functionality satisfaction, body appreciation, and body complexity at Posttest, and at both Follow-Ups. Neither body complexity nor body-self integration mediated intervention effects. Media exposure decreased state body satisfaction among intervention and control participants, but neither body complexity nor body-self integration moderated these effects. The findings underscore the value of focusing on body functionality for improving body image and show that effects persist one month post-intervention

    Measurement of the CP-violating phase ϕs in B0s→J/ψϕ decays in ATLAS at 13 TeV

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    A measurement of the B0s→J/ψϕ decay parameters using 80.5fb−1 of integrated luminosity collected with the ATLAS detector from 13 TeV proton–proton collisions at the LHC is presented. The measured parameters include the CP-violating phase ϕs, the width difference ΔΓs between the B0s meson mass eigenstates and the average decay width Γs. The values measured for the physical parameters are combined with those from 19.2fb−1 of 7 and 8 TeV data, leading to the following: ϕs=−0.087±0.036 (stat.)±0.021 (syst.) rad ΔΓs=0.0657±0.0043 (stat.)±0.0037 (syst.) ps−1 Γs=0.6703±0.0014 (stat.)±0.0018 (syst.) ps−1 Results for ϕs and ΔΓs are also presented as 68% confidence level contours in the ϕs–ΔΓs plane. Furthermore the transversity amplitudes and corresponding strong phases are measured. ϕs and ΔΓs measurements are in agreement with the Standard Model predictions.publishedVersio

    Search for charginos and neutralinos in final states with two boosted hadronically decaying bosons and missing transverse momentum in pp collisions at √s=13  TeV with the ATLAS detector

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    A search for charginos and neutralinos at the Large Hadron Collider using fully hadronic final states and missing transverse momentum is reported. Pair-produced charginos or neutralinos are explored, each decaying into a high-pT Standard Model weak boson. Fully hadronic final states are studied to exploit the advantage of the large branching ratio, and the efficient rejection of backgrounds by identifying the high-pT bosons using large-radius jets and jet substructure information. An integrated luminosity of 139  fb−1 of proton-proton collision data collected by the ATLAS detector at a center-of-mass energy of 13 TeV is used. No significant excess is found beyond the Standard Model expectation. Exclusion limits at the 95% confidence level are set on wino or higgsino production with various assumptions about the decay branching ratios and the type of lightest supersymmetric particle. A wino (higgsino) mass up to 1060 (900) GeV is excluded when the lightest supersymmetry particle mass is below 400 (240) GeV and the mass splitting is larger than 400 (450) GeV. The sensitivity to high-mass winos and higgsinos is significantly extended relative to previous LHC searches using other final states.publishedVersio

    Measurement of the tt¯ tt¯ production cross section in pp collisions at √s = 13 TeV with the ATLAS detector

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    A measurement of four-top-quark production using proton-proton collision data at a centre-of-mass energy of 13 TeV collected by the ATLAS detector at the Large Hadron Collider corresponding to an integrated luminosity of 139 fb−1 is presented. Events are selected if they contain a single lepton (electron or muon) or an opposite-sign lepton pair, in association with multiple jets. The events are categorised according to the number of jets and how likely these are to contain b-hadrons. A multivariate technique is then used to discriminate between signal and background events. The measured four-top-quark production cross section is found to be 26+17−15 fb, with a corresponding observed (expected) significance of 1.9 (1.0) standard deviations over the background-only hypothesis. The result is combined with the previous measurement performed by the ATLAS Collaboration in the multilepton final state. The combined four-top-quark production cross section is measured to be 24+7−6 fb, with a corresponding observed (expected) signal significance of 4.7 (2.6) standard deviations over the background-only predictions. It is consistent within 2.0 standard deviations with the Standard Model expectation of 12.0 ± 2.4 fb.publishedVersio

    Search for new phenomena with top quark pairs in final states with one lepton, jets, and missing transverse momentum in pp collisions at √s = 13 TeV with the ATLAS detector

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    A search for new phenomena with top quark pairs in final states with one isolated electron or muon, multiple jets, and large missing transverse momentum is performed. Signal regions are designed to search for two-, three-, and four-body decays of the directly pair-produced supersymmetric partner of the top quark (stop). Additional signal regions are designed specifically to search for spin-0 mediators that are produced in association with a pair of top quarks and decay into a pair of dark-matter particles. The search is performed using the Large Hadron Collider proton-proton collision dataset at a centre-of-mass energy of √s = 13 TeV recorded by the ATLAS detector from 2015 to 2018, corresponding to an integrated luminosity of 139 fb−1. No significant excess above the Standard Model background is observed, and limits at 95% confidence level are set in the stop-neutralino mass plane and as a function of the mediator mass or the dark-matter particle mass. Stops are excluded up to 1200 GeV (710 GeV) in the two-body (three-body) decay scenario. In the four-body scenario stops up to 640 GeV are excluded for a stop-neutralino mass difference of 60 GeV. Scalar and pseudoscalar dark-matter mediators are excluded up to 200 GeV when the coupling strengths of the mediator to Standard Model and dark-matter particles are both equal to one and when the mass of the dark-matter particle is 1 GeV.publishedVersio
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