11 research outputs found

    A SOA-Based Platform-Specific Framework for Context-Aware Mobile Applications

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    Context-aware mobile applications are intelligent applications that can monitor the user’s context and, in case of changes in this context, consequently adapt their behaviour in order to satisfy the user’s current needs or anticipate the user’s intentions. The design of such applications relies on dynamic middleware platforms that consist of a variety of components. These components are distributed in the environment and interoperate by making use of each other’s services. In the A-MUSE project, we defined a design methodology based on MDA principles that relies on a SOA reference architecture for context-aware mobile applications. This paper shows how abstract concepts in the design of such applications can be applied to realize concrete components that guarantee architectural interoperability. We also present a platform-specific framework that uses BPEL, UDDI registry and web services as target technologies to implement our reference architecture

    Loss of function NFKB1 variants are the most common monogenic cause of CVID in Europeans.

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    BACKGROUND: The genetic etiology of primary immunodeficiency disease (PID) carries prognostic information. OBJECTIVE: We conducted a whole-genome sequencing study assessing a large proportion of the NIHR-BioResource - Rare Disease cohort. METHODS: In the predominantly European study population of principally sporadic unrelated PID cases (n=846), a novel Bayesian method identified NFKB1 as one most strongly associated with PID, and the association was explained by 16 novel heterozygous truncating, missense and gene deletion variants. This accounted for 4% of common variable immunodeficiency (CVID) cases (n=390) in the cohort. Amino-acid substitutions predicted to be pathogenic were assessed by analysis of structural protein data. Immunophenotyping, immunoblotting and ex vivo stimulation of lymphocytes determined the functional effects of these variants. Detailed clinical and pedigree information was collected for genotype-phenotype co-segregation analyses. RESULTS: Both sporadic and familial cases demonstrated evidence of the non-infective complications of CVID, including massive lymphadenopathy (24%), unexplained splenomegaly (48%) and autoimmune disease (48%), features prior studies correlate with worse clinical prognosis. Although partial penetrance of clinical symptoms was noted in certain pedigrees, all carriers have a deficiency in B lymphocyte differentiation. Detailed assessment of B lymphocyte numbers, phenotype and function identifies the presence of a raised CD21lowB cell population: combined with identification of the disease-causing variant, this distinguishes between healthy individuals, asymptomatic carriers and clinically affected cases. CONCLUSION: We show that heterozygous loss-of-function variants in NFKB1 are the most common known monogenic cause of CVID that results in a temporally progressive defect in the formation of immunoglobulin-producing B cells.This study was supported by The National Institute for Health Research England (grant number RG65966), and by the Center of Immunodeficiencies Amsterdam (CIDA). JET is supported by an MRC Clinician Scientist Fellowship (MR/L006197/1). AJT is supported by both the Wellcome Trust (104807/Z/14/Z) and by the National Institute for Health Research Biomedical Research Centre at Great Ormond Street Hospital for Children NHS Foundation Trust and University College London. EO receives personal fees from CSL Behring and MSD

    Large-scale context provisioning: A use-case for homogenous cloud federation

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    Improving cloud datacentre scalability, agility and performance using OpenFlow

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    Search for physics beyond the standard model in events with jets and two same-sign or at least three charged leptons in proton-proton collisions at √s=13TeV

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    A data sample of events from proton-proton collisions with at least two jets, and two isolated same-sign or three or more charged leptons, is studied in a search for signatures of new physics phenomena. The data correspond to an integrated luminosity of 137fb-1 at a center-of-mass energy of 13TeV, collected in 2016–2018 by the CMS experiment at the LHC. The search is performed using a total of 168 signal regions defined using several kinematic variables. The properties of the events are found to be consistent with the expectations from standard model processes. Exclusion limits at 95% confidence level are set on cross sections for the pair production of gluinos or squarks for various decay scenarios in the context of supersymmetric models conserving or violating R parity. The observed lower mass limits are as large as 2.1TeV for gluinos and 0.9TeV for top and bottom squarks. To facilitate reinterpretations, model-independent limits are provided in a set of simplified signal regions. © 2020, CERN for the benefit of the CMS collaboration

    Measurement of single-diffractive dijet production in proton–proton collisions at √s=8Te with the CMS and TOTEM experiments

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    Measurements are presented of the single-diffractive dijet cross section and the diffractive cross section as a function of the proton fractional momentum loss ξ and the four-momentum transfer squared t. Both processes pp→pX and pp→Xp, i.e. with the proton scattering to either side of the interaction point, are measured, where X includes at least two jets; the results of the two processes are averaged. The analyses are based on data collected simultaneously with the CMS and TOTEM detectors at the LHC in proton–proton collisions at s=8Te during a dedicated run with β∗=90m at low instantaneous luminosity and correspond to an integrated luminosity of 37.5nb-1. The single-diffractive dijet cross section σjjpX, in the kinematic region ξ< 0.1 , 0.03<|t|<1Ge2, with at least two jets with transverse momentum pT>40Ge, and pseudorapidity | η| < 4.4 , is 21.7±0.9(stat)-3.3+3.0(syst)±0.9(lumi)nb. The ratio of the single-diffractive to inclusive dijet yields, normalised per unit of ξ, is presented as a function of x, the longitudinal momentum fraction of the proton carried by the struck parton. The ratio in the kinematic region defined above, for x values in the range - 2.9 ≤ log 10x≤ - 1.6 , is R=(σjjpX/Δξ)/σjj=0.025±0.001(stat)±0.003(syst), where σjjpX and σjj are the single-diffractive and inclusive dijet cross sections, respectively. The results are compared with predictions from models of diffractive and nondiffractive interactions. Monte Carlo predictions based on the HERA diffractive parton distribution functions agree well with the data when corrected for the effect of soft rescattering between the spectator partons. © 2020, CERN for the benefit of the CMS and TOTEM collaborations

    Measurements with silicon photomultipliers of dose-rate effects in the radiation damage of plastic scintillator tiles in the CMS hadron endcap calorimeter

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    Measurements are presented of the reduction of signal output due to radiation damage for two types of plastic scintillator tiles used in the hadron endcap (HE) calorimeter of the CMS detector. The tiles were exposed to particles produced in proton-proton (pp) collisions at the CERN LHC with a center-of-mass energy of 13 TeV, corresponding to a delivered luminosity of 50 fb-1. The measurements are based on readout channels of the HE that were instrumented with silicon photomultipliers, and are derived using data from several sources: A laser calibration system, a movable radioactive source, as well as hadrons and muons produced in pp collisions. Results from several irradiation campaigns using 60Co sources are also discussed. The damage is presented as a function of dose rate. Within the range of these measurements, for a fixed dose the damage increases with decreasing dose rate
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