821 research outputs found

    Analysis of Documental Heritage of CETA in Standardization of the Spanish Manufacturing Automotive Industry

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    AbstractStandardization has taken an active role in the development of several main industrial sectors and the automotive sector has been one of them. Current work shows the collection, analysis, revision and management process developed on CETA (Center of Automotive Technical Study) standards related to the manufacturing automotive sector, an unknown element of Spanish standardization heritage. Also, a comparative evaluation between CETA standards and current UNE standards has been performed. The results show that CETA standards have had a positive influence in the evolution of the automotive sector as well as an important contribution to the contents of the UNE standards

    On Standard Reductions to Relative Gravity Measurements. A Case Study Through the Establishment of the New Local Gravity Net in the Province of Valencia (Spain)

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    This is an author's accepted manuscript of an article published in: “Survey Review"; Volume 43, Issue 319, 2011; copyright Taylor & Francis; available online at: http://dx.doi.org/10.1179/003962610X12747001420825Standard reductions to gravity readings due to Earth tides, ocean loading and attraction, polar motion, instrumental height and air pressure variations and loading of atmospheric masses are studied in this paper from a practical point of view, that is, taking into account their numerical values and their influence on gravimetric readings and relative gravimetric observations. The study was carried out using the observations and definition of a new local gravimetric net. This new local gravimetric net has been established in the province of Valencia (Eastern Spain) to meet the increasing requirements of geophysics, geology, geodesy and geodynamics. The net comprises 21 sites, which are an average of 45 km apart and was measured using Lacoste & Romberg D203 and G301 gravimeters. Gravity values were determined using one fixed station in relation to an absolute one and 202 relative gravimetric observables. Reductions are applied for Earth tides (with real accurate amplitude and phase-difference for the principal tidal waves analysed from 301 digitally recorded days of gravity readings) where oceanic attraction and loading has been considered. In addition, reductions for polar motion, vertical gradient to instrument height and air pressure and loading of atmospheric masses have been applied. The net was established using least square adjustment where the weights of each relative gravimetric observable were determined by iterative estimation in accordance with the Huber robust estimation procedure. Obtained standard deviations of the final gravity values have an average value of 18x10-8 ms-2 (18 µGal), minimum value of 10x10-8 ms-2 and maximum value of 26x10-8 ms-2 . The statistical analysis of the results concludes with a precision and reliability determination. Discussion of the numerical values obtained in the standard gravimetric reductions shows the importance of each one in the final solution, bearing in mind that the relative gravimetric observables have been obtained using Lacoste & Romberg instruments and the geographical location of the net. The main conclusion is that only Earth tides reduction (with approximate amplitude and phase-difference numbers for the principal tidal waves) have to be taken into accountMartín Furones, ÁE.; Anquela Julián, AB.; Padin Devesa, J.; Berné Valero, JL. (2011). On Standard Reductions to Relative Gravity Measurements. A Case Study Through the Establishment of the New Local Gravity Net in the Province of Valencia (Spain). Survey Review. 43(319):16-29. doi:10.1179/003962610X12747001420825S162943319Boedecker, G., & Richter, B. (1981). The new gravity base net 1976 of the Federal Republic of Germany (DSGN 76). Bulletin Géodésique, 55(3), 250-266. doi:10.1007/bf02530865Cartwright, D. E., & Tayler, R. J. (2007). New Computations of the Tide-generating Potential. Geophysical Journal of the Royal Astronomical Society, 23(1), 45-73. doi:10.1111/j.1365-246x.1971.tb01803.xCharles, K. and Hipkin, R.G. 1994. British precise gravity net 1993. Joint symposium of the International Gravity Comission and the International Geoid Comission, Symposium 113: 39–45, Graz, Austria. Ed. Springer-Verlag.Farrell, W. E. (1972). Deformation of the Earth by surface loads. Reviews of Geophysics, 10(3), 761. doi:10.1029/rg010i003p00761Jentzsch, G. (s. f.). Earth tides and ocean tidal loading. Lecture Notes in Earth Sciences, 145-171. doi:10.1007/bfb0011461Torge, W. 1989. Gravimetry. Ed. Walter de Gruyter, Berlin-New York. 465 pages.Wahr, J. M. (1985). Deformation induced by polar motion. Journal of Geophysical Research, 90(B11), 9363. doi:10.1029/jb090ib11p09363Wenzel, G. 1998. Format and structure for the exchange of high precision tidal data, http://www.ife.uni-hannover.de/∼Wenzel/format/format.html, acceded on February 1999

    The EGR2 gene is involved in axonal Charcot-Marie-Tooth disease

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    [EN] Background and purpose: A three-generation family affected by axonal Charcot-Marie-Tooth disease (CMT) was investigated with the aim of discovering genetic defects and to further characterize the phenotype. Methods: The clinical, nerve conduction studies and muscle magnetic resonance images of the patients were reviewed. A whole exome sequencing was performed and the changes were investigated by genetic studies, in silico analysis and luciferase reporter assays. Results: A novel c.1226G>A change (p.R409Q) in the EGR2 gene was identified. Patients presented with a typical, late-onset axonal CMT phenotype with variable severity that was confirmed in the ancillary tests. The in silico studies showed that the residue R409 is an evolutionary conserved amino acid. The p.R409Q mutation, which is predicted as probably damaging, would alter the conformation of the protein slightly and would cause a decrease of gene expression. Conclusions: This is the first report of an EGR2 mutation presenting as an axonal CMT phenotype with variable severity. This study broadens the phenotype of the EGR2-related neuropathies and suggests that the genetic testing of patients suffering from axonal CMT should include the EGR2 gene.This collaborative joint project is awarded by IRDiRC and funded by the Instituto de Salud Carlos III (ISCIII) - Subdireccion General de Evaluacion y Fomento de la Investigacion within the framework of the National R+D+I Plan (Grants IR11/TREAT-CMT, PI12/00946 and PI12/00453), co-funded with FEDER funds. C.E. has a "Miguel Servet' contract funded by the ISCIII and Centro de Investigacion Principe Felipe (CIPF) (Grant no. CPII14/00002). We are also grateful to Itziar Llopis for sample management.Sevilla, T.; Sivera, R.; Martínez-Rubio, D.; Lupo, V.; Chumillas, M.; Calpena-Corpas, E.; Dopazo, J.... (2015). The EGR2 gene is involved in axonal Charcot-Marie-Tooth disease. European Journal of Neurology. 22(12):1548-1555. https://doi.org/10.1111/ene.1278215481555221

    Global methylome scores correlate with histological subtypes of colorectal carcinoma and show different associations with common clinical and molecular features

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    Background. The typical methylation patterns associated with cancer are hypermethylation at gene promoters and global genome hypomethylation. Aberrant CpG island hypermethylation at promoter regions and global genome hypomethylation have not been associated with histological colorectal carcinomas (CRC) subsets. Using Illumina’s 450 k Infinium Human Methylation beadchip, the methylome of 82 CRCs were analyzed, comprising different histological subtypes: 40 serrated adenocarcinomas (SAC), 32 conventional carcinomas (CC) and 10 CRCs showing histological and molecular features of microsatellite instability (hmMSI‐H), and, additionally, 35 normal adjacent mucosae. Scores reflecting the overall methylation at 250 bp, 1 kb and 2 kb from the transcription starting site (TSS) were studied. Results. SAC has an intermediate methylation pattern between CC and hmMSI‐H for the three genome locations. In addition, the shift from promoter hypermethylation to genomic hypomethylation occurs at a small sequence between 250 bp and 1 Kb from the gene TSS, and an asymmetric distribution of methylation was observed between both sides of the CpG islands (N vs. S shores). Conclusion. These findings show that different histological subtypes of CRC have a particular global methylation pattern depending on sequence distance to TSS and highlight the so far underestimated importance of CpGs aberrantly hypomethylated in the clinical phenotype of CRCs.This work was supported by two grants from Instituto de Salud Carlos III, Ministerio de Sanidad, Spain and FEDER funds (refs: PI12-1232, PI18-0144) and another from the European Union’s Horizon 2020 research and innovation program (ref. 848098

    Epilepsy and neuropsychiatric comorbidities in mice carrying a recurrent Dravet syndrome SCN1A missense mutation

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    Dravet Syndrome (DS) is an encephalopathy with epilepsy associated with multiple neuropsychiatric comorbidities. In up to 90% of cases, it is caused by functional happloinsufficiency of the SCN1A gene, which encodes the alpha subunit of a voltage-dependent sodium channel (Nav1.1). Preclinical development of new targeted therapies requires accessible animal models which recapitulate the disease at the genetic and clinical levels. Here we describe that a C57BL/6 J knock-in mouse strain carrying a heterozygous, clinically relevant SCN1A mutation (A1783V) presents a full spectrum of DS manifestations. This includes 70% mortality rate during the first 8 weeks of age, reduced threshold for heat-induced seizures (4.7 °C lower compared with control littermates), cognitive impairment, motor disturbances, anxiety, hyperactive behavior and defects in the interaction with the environment. In contrast, sociability was relatively preserved. Electrophysiological studies showed spontaneous interictal epileptiform discharges, which increased in a temperature-dependent manner. Seizures were multifocal, with different origins within and across individuals. They showed intra/inter-hemispheric propagation and often resulted in generalized tonic-clonic seizures. 18F-labelled flourodeoxyglucose positron emission tomography (FDG-PET) revealed a global increase in glucose uptake in the brain of Scn1aWT/A1783V mice. We conclude that the Scn1aWT/A1783V model is a robust research platform for the evaluation of new therapies against DS

    Time-integrated luminosity recorded by the BABAR detector at the PEP-II e+e- collider

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    This article is the Preprint version of the final published artcile which can be accessed at the link below.We describe a measurement of the time-integrated luminosity of the data collected by the BABAR experiment at the PEP-II asymmetric-energy e+e- collider at the ϒ(4S), ϒ(3S), and ϒ(2S) resonances and in a continuum region below each resonance. We measure the time-integrated luminosity by counting e+e-→e+e- and (for the ϒ(4S) only) e+e-→μ+μ- candidate events, allowing additional photons in the final state. We use data-corrected simulation to determine the cross-sections and reconstruction efficiencies for these processes, as well as the major backgrounds. Due to the large cross-sections of e+e-→e+e- and e+e-→μ+μ-, the statistical uncertainties of the measurement are substantially smaller than the systematic uncertainties. The dominant systematic uncertainties are due to observed differences between data and simulation, as well as uncertainties on the cross-sections. For data collected on the ϒ(3S) and ϒ(2S) resonances, an additional uncertainty arises due to ϒ→e+e-X background. For data collected off the ϒ resonances, we estimate an additional uncertainty due to time dependent efficiency variations, which can affect the short off-resonance runs. The relative uncertainties on the luminosities of the on-resonance (off-resonance) samples are 0.43% (0.43%) for the ϒ(4S), 0.58% (0.72%) for the ϒ(3S), and 0.68% (0.88%) for the ϒ(2S).This work is supported by the US Department of Energy and National Science Foundation, the Natural Sciences and Engineering Research Council (Canada), the Commissariat à l’Energie Atomique and Institut National de Physique Nucléaire et de Physiquedes Particules (France), the Bundesministerium für Bildung und Forschung and Deutsche Forschungsgemeinschaft (Germany), the Istituto Nazionale di Fisica Nucleare (Italy), the Foundation for Fundamental Research on Matter (The Netherlands), the Research Council of Norway, the Ministry of Education and Science of the Russian Federation, Ministerio de Ciencia e Innovación (Spain), and the Science and Technology Facilities Council (United Kingdom). Individuals have received support from the Marie-Curie IEF program (European Union) and the A.P. Sloan Foundation (USA)

    Search for direct production of charginos and neutralinos in events with three leptons and missing transverse momentum in √s = 7 TeV pp collisions with the ATLAS detector

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    A search for the direct production of charginos and neutralinos in final states with three electrons or muons and missing transverse momentum is presented. The analysis is based on 4.7 fb−1 of proton–proton collision data delivered by the Large Hadron Collider and recorded with the ATLAS detector. Observations are consistent with Standard Model expectations in three signal regions that are either depleted or enriched in Z-boson decays. Upper limits at 95% confidence level are set in R-parity conserving phenomenological minimal supersymmetric models and in simplified models, significantly extending previous results

    Jet size dependence of single jet suppression in lead-lead collisions at sqrt(s(NN)) = 2.76 TeV with the ATLAS detector at the LHC

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    Measurements of inclusive jet suppression in heavy ion collisions at the LHC provide direct sensitivity to the physics of jet quenching. In a sample of lead-lead collisions at sqrt(s) = 2.76 TeV corresponding to an integrated luminosity of approximately 7 inverse microbarns, ATLAS has measured jets with a calorimeter over the pseudorapidity interval |eta| < 2.1 and over the transverse momentum range 38 < pT < 210 GeV. Jets were reconstructed using the anti-kt algorithm with values for the distance parameter that determines the nominal jet radius of R = 0.2, 0.3, 0.4 and 0.5. The centrality dependence of the jet yield is characterized by the jet "central-to-peripheral ratio," Rcp. Jet production is found to be suppressed by approximately a factor of two in the 10% most central collisions relative to peripheral collisions. Rcp varies smoothly with centrality as characterized by the number of participating nucleons. The observed suppression is only weakly dependent on jet radius and transverse momentum. These results provide the first direct measurement of inclusive jet suppression in heavy ion collisions and complement previous measurements of dijet transverse energy imbalance at the LHC.Comment: 15 pages plus author list (30 pages total), 8 figures, 2 tables, submitted to Physics Letters B. All figures including auxiliary figures are available at http://atlas.web.cern.ch/Atlas/GROUPS/PHYSICS/PAPERS/HION-2011-02

    Measurement of the polarisation of W bosons produced with large transverse momentum in pp collisions at sqrt(s) = 7 TeV with the ATLAS experiment

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    This paper describes an analysis of the angular distribution of W->enu and W->munu decays, using data from pp collisions at sqrt(s) = 7 TeV recorded with the ATLAS detector at the LHC in 2010, corresponding to an integrated luminosity of about 35 pb^-1. Using the decay lepton transverse momentum and the missing transverse energy, the W decay angular distribution projected onto the transverse plane is obtained and analysed in terms of helicity fractions f0, fL and fR over two ranges of W transverse momentum (ptw): 35 < ptw < 50 GeV and ptw > 50 GeV. Good agreement is found with theoretical predictions. For ptw > 50 GeV, the values of f0 and fL-fR, averaged over charge and lepton flavour, are measured to be : f0 = 0.127 +/- 0.030 +/- 0.108 and fL-fR = 0.252 +/- 0.017 +/- 0.030, where the first uncertainties are statistical, and the second include all systematic effects.Comment: 19 pages plus author list (34 pages total), 9 figures, 11 tables, revised author list, matches European Journal of Physics C versio

    Observation of a new chi_b state in radiative transitions to Upsilon(1S) and Upsilon(2S) at ATLAS

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    The chi_b(nP) quarkonium states are produced in proton-proton collisions at the Large Hadron Collider (LHC) at sqrt(s) = 7 TeV and recorded by the ATLAS detector. Using a data sample corresponding to an integrated luminosity of 4.4 fb^-1, these states are reconstructed through their radiative decays to Upsilon(1S,2S) with Upsilon->mu+mu-. In addition to the mass peaks corresponding to the decay modes chi_b(1P,2P)->Upsilon(1S)gamma, a new structure centered at a mass of 10.530+/-0.005 (stat.)+/-0.009 (syst.) GeV is also observed, in both the Upsilon(1S)gamma and Upsilon(2S)gamma decay modes. This is interpreted as the chi_b(3P) system.Comment: 5 pages plus author list (18 pages total), 2 figures, 1 table, corrected author list, matches final version in Physical Review Letter
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