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Aujourd’hui, l’énergie nucléaire représente une partie non-négligeable du marché énergétique mondial, très probablement vouée à croître dans les prochaines décennies. Les réacteurs du futur devront notamment répondre à des critères supplémentaires économiques mais surtout de sûreté, de non-prolifération, de gestion optimisée du combustible et d’une gestion responsable des déchets nucléaires. Dans le cadre de cette thèse, des études concernant la non-prolifération des armes nucléaires sont abordées, dans le cadre de la recherche et développement d’un nouvel outil potentiel de surveillance des réacteurs nucléaires ; la détection des antineutrinos des réacteurs. En effet, les propriétés de ces particules pourraient intéresser l’Agence Internationale de l’Energie Atomique (AIEA) en charge de l’application du Traité de non-prolifération des armes nucléaires. L’AIEA encourage ainsi ses états membres à mener une étude de faisabilité. Une première étude de non-prolifération est réalisée avec la simulation d’un scénario proliférant utilisant un réacteur de type CANDU et de l’émission en antineutrinos associée. Nous en déduisons une prédiction de la sensibilité d’un détecteur d’antineutrinos de taille modeste à la diversion d’une quantité significative de plutonium. Une seconde étude est réalisée dans le cadre du projet Nucifer, détecteur d’antineutrinos placé auprès du réacteur de recherche OSIRIS. Nucifer est un détecteur d’antineutrinos dédié à la non-prolifération à l’efficacité optimisée conçu pour être un démonstrateur pour l’AIEA. La simulation du réacteur OSIRIS est développée ici pour le calcul de l’émission d’antineutrinos qui sera comparée aux données mesurées par le détecteur ainsi que pour caractériser le bruit de fond important émis par le réacteur détecté dans Nucifer. De façon générale, les antineutrinos des réacteurs sont émis lors des décroissances radioactives des produits de fission. Ces décroissances radioactives sont également à l’origine de la puissance résiduelle émise après l’arrêt d’un réacteur nucléaire, dont l’estimation est un enjeu de sûreté. Nous présenterons dans cette thèse un travail expérimental dont le but est de mesurer les propriétés de décroissance bêta de produits de fission importants pour la non-prolifération et la puissance résiduelle des réacteurs. Des premières mesures utilisant la technique de Spectroscopie par Absorption Totale (TAGS) ont été réalisées auprès du dispositif de l’Université de Jyväskylä. Nous présenterons la technique employée, le dispositif expérimental ainsi qu’une partie de l’analyse de cette expérience. / Today, nuclear energy represents a non-negligible part of the global energy market, most likely a rolling wheel to grow in the coming decades. Reactors of the future must face the criteria including additional economic but also safety, non-proliferation, optimized fuel management and responsible management of nuclear waste. In the framework of this thesis, studies on non-proliferation of nuclear weapons are discussed in the context of research and development of a new potential tool for monitoring nuclear reactors, the detection of reactor antineutrinos, because the properties of these particles may be of interest for the International Agency of Atomic Energy (IAEA), in charge of the verification of the compliance by States with their safeguards obligations as well as on matters relating to international peace and security. The IAEA encouraged its member states to carry on a feasibility study. A first study of non-proliferation is performed with a simulation, using a proliferating scenario with a CANDU reactor and the associated antineutrinos emission. We derive a prediction of the sensitivity of an antineutrino detector of modest size for the purpose of the diversion of a significant amount of plutonium. A second study was realized as part of the Nucifer project, an antineutrino detector placed nearby the OSIRIS research reactor. The Nucifer antineutrino detector is dedicated to non-proliferation with an optimized efficiency, designed to be a demonstrator for the IAEA. The simulation of the OSIRIS reactor is developed here for calculating the emission of antineutrinos which will be compared with the data measured by the detector and also for characterizing the level of background noises emitted by the reactor detected in Nucifer. In general, the reactor antineutrinos are emitted during radioactive decay of fission products. These radioactive decays are also the cause of the decay heat emitted after the shutdown of a nuclear reactor of which the estimation is an issue of nuclear safety. In this thesis, we present an experimental work which aims to measure the properties of beta decay of fission products important to the non-proliferation and reactor decay heat. First steps using the technique of Total Absorption Gamma-ray Spectroscopy (TAGS) were carried on at the radioactive beam facility of the University of Jyvaskyla. We will present the technique used, the experimental setup and part of the analysis of this experiment.

The role of scalar mesons in radiative vector meson decays is investigated. The effects of scalar-isoscalar f_{0}(980) and scalar-isovector a_{0}(980) mesons are studied in the mechanism of the radiative Phi-&gt / pi{+}pi{-}gamma and phi-&gt / pi{0}eta gamma decays, respectively. A phenomenological approach is used to study the radiative phi-&gt / pi{+}p{-}gamma decay by considering the contributions of sigma-meson, rho-meson and f_{0}-meson. The interference effects between different contributions are analyzed and the branching ratio for this decay is calculated. The radiative phi-&gt / pi{0}eta gamma decay is studied within the framework of a phenomenological approach in which the contributions of rho-meson, chiral loop and a_{0}-meson are considered. The interference effects between different contributions are examined and the coupling constants g_{phi a_{0} gamma} and g_{a_{0}K{+}K{-}} are estimated using the experimental branching ratio for the phi-&gt / pi{0}eta gamma decay. Furthermore, the radiative rho{0}pi{+}pi{-}gamma$ and rho{0}-&gt / pi{0}pi{0}gamma decays are studied to investigate the role of scalar-isoscalar sigma-meson. The branching ratios of the rho{0}-&gt / pi{+}pi{-}gamma and rho{0}-&gt / pi{0}pi{0}gamma decays are calculated using a phenomenological approach by adding to the amplitude calculated within the framework of chiral perturbation theory and vector meson dominance the amplitude of sigma-meson intermediate state. In all the decays studied the scalar meson intermediate states make important contributions to the overall amplitude.

Hintergrund: Der Insulin-like growth factor receptor (IGF1R) spielt eine zentrale Rolle bei Wachstumsprozessen. Heterozygote IGF1R-Mutationen führen durch eine partielle IGF1-Resistenz zu Kleinwuchs. Methoden: Auxologische und endokrinologische Daten des Patienten wurden erhoben. Anhand von Fibroblasten wurde die IGF1R-Deletion charakterisiert und die Auswirkungen auf die mRNA- und Protein-Expression sowie die Signaltransduktion untersucht. Ergebnisse: Der Junge, der eine heterozygote Exon 6 Deletion im IGF1R – durch Alu-Rekombination verursacht – und eine heterozygote SHOX-Variante (p.Met240Ile) in seinem Genom vereint, kam ‚appropriate for gestational age‘ zur Welt, entwickelte aber postnatal eine Wachstumsretardierung. Die Endokrinologischen Daten waren unauffällig. Der Patient zeigt keine Stigmata, die bei anderen IGF1- oder SHOX-Mutationsträgern beschrieben wurden. Durch Nonsense-Mediated mRNA Decay kommt es zu einer Dosisreduktion der IGF1-Rezeptoren und einer entsprechenden verminderten Aktivierung der Rezeptoren, nicht aber des Signalwegs. Zusammenfassung: Der Patient trägt eine bisher unbeschrieben heterozygote IGF1R-Deletion, die zu Kleinwuchs führt. Ursächlich dafür ist eine durch die Mutation verursachte Dosisreduktion der IGF1-Rezeptoren.

A general analysis of the photon and lepton polarizations in the rare Bs &amp / #8594 / &amp / #61543 / l+l- decay by using the most general model independent form of the effective Hamiltonian is presented. The total and the differential branching ratios for these decays, when photon is in the positive and negative helicity states, are studied. The sensitivity of &quot / photon polarization asymmetry&quot / and the longitudinal, transverse and normal polarization asymmetries of final state leptons, as well as lepton-antilepton combined asymmetries in Bs &amp / #8594 / &amp / #61543 / l+l- decay to the new Wilson coefficients are also investigated. It is shown that all these physical observables are very sensitive to the existence of new physics beyond SM and their experimental measurements can give valuable information about it.

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Made available in DSpace on 2014-10-09T12:41:56Z (GMT). No. of bitstreams: 0 / Made available in DSpace on 2014-10-09T14:04:33Z (GMT). No. of bitstreams: 0 / O Laboratório de Metrologia Nuclear (LMN) do Instituto de Pesquisas Energéticas e Nucleares (IPEN) desenvolveu recentemente o Sistema de Coincidência por Software (SCS), para a digitalização e registro dos sinais de seus sistemas de coincidências 4πβ-γ utilizados na padronização de radionuclídeos. O sistema SCS possui quatro entradas analógicas independentes que possibilitam o registro simultâneo dos sinais de até quatro detectores (vias β e γ). A análise dos dados é realizada a posteriori, por software, incluindo discriminação de amplitudes, simulação do tempo-morto da medida e definição do tempo de resolução de coincidências. O software então instalado junto ao SCS estabeleceu a metodologia básica de análise, aplicável a radionuclídeos com decaimento simples, como o 60Co. O presente trabalho amplia a metodologia de análise de dados obtidos com o SCS, de modo a possibilitar o uso de detectores com alta resolução em energia (HPGe), para padronização de radionuclídeos com decaimentos mais complexos, com diferentes ramos de decaimento ou com transições metaestáveis. A expansão metodológica tem suporte na elaboração do programa de análise denominado Coincidence Analyzing Task (CAT). A seção de aplicação inclui as padronizações do 152Eu (diferentes ramos de decaimento) e do 67Ga (nível metaestável). A padronização do 152Eu utilizou uma amostra de uma comparação internacional promovida pelo BIPM (Bureau International des Poids et Mesures), podendo-se comparar a atividade obtida com o valores de laboratórios mundialmente reconhecidos, de modo a avaliar e validar a metodologia desenvolvida. Para o 67Ga, foram obtidas: a meia-vida do nível metaestável de 93 keV, por três diferentes técnicas de análise do conjunto de dados (βpronto-γatrasado-HPGe, βpronto-γatrasado-NaI e βpronto- βatrasado); as atividades de cinco amostras, normalizadas por Monte Carlo e as probabilidades de emissão gama por decaimento, para nove transições. / Tese (Doutoramento) / IPEN/T / Instituto de Pesquisas Energeticas e Nucleares - IPEN-CNEN/SP

Made available in DSpace on 2014-10-09T12:51:57Z (GMT). No. of bitstreams: 0 / Made available in DSpace on 2014-10-09T14:07:25Z (GMT). No. of bitstreams: 0 / Neste trabalho foram estudados os níveis excitados do 193Ir produzidos a partir do decaimento β do 193Os (T1/2 ~ 30h). Para a obtenção das amostras radioativas, ~ 5mg de Ósmio metálico (99% enriquecido em 192Os) foram irradiados no reator IEA-R1, sob um fluxo de nêutrons da ordem de 1012cm2 s1 e depois analisadas, empregando-se a técnica de espectroscopia γ simples (unidimensional) com espectrômetro γ de alta resolução, bem como as técnicas de espectroscopia de coincidência γγ e de correlação angular direcional γγ (θ) usando um sistema de aquisição multiparamétrica composto por 4 detectores HPGe. A partir destes dados, foi possível adicionar 28 transições a este esquema de decaimento, 11 das quais já eram conhecidas de outros estudos envolvendo reações nucleares, além de 17 observadas pela primeira vez. Também foram acrescentados 8 níveis excitados ao esquema de decaimento, sendo 3 conhecidos por outras reações e 5 completamente novos. Além disso, foi possível confirmar a suspeita encontrada na literatura de que os níveis a 848,93keV e 849,093keV são o mesmo, além de confirmar a existência de um nível excitado a 806,9keV. A análise de correlação angular direcional permitiu a definição do spin de do nível excitado a 874kev (5/2+), além de propor uma probabilidade de 79% do nível a 1078keV ter spin 5/2, e de restringir as possibilidades de spin para o nível novo a 960keV para duas (1/2 ou 3/2). Também foi possível determinar a razão de mistura multipolar (δLn+1/Ln) para 43 transições, sendo 19 pela primeira vez e praticamente todas as restantes com precisão melhor que a encontrada na literatura. Finalmente, foi feita uma tentativa de compreender o núcleo através de um modelo teórico, o qual reproduziu muito bem o estado fundamental e os dois primeiros estados excitados do 193Ir. / Tese (Doutoramento) / IPEN/T / Instituto de Pesquisas Energeticas e Nucleares - IPEN-CNEN/SP