Le développement récent de la théorie quantique de l’information porte la communauté scientifique à s’intéresser de plus en plus aux sources de photons uniques. En effet, ces sources peuvent par exemple être utilisées pour le calcul quantique optique ou la cryptographie quantique pour améliorer les performances de distribution des clés et éviter les écoutes ou tentatives de hacking. Par conséquent, il est nécessaire de disposer de sources fiables et pour des applications réalistes, le défi est d'obtenir des sources de photons uniques qui fonctionnent jusqu'à température ambiante.Notre groupe à récemment démontré qu'en insérant une boîte quantique de CdSe dans un nanofil de ZnSe, l'émission de photons uniques pouvait être obtenue jusqu'à température ambiante. Néanmoins, ces nanofils avaient un rendement quantique faible et n'étaient pas orientés verticalement à la surface des échantillons du fait de leur croissance suivant l'orientation cristallographique (001). Ces nanofils verticaux ont pour intérêt de pouvoir être aisément couplés à des structures photoniques pour augmenter la collection des photons et leur croissance est favorisée avec des substrats orientés suivant l'orientation cristallographique (111).Dans ce contexte, le but de ce travail de doctorat est de développer la croissance de boîtes quantiques de CdSe insérées dans des nanofils de ZnSe verticaux suivant l'orientation cristallographique (111) par épitaxie par jet moléculaire, d'en étudier les propriétés optiques jusqu'à température ambiante pour des applications potentielles en tant que sources de photons uniques, et de coupler ces nano-objets à des structures photoniques pour augmenter la collection de photons. Pour atteindre ces objectifs, nous avons divisé notre étude en trois points.La première étape de ce travail est concentrée sur le développement de la croissance de nanofils de ZnSe verticalement orientés et passivés par une coquille semiconductrice de ZnMgSe. Nous observons que grâce à cette coquille, l’émission lumineuse de nanofils uniques augmente de plus de deux ordres de grandeur. Dans un second temps, nous démontrons la possibilité d’insérer des boîtes de CdSe dans ces nanofils de ZnSe suivant différentes conditions de croissance. L’influence de ces conditions de croissance est mise en évidence par des études structurales et de composition de ces nano-objets. Des études optiques en fonction de la température montrent que ces nanofils émettent jusqu'à température ambiante. De plus, l'étude du temps de déclin de nanofils uniques révèle que ces fils sont robustes et insensibles aux canaux de recombinaison non-radiatifs jusqu'à 200 K. La troisième étape de ce travail concerne l'augmentation de la collection des photons de ces nano-objets. Nous montrons dans un premier temps qu’en changeant l’environnement diélectrique d’une boîte quantique, son taux d’émission spontanée peut être augmenté. Puis nous montrons la possibilité de créer des fils photoniques à partir des boîtes quantiques insérées dans des nanofils, en recouvrant ces fils d'une épaisse coquille diélectrique. A la lumière d'expériences de microphotoluminescence - qui montrent que ces fils photoniques augmentent efficacement la collection de photons - et de simulations, nous discutons l'intérêt de l'orientation du dipôle (parallèle ou perpendiculaire à l'axe de croissance du nanofil) dans ces structures. / The recent development of the quantum information theory focuses the interest of the scientific community on single-photon sources. Indeed, these sources can be used for instance for optical quantum computing or quantum cryptography to improve the quantum key distribution performances and avoid eavesdropping. Consequently, it is necessary to have reliable single-photon sources and for realistic applications, the challenge is to get a single-photon source operating up to room temperature.Our group recently demonstrated that by inserting a quantum dot of CdSe in a nanowire of ZnSe, single-photon emission could be obtained up to room temperature. Still, these nanowires had a low quantum yield and were not vertically oriented on the as-grown sample since they were grown along the (001) crystallographic orientation. The interest of vertically oriented nanowires is that they can be coupled to photonic structures to increase their photons collection and their growth is favored on (111)-oriented substrates.In this context, the aim of this PhD work is to develop the growth of vertically oriented ZnSe-CdSe nanowire quantum dots along the (111) crystallographic orientation by molecular beam epitaxy, to study their luminescence up to room temperature for single-photon sources applications, and to couple these nano-objects to photonic structures to increase the photons collection. To reach this goal, we divided this project in three steps.The first step focuses on the development of vertically oriented ZnSe nanowires, passivated with a semiconductor shell of ZnMgSe to enhance their luminescence. In a second step, we demonstrate the possibility to insert CdSe quantum dots in these ZnSe nanowires, using different growth conditions for the quantum dot. The influence of these growth conditions is studied with structural and composition analysis of these nano-objects. Optical studies as a function of the temperature show that these nanowires emit up to room temperature. Moreover, decay-time studies on single nanowire quantum dots reveal that these nanowires are robust and insensitive to non-radiative recombination channels up to 200 K. The third step of this work concerns the enhancement of the light collection from these nano-objects. First, we show that by changing the dielectric environment of the quantum dot, its decay-rate can be increased. Then, we show the possibility to create photonic wires by covering these nanowire quantum dots with a thick dielectric shell. In the light of microphotoluminescence experiments – which show that these photonic wires efficiently increase the photons collection – and simulations, we discuss the interest of the dipole orientation (parallel or perpendicular to the nanowire growth axis) in these structures.

A mucopolissacaridose tipo VI (MPS VI) ou Síndrome de Maroteaux-Lamy, é uma doença autossômica recessiva causada pela deficiência da enzima lisossomal Nacetilgalactosamina- 4-sulfatase (ARSB), a qual resulta no armazenamento lisossômico de dermatan sufato em vários tecidos e órgãos, dando origem a uma condição clínica de espectro variável, desde formas mais graves até formas mais atenuadas. O acúmulo de substrato não degradado causa um importante comprometimento ósseo, problemas respiratórios, baixa estatura e outros problemas, afetando os olhos, o coração e outros órgãos. Embora a síndrome de Maroteaux-Lamy não tenha uma incidência definida no Brasil, é reconhecido que, no nosso meio, é muito mais freqüente do que em outros países e regiões. Ela é particularmente freqüente no município de Monte Santo (Bahia), de aproximadamente 50.000 habitantes e onde já foram registrados 13 casos da doença. O diagnóstico é importante porque existe hoje um tratamento específico para a doença, a terapia de reposição enzimática (TRE), que vem mostrando bons resultados, especialmente quando iniciada em idade precoce. Descrevemos neste trabalho uma adaptação para microplacas da medida fluorimétrica da atividade de ARSB, e uma nova metodologia de análise molecular, ambas padronizados para sangue total impregnado em papel-filtro (STIPF). Essas técnicas foram desenvolvidas para incluir um teste de triagem neonatal para MPS VI, realizado nas amostras coletadas para o “teste do pezinho” nos neonatos do município de Monte Santo. Esses métodos permitem a detecção de pacientes com MPS VI e dos portadores da mutação específica que parece ser responsável pela alta incidência de MPS VI nessa localidade, uma vez que todos os pacientes lá diagnosticados apresentavam a mesma mutação (p.H178L) em homozigose. O trabalho foi desenvolvido em três etapas: na primeira foi realizada a padronização das técnicas em 100 amostras de STIPF; na segunda foi feito um teste-piloto com amostras de neonatos de Monte Santo, para avaliação das técnicas padronizadas e para o estudo de termoestabilidade em controles hígidos; na terceira foram analisadas amostras de STIPF de neonatos provenientes de Monte Santo pelos dois métodos (bioquímico e molecular). A padronização para realização da medida fluorimétrica da atividade enzimática de ARSB em microplacas indicou que o método é sensível, permitiu diferenciar os valores da população normal dos valores dos pacientes afetados e possibilitou a identificação segura de pacientes com MPS VI. Nas padronizações da análise molecular da mutação p.H178L em STIPF foi possível diferenciar os indivíduos normais, heterozigotos e homozigotos. Os resultados preliminares disponíveis indicam que o protocolo de triagem neonatal para MPS VI desenvolvido no presente trabalho poderá ser facilmente incorporado por laboratórios de referência, contribuindo para a detecção e tratamento precoce dos pacientes afetados por MPS VI. / Mucopolysaccharidosis type VI (MPS VI) or Maroteux-Lamy syndrome, is an autosomal recessive disorder caused by deficiency of the lysosomal enzyme Nacetylgalactosamine- 4-sulfatase (ARSB), which results in lysosomal storage of dermatan sufate in various tissues and organs and leads to a variable clinical spectrum, including more severe and attenuated forms. The accumulation of undegraded substrate causes bone involvement, respiratory problems and short stature, among other signs and symptoms, affecting the eyes, heart and other organs. Although the Maroteaux-Lamy syndrome does not have a defined incidence in Brazil, it is recognized that in our environment it is much more frequent than in other countries and regions. It is particularly frequent in the municipality of Monte Santo (Bahia) approximately 50,000 inhabitants and where there have already been 13 cases of the disease. The diagnosis is important because today there is a specific treatment for the disease, enzyme replacement therapy (ERT) which has shown good results, especially when started at an early age. We describe herein the standardization of the microplate fluorometric method for the ARSB test and a new methodology of molecular analysis, both adapted for dried blood spots (DBS) samples. These techniques were developed for inclusion of MPS VI in the newborn screening program that already tests the neonates of the city of Monte Santo, Bahia, Brasil for metabolic diseases. The methods were developed to detect patients with MPS VI and also for carriers, once the disease seems to have a high incidence (around 1:5.000) at this location. Also, all patients that have already been diagnosed in this city presented the same mutation (p.H178L) in homozygosis. The study was conducted in three stages: in the first was performed in 100 DBS samples an standardization of the techniques; in the second was done a pilot test with samples of newborns of Monte Santo, for the evaluation of standardized techniques and for the thermostability study in healthy controls; in the third were analyzed newborns samples from Monte Santo for both biochemical and molecular methods. Standardization on microplate for fluorimetric enzyme activity of the ARSB showed the assay sensitivity, differentiating values between normal and affected and allowing a reliable detection of patients with MPS VI. On the standardization for molecular analysis in DBS it was possible to differentiate the results for normal individuals, heterozygous and affected for the mutation p.H178L. The preliminary results available indicate that the protocol of neonatal screening for MPS VI developed in this work can be easily incorporated by reference laboratories, contributing to the detection and premature treatment of MPS VI affected patients.

Ce travail vise à comprendre et quantifier le comportement de U(VI) dans les argilites du Callovo-Oxfordien(COₓ), prévues pour accueillir les déchets nucléaires de haute activité en France. L’effet de la température sur ce comportement est particulièrement étudié. La première partie du travail s'est concentrée sur les propriétés thermodynamiques des complexes ternaires Ca-U(VI)-CO₃ qui contrôlent la spéciation de U(VI) en solution. Ces dernières ont été mesurées par une méthode de compétition en présence d’une résine sous pCO₂ et pH contrôlés. Les résultats indiquent que la température ne favorise pas la formation de CaUO₂(CO₃)₃²⁻ (log₁₀ βº₁₁₃ = 27,3 ± 0,3 ; ΔrHº = -27,4 ± 8 kJ/mol) et n'affecte pas la formation de Ca₂UO₂(CO₃)₃(aq)(log₁₀ βº₂₁₃ = 29,7 ± 0,3 ; ΔrHº = 0 ± 2 kJ/mol). Une approche « bottom-up » avec le modèle « 2SPNE SC /CE » publié dans la littérature a été suivie pour décrire les phénomènes de sorption, en considérant que la fraction argileuse du COₓ (Illite et I/S) gouverne la sorption de U(VI). Ce modèle a été appliqué avec succès pour reproduire une multitude de données expérimentales obtenues avec l'illite, la fraction argileuse du COₓ et les argilites du COₓ en fonction de paramètres clés (pH, pCO₂, [U(VI)], [Ca]) à 20 °C. Le phénomène de rétention dans les conditions in-situ est principalement régi par la sorption des complexes U(VI)-carbonate et une nouvelle constante d´équilibre de réaction de complexation de surface est proposée pour l'illite. Une augmentation de la température à 80 °C conduit à une augmentation de la rétention de U(VI) sur le COₓ. Cette augmentation est accompagnée d'un changement de pCO₂et de pH. Le modèle de rétention testé à 20 °C combiné avec les paramètres thermodynamiques décrivant le comportement de U(VI) en solution expliquent cette augmentation sans pour autant obtenir un accord satisfaisant avec l’expérience. Le modèle est amélioré en intégrant des valeurs de ΔrHº obtenues pour les réactions de complexation de surface à partir du système U(VI)/illite. / The aim of this study was to understand and quantify the behaviour of U(VI) on the Callovo-Oxfordian(COx) clay which is envisioned to host high-level radioactive waste in France. The temperature effect up to 80°C on this behaviour was particularly studied. The first part of the work focussed on the thermodynamic properties of the calcium uranyl carbonate aqueous complexes which govern U(VI) speciation in solution. They were measured indirectly by sorption-based methodologies under controlled pCO₂ and pH. The results indicate that the temperature does not favour the formation of CaUO₂(CO₃)₃²⁻ (log₁₀ βº₁₁₃ = 27,3 ± 0,3 ; ΔrHº = -27,4 ± 8 kJ/mol) and does not affect the formation of Ca₂UO₂(CO₃)₃(aq)(log₁₀ βº₂₁₃ = 29,7 ± 0,3 ; ΔrHº = 0 ± 2 kJ/mol). A bottom-up approach with the published “2SPNE SC/CE”model was used for describing the sorption processes, with the assumption that the clay fraction of the COx (Illite, andI/S) governs U(VI) sorption.The model was successfully applied to reproduce a wealth of experimental data obtained with illite, the COₓ clay fractionand the COₓ clay rock as a function of key parameters (pH, pCO2, [U(VI)], [Ca]) at 20 °C. The sorption on COₓ conditions is mainly governed by the sorption of U(VI)-CO3 complexes and a new sorption constant is proposed for illite. An increase in temperature to 80 °C leads to an in-crease of U(VI) retention on COx. This increase is ac-companiedby a change of both pCO₂ and pH. The sorption model developed at 20 °C, together with the thermodynamic parameters describing U(VI) speciation in solution, can explain this increase but without obtaining a good agreement with the experiment. The model is improved by considering ΔrHº values for sur-face complexation reactions obtained for the U(VI))/illite system.

Le système de sécrétion de type VI (SST6) est un complexe multi-protéique permettant l’export d’effecteurs. Ce mécanisme est impliqué à la fois dans la virulence envers les cellules eucaryotes, dans l’activité antibactérienne mais également dans l’acquisition d’ions présents dans ’environnement. Ainsi, le SST6 joue un rôle important dans l’adaptation et la compétition, éléments essentiels dans la colonisation et la persistance au sein d’une niche écologique. Actuellement, très peu d’études portent sur l’importance du SST6 chez des souches environnementales, contrairement aux nombreuses études portant sur des pathogènes tels que Pseudomonas aeruginosa, Burkholderia thailandensis, Vibrio cholerae ou Escherichia coli. Mon sujet de recherche avait pour objectif de caractériser le ou les rôles du SST6 de la souche environnementale Pseudomonas fluorescens MFE01. Ces travaux ont permis d’appréhender certaines fonctions du SST6 de cette souche. Le génome de MFE01 ne comporte qu’un seul cluster de gènes du SST6 où sont regroupés les gènes codant pour la machinerie du SST6 (le « core-component ») à l’exception des gènes hcp. Les protéines Hcp sont des éléments structuraux du SST6 dont elles forment le tube interne qui permet le transfert des effecteurs. Différents gènes hcp sont disséminés sur le chromosome et parmi ces « hcp » orphelins, hcp2 et hcp3 codent respectivement pour les protéines Hcp2 et Hcp3. Ces deux Hcp sécrétées par le SST6, sont associées à l’activité antibactérienne de MFE01 sur différentes souches pathogènes et environnementales, tels que P. aeruginosa, P. fluorescens MFN1032 et Pectobacterium atrosepticum. La protéine Hcp1, codée par le gène orphelin hcp1, est impliquée dans l’inhibition de mobilité de souche compétitrice. Hcp1 permettrait la sécrétion d’au moins deux toxines qui perturberaient l’assemblage du flagelle. Chez MFE01Δhcp1 et MFE01ΔtssC (TssC est un élément de la gaine contractile du SST6), ces toxines seraient accumulées dans le cytoplasme, inhibant ainsi ’assemblage de leur propre flagelle. La surproduction du régulateur FliA, qui contrôle notamment l’assemblage du filament flagellaire, restaure la mobilité chez ces deux mutants. En parallèle, le SST6 de la souche MFE01 est essentiel à la formation et la maturation de biofilm mais également à la compétition bactérienne en biofilm mixte. Ce système interviendrait dans la communication bactérienne indispensable au comportement social, requis lors de l’élaboration des biofilms. / Type VI secretion system (T6SS) is a multiproteic apparatus that secreted proteinaceous effectors. T6SS participate in a variety of functions, whose eukaryote virulence, antibacterial activity or metal ion uptake. These capacities conferring an advantage in adaptation and competition, crucial to colonization or persistence within ecological niche. As well, only a few studies have focused on the T6SS functions of environmental strains, contrary to numerous studies dealing with pathogens as Pseudomonas aeruginosa, Burkholderia thailandensis, Vibrio cholerae or Escherichia coli. The purpose of my research project was to characterize the T6SS function(s) of the environmental strain Pseudomonas fluorescens MFE01. This work had led to understand the various functions of T6SS of MFE01 strain. This strain has a single T6SS cluster where all the core component proteins were gathered, except hcp genes. Three orphan hcp genes where found and are scattered in genome. Hcp proteins form the inner tube allowing effectors secretion. Both Hcp2 and Hcp3 proteins were involved in antibacterial activity on pathogens or environmental strains like P. aeruginosa, P. fluorescens or Pectobacterium atrosepticum. Characterization of Hcp1 proteins role constituted a major focus of this project. Hcp1 proteins participate to motility inhibition of competitive strains through T6SS. Hcp1 may be associated with secretion of at least two toxins perturbing the flagellar filament assembly. In MFE01Δhcp1 and MFE01ΔtssC mutants (Tss is a contractile sheath constituent), these toxins may be accumulated into cytoplasm and perturb assembly of their own flagella. Interestingly, overproduction of FliA flagellar regulator, which controls assembly of flagellar filament, restores motility of both mutants. Simultaneously, T6SS of MFE01 strain contributes to maturation and biofilm formation but also in bacterial competition within mixed biofilm. T6SS may be a mean of bacterial communication and thus coordinate a social behavior, primordial for biofilm formation.

Made available in DSpace on 2014-06-11T19:26:21Z (GMT). No. of bitstreams: 0 Previous issue date: 2010-05-20Bitstream added on 2014-06-13T19:54:35Z : No. of bitstreams: 1 lima_ccr_me_fran.pdf: 1385718 bytes, checksum: 43bd0b708861182d6d621af1c8cc5897 (MD5) / Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES) / Propõe-se, nesta pesquisa, refletir sobre a formação do pensamento brasileiro no período que antecede ao denominado Romantismo, destacando a “participação”, durante o momento de passagem da colônia para país independente, da literatura de viagem no processo de formação da identidade nacional. Através da leitura de alguns periódicos que circularam no Rio de Janeiro no início do século XIX, mais precisamente entre a chegada da corte (1808) e o lançamento da revista Nitheroy (1836), pretende-se demonstrar como a formação da cultura escrita nacional esteve intimamente ligada à ideia que os europeus tinham dos trópicos. Para tanto, a proposta é mapear em tais periódicos as menções aos viajantes estrangeiros e ao conteúdo de suas narrativas, de modo a conhecer o que a intelectualidade carioca dos primeiros decênios dos oitocentos pensava das imagens do Brasil e dos brasileiros vinculadas nestes escritos / The objective of this work is to study the construction of the brazilian thought in the period that is right before the Romantism age, highlighting the participation of the type of literature known as 'travel literature' in the process of construction of the national identity when Brazil was being transformed from a colony into an independent country. Based on journals that were on circulation in the beginning of the 19th century, precisely in the period between the Court arrives in the country (1808) and the release of the magazine Nitheroy (1836), and conceiving them as the stands of the construction of the country identity, we aim to show how the formation of the national literature was attached to the idea that european people had about the tropical lands. For that matter, the purpose is to map in this periodics the messages for the foreign travellers and the content of this narratives, such as references, quotes and comments in order to indicate the books that the intelectuals from Rio de Janeiro read in the first decades of the 18th century

Ce travail de thèse présente les caractéristiques optiques et électriques de dopants dans des couches de CdHgTe, CdZnTe et CdS. Ces 3 matériaux II-VI ont pour point commun d'être utilisés dans des dispositifs de détection, que ce soit la détection de lumière infrarouge pour les couches de CdHgTe et CdZnTe ou la détection visible comme c'est le cas pour le CdS. La caractérisation optique de ces couches de matériaux II-VI a été réalisée par la technique de photoluminescence et corrélée à des mesures électriques effectuées par effet Hall en température. Dans un premier temps, une étude du dopage intrinsèque par les lacunes de mercure et du dopage extrinsèque par incorporation d'arsenic de l'alliage CdHgTe, couche active des détecteurs IR a été réalisée. Pour cela, des mesures optiques par photoluminescence (sur un banc mis en place au laboratoire pendant la 1ere année de thèse permettant de travailler depuis les basses températures jusqu'à l'ambiante entre 1µm et 12 µm dans l'IR) sur des couches de CdHgTe réalisées par épitaxie en phase liquide (EPL) de différentes compositions en Cd ont été effectuées. La corrélation de ces mesures optiques avec des mesures électriques par effet Hall en température a permis d'identifier les énergies d'activation des 2 niveaux de la lacune de mercure ainsi que de démontrer le phénomène de U-négativité de la lacune de mercure dans le CdHgTe. De plus, la comparaison de spectres de PL d'échantillons dopés arsenic pendant la croissance par épitaxie par jets moléculaires (EJM) avec des mesures disponibles réalisées par absorption de rayons X (EXAFS) a permis d'observer des transitions optiques associées aux différents complexes arsenic formés avant et pendant le recuit d'activation. Par ailleurs, un travail de modélisation du phénomène de désordre d'alliage dans le CdHgTe a été réalisé. Plus précisément, un modèle basé sur une statistique gaussienne associée aux fluctuations d'alliage autour d'un gap moyen et une statistique de Boltzman a été développé pour ajuster dans un premier temps des spectres d'absorption puis pour ajuster les spectres de photoluminescence. Ce modèle nous a permis d'ajuster étroitement les spectres de photoluminescence et d'absorption, tout en prenant en compte intrinsèquement le désordre d'alliage du matériau. Nous avons ainsi constaté que l'ajustement des spectres par des fonctions gaussiennes comme il est réalisé communément dans la littérature permet de trouver les bons écarts entre les pics d'émission et donc les bonnes énergies d'ionisation. Dans un deuxième temps, toujours dans le cas de la détection infrarouge, le travail a porté sur l'étude du substrat CdZnTe utilisé pour l'épitaxie du CdHgTe. Des comparaisons des spectres de PL avec les paramètres de croissance ont été effectuées. Plus particulièrement, une étude sur une zone spécifique de certains échantillons présentant une absorption du rayonnement IR a été réalisée afin d'en comprendre l'origine. Enfin, nous nous sommes intéressés à la couche de CdS, matériau II-VI dopé intrinsèquement (type n) utilisé comme fenêtre transparente et formant la jonction p-n avec le CdTe dans les cellules solaires, détecteurs de lumière visible. Dans cette partie, nous avons cherché à étudier l'influence des différentes méthodes de dépôt, sublimation ou bain chimique de la couche de CdS sur un substrat de verre, en comparant les spectres d'émission de photoluminescence obtenus ainsi que les types de traitements thermiques effectués après dépôts. Ces mesures ont été corrélées avec le rendement des cellules solaires finales. / This thesis presents a study of the optical and electrical characteristics of dopants in HgCdTe, CdZnTe and CdS, three materials that play a major role in industry. HgCdTe and CdZnTe are important for infrared light detection and CdS for the fabrication of solar cells. Layers of those II VI materials are characterized by photoluminescence (PL) and temperature-dependent Hall effect. The PL apparatus, built in house, is equipped with a liquid helium cryostat and allows one to scan the entire range of interest between 1 and 12 µm. We address one of the major problems in current HgCdTe technology: p-type doping by both Hg vacancies and arsenic. Low temperature PL and temperature-dependent Hall measurements are first carried out on HgCdTe layers grown by liquid phase epitaxy (LPE). They yield the activation energies of the 2 the Hg vacancy acceptor levels and reveal the U-negative nature of the Hg vacancy in HgCdTe. HgCdTe layers doped with As during molecular beam epitaxial (MBE) growth are also investigated by PL and the results correlated with existing X-ray absorption fine structure (EXAFS) studies. This work allows us to assign the observed optical transitions to the different arsenic complexes formed before and during activation annealing. Furthermore, a model for alloy disorder is developed to correctly fit the data. More precisely, a Gaussian model for the statistical fluctuation of the gap energy due to alloying is formulated and Boltzmann statistics is then introduced in order to fit both the transmission and the PL spectra. The introduction of such model allows one to find the correct emission peak energies, hence the correct ionization energies. Complementary to the study of HgCdTe layer growth, we examine the optical properties of CdZnTe substrates used for HgCdTe epitaxy. In particular, we correlate the PL spectra with the growth parameters, to understand the anomalous infrared absorption of certain CdZnTe ingots. Last, we became interested in CdS, an intrinsically doped (n-type) material used as transparent junction partner in CdTe in solar cells and visible light detectors. We study the impact of different deposition methods, namely sublimation or chemical bath on a glass substrate, by obtaining the PL spectra of as-grown layers and layers that underwent thermal treatment after deposition. These measurements are finally correlated with the final solar cell efficiency.

A mucopolissacaridose tipo VI (MPS VI) ou Síndrome de Maroteaux-Lamy, é uma doença autossômica recessiva causada pela deficiência da enzima lisossomal Nacetilgalactosamina- 4-sulfatase (ARSB), a qual resulta no armazenamento lisossômico de dermatan sufato em vários tecidos e órgãos, dando origem a uma condição clínica de espectro variável, desde formas mais graves até formas mais atenuadas. O acúmulo de substrato não degradado causa um importante comprometimento ósseo, problemas respiratórios, baixa estatura e outros problemas, afetando os olhos, o coração e outros órgãos. Embora a síndrome de Maroteaux-Lamy não tenha uma incidência definida no Brasil, é reconhecido que, no nosso meio, é muito mais freqüente do que em outros países e regiões. Ela é particularmente freqüente no município de Monte Santo (Bahia), de aproximadamente 50.000 habitantes e onde já foram registrados 13 casos da doença. O diagnóstico é importante porque existe hoje um tratamento específico para a doença, a terapia de reposição enzimática (TRE), que vem mostrando bons resultados, especialmente quando iniciada em idade precoce. Descrevemos neste trabalho uma adaptação para microplacas da medida fluorimétrica da atividade de ARSB, e uma nova metodologia de análise molecular, ambas padronizados para sangue total impregnado em papel-filtro (STIPF). Essas técnicas foram desenvolvidas para incluir um teste de triagem neonatal para MPS VI, realizado nas amostras coletadas para o “teste do pezinho” nos neonatos do município de Monte Santo. Esses métodos permitem a detecção de pacientes com MPS VI e dos portadores da mutação específica que parece ser responsável pela alta incidência de MPS VI nessa localidade, uma vez que todos os pacientes lá diagnosticados apresentavam a mesma mutação (p.H178L) em homozigose. O trabalho foi desenvolvido em três etapas: na primeira foi realizada a padronização das técnicas em 100 amostras de STIPF; na segunda foi feito um teste-piloto com amostras de neonatos de Monte Santo, para avaliação das técnicas padronizadas e para o estudo de termoestabilidade em controles hígidos; na terceira foram analisadas amostras de STIPF de neonatos provenientes de Monte Santo pelos dois métodos (bioquímico e molecular). A padronização para realização da medida fluorimétrica da atividade enzimática de ARSB em microplacas indicou que o método é sensível, permitiu diferenciar os valores da população normal dos valores dos pacientes afetados e possibilitou a identificação segura de pacientes com MPS VI. Nas padronizações da análise molecular da mutação p.H178L em STIPF foi possível diferenciar os indivíduos normais, heterozigotos e homozigotos. Os resultados preliminares disponíveis indicam que o protocolo de triagem neonatal para MPS VI desenvolvido no presente trabalho poderá ser facilmente incorporado por laboratórios de referência, contribuindo para a detecção e tratamento precoce dos pacientes afetados por MPS VI. / Mucopolysaccharidosis type VI (MPS VI) or Maroteux-Lamy syndrome, is an autosomal recessive disorder caused by deficiency of the lysosomal enzyme Nacetylgalactosamine- 4-sulfatase (ARSB), which results in lysosomal storage of dermatan sufate in various tissues and organs and leads to a variable clinical spectrum, including more severe and attenuated forms. The accumulation of undegraded substrate causes bone involvement, respiratory problems and short stature, among other signs and symptoms, affecting the eyes, heart and other organs. Although the Maroteaux-Lamy syndrome does not have a defined incidence in Brazil, it is recognized that in our environment it is much more frequent than in other countries and regions. It is particularly frequent in the municipality of Monte Santo (Bahia) approximately 50,000 inhabitants and where there have already been 13 cases of the disease. The diagnosis is important because today there is a specific treatment for the disease, enzyme replacement therapy (ERT) which has shown good results, especially when started at an early age. We describe herein the standardization of the microplate fluorometric method for the ARSB test and a new methodology of molecular analysis, both adapted for dried blood spots (DBS) samples. These techniques were developed for inclusion of MPS VI in the newborn screening program that already tests the neonates of the city of Monte Santo, Bahia, Brasil for metabolic diseases. The methods were developed to detect patients with MPS VI and also for carriers, once the disease seems to have a high incidence (around 1:5.000) at this location. Also, all patients that have already been diagnosed in this city presented the same mutation (p.H178L) in homozygosis. The study was conducted in three stages: in the first was performed in 100 DBS samples an standardization of the techniques; in the second was done a pilot test with samples of newborns of Monte Santo, for the evaluation of standardized techniques and for the thermostability study in healthy controls; in the third were analyzed newborns samples from Monte Santo for both biochemical and molecular methods. Standardization on microplate for fluorimetric enzyme activity of the ARSB showed the assay sensitivity, differentiating values between normal and affected and allowing a reliable detection of patients with MPS VI. On the standardization for molecular analysis in DBS it was possible to differentiate the results for normal individuals, heterozygous and affected for the mutation p.H178L. The preliminary results available indicate that the protocol of neonatal screening for MPS VI developed in this work can be easily incorporated by reference laboratories, contributing to the detection and premature treatment of MPS VI affected patients.

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A mucopolissacaridose tipo VI (MPS VI) ou Síndrome de Maroteaux-Lamy, é uma doença autossômica recessiva causada pela deficiência da enzima lisossomal Nacetilgalactosamina- 4-sulfatase (ARSB), a qual resulta no armazenamento lisossômico de dermatan sufato em vários tecidos e órgãos, dando origem a uma condição clínica de espectro variável, desde formas mais graves até formas mais atenuadas. O acúmulo de substrato não degradado causa um importante comprometimento ósseo, problemas respiratórios, baixa estatura e outros problemas, afetando os olhos, o coração e outros órgãos. Embora a síndrome de Maroteaux-Lamy não tenha uma incidência definida no Brasil, é reconhecido que, no nosso meio, é muito mais freqüente do que em outros países e regiões. Ela é particularmente freqüente no município de Monte Santo (Bahia), de aproximadamente 50.000 habitantes e onde já foram registrados 13 casos da doença. O diagnóstico é importante porque existe hoje um tratamento específico para a doença, a terapia de reposição enzimática (TRE), que vem mostrando bons resultados, especialmente quando iniciada em idade precoce. Descrevemos neste trabalho uma adaptação para microplacas da medida fluorimétrica da atividade de ARSB, e uma nova metodologia de análise molecular, ambas padronizados para sangue total impregnado em papel-filtro (STIPF). Essas técnicas foram desenvolvidas para incluir um teste de triagem neonatal para MPS VI, realizado nas amostras coletadas para o “teste do pezinho” nos neonatos do município de Monte Santo. Esses métodos permitem a detecção de pacientes com MPS VI e dos portadores da mutação específica que parece ser responsável pela alta incidência de MPS VI nessa localidade, uma vez que todos os pacientes lá diagnosticados apresentavam a mesma mutação (p.H178L) em homozigose. O trabalho foi desenvolvido em três etapas: na primeira foi realizada a padronização das técnicas em 100 amostras de STIPF; na segunda foi feito um teste-piloto com amostras de neonatos de Monte Santo, para avaliação das técnicas padronizadas e para o estudo de termoestabilidade em controles hígidos; na terceira foram analisadas amostras de STIPF de neonatos provenientes de Monte Santo pelos dois métodos (bioquímico e molecular). A padronização para realização da medida fluorimétrica da atividade enzimática de ARSB em microplacas indicou que o método é sensível, permitiu diferenciar os valores da população normal dos valores dos pacientes afetados e possibilitou a identificação segura de pacientes com MPS VI. Nas padronizações da análise molecular da mutação p.H178L em STIPF foi possível diferenciar os indivíduos normais, heterozigotos e homozigotos. Os resultados preliminares disponíveis indicam que o protocolo de triagem neonatal para MPS VI desenvolvido no presente trabalho poderá ser facilmente incorporado por laboratórios de referência, contribuindo para a detecção e tratamento precoce dos pacientes afetados por MPS VI. / Mucopolysaccharidosis type VI (MPS VI) or Maroteux-Lamy syndrome, is an autosomal recessive disorder caused by deficiency of the lysosomal enzyme Nacetylgalactosamine- 4-sulfatase (ARSB), which results in lysosomal storage of dermatan sufate in various tissues and organs and leads to a variable clinical spectrum, including more severe and attenuated forms. The accumulation of undegraded substrate causes bone involvement, respiratory problems and short stature, among other signs and symptoms, affecting the eyes, heart and other organs. Although the Maroteaux-Lamy syndrome does not have a defined incidence in Brazil, it is recognized that in our environment it is much more frequent than in other countries and regions. It is particularly frequent in the municipality of Monte Santo (Bahia) approximately 50,000 inhabitants and where there have already been 13 cases of the disease. The diagnosis is important because today there is a specific treatment for the disease, enzyme replacement therapy (ERT) which has shown good results, especially when started at an early age. We describe herein the standardization of the microplate fluorometric method for the ARSB test and a new methodology of molecular analysis, both adapted for dried blood spots (DBS) samples. These techniques were developed for inclusion of MPS VI in the newborn screening program that already tests the neonates of the city of Monte Santo, Bahia, Brasil for metabolic diseases. The methods were developed to detect patients with MPS VI and also for carriers, once the disease seems to have a high incidence (around 1:5.000) at this location. Also, all patients that have already been diagnosed in this city presented the same mutation (p.H178L) in homozygosis. The study was conducted in three stages: in the first was performed in 100 DBS samples an standardization of the techniques; in the second was done a pilot test with samples of newborns of Monte Santo, for the evaluation of standardized techniques and for the thermostability study in healthy controls; in the third were analyzed newborns samples from Monte Santo for both biochemical and molecular methods. Standardization on microplate for fluorimetric enzyme activity of the ARSB showed the assay sensitivity, differentiating values between normal and affected and allowing a reliable detection of patients with MPS VI. On the standardization for molecular analysis in DBS it was possible to differentiate the results for normal individuals, heterozygous and affected for the mutation p.H178L. The preliminary results available indicate that the protocol of neonatal screening for MPS VI developed in this work can be easily incorporated by reference laboratories, contributing to the detection and premature treatment of MPS VI affected patients.

Les matériaux cimentaires sont largement utilisés dans la conception et la construction des sites de stockage de déchets radioactifs. Une des manières d’améliorer leur performance est d’introduire des adjuvants organiques dans la structure. La présence de matière organique dans l’eau porale peut affecter la mobilité des radionucléides : les molécules organiques forment des complexes solubles et peuvent être en compétition avec les radionucléides au niveau des sites de sorption. Ce travail avait pour but de comprendre les mécanismes de telles interactions au niveau moléculaire. Le système modèle a trois composantes. D’abord, des phases C-S-H ont été choisies en tant que modèles du ciment.Ensuite, le gluconate est sélectionné en tant que modèle d’additif organique pour sonder les mécanismes d’interaction à l’échelle moléculaire. Un système plus complexe impliquant un superplastifiant (PCE) a été testé. La troisième espèce, U(VI), est représentative d’un radionucléide de la série des actinides. Le développement de la description des effets de postproduction des espèces organiques pour les applications de stockage des déchets radioactifs était l’objectif principal de ce travail. L’étude des systèmes binaires fournit des données de référence pour l’investigation de systèmes ternaires C-S-H/matière organique/U(VI) plus complexes. Des cinétiques et des isothermes de sorption/désorption pour les espèces sur les C-S-H sont mesurés. En parallèle, des modèles atomiques ont été développés pour les interfaces d’intérêt. Les aspects structuraux, énergétiques et dynamiques des processus de sorption sur les surfaces de ciment sont modélisés par la technique de la dynamique moléculaire. / Cementitious materials are extensively used in the design and construction of radioactive waste repositories. One of the ways to enhance their performance is to introduce organic admixtures into the cement structure. However, the presence of organics in the pore water may affect the radionuclide mobility: organic molecules can form water-soluble complexes and compete for sorption sites. This work was designed to get detailed understanding of the mechanisms of such interactions on the molecular level. The model system has three components. First, pure C-S-H phases with different Ca/Si ratios were chosen as a cement model. Secondly, gluconate (a simple well-described molecule) is selected as a good starting organic additive model to probe the interaction mechanisms on the molecular scale. A more complex system involving polycarboxylate superplasticizer (PCE) was also tested. The third, U (VI), is a representative of the actinide radionuclide series. The development of description of the effects of organics for radioactive waste disposal applications was the primary objective of this work. The study of binary systems provides reference data for the investigation of more complex ternary (C-S-H/organic/U(VI)). The interactions are studied by means of both experimental and computational molecular modelling techniques. Data on sorption and desorption kinetics and isotherms for additives and for U (VI) on C-S-H are acquired in this work. In parallel, atomistic models are developed for the interfaces of interest. Structural, energetic, and dynamic aspects of the sorption processes on surface of cement are quantitatively modeled by molecular dynamics technique.