No description available.

Mucopolysaccharidosis IIIB (MPS IIIB) is a rare, metabolic disorder that results from a deficiency in the lysosomal hydrolase, α-N-acetylglucosaminidase (Naglu). Naglu is a housekeeping enzyme involved in the degradation pathway of heparan sulfate. A deficiency in active Naglu leads to an accumulation of heparan sulfate within the lysosome, initiating a pathological cascade within the cell. Patients with MPS IIIB experience progressive central nervous system degeneration and die within the first few decades of life. Presently, enzyme replacement therapy, which is a standard of care for other lysosomal storage disorders, is an ineffective treatment for MPS IIIB. This is due to impermeability of the blood-brain barrier (BBB) to exogenous recombinant enzymes. A promising approach to this therapeutic obstacle is protein transduction domains. Protein transduction domains have been shown to facilitate the delivery of active enzyme across the BBB in mice. Previously, our laboratory used Spodoptera frugiperda (Sf9) insect cell system to express human recombinant Naglu fused to a synthetic protein transduction domain (PTD4). The purpose was to use PTD4 to the facilitate the delivery of Naglu across biological membranes, including the blood-brain barrier. However, a missing stop codon following PTD4 limited its transducibility. The stop codon was re-introduced and the improved fusion enzyme, Naglu-PTD4X, was stably expressed in Sf9 cells. The overarching goal of this project is to create a large-scale production of human recombinant Naglu that has the potential to be used to treat the neuropathology of patients with MPS IIIB. This project used a three-step purification system to purify Naglu-PTD4X. Uptake of Naglu-PTD4X was assessed in MPS IIIB fibroblasts using a fluorogenic activity assay, immunoblotting, and immunocytochemistry. Our purification system was successful at purifying Naglu-PTD4X to homogeneity with a 26% yield and specific activity of 84,000 units/mg. An increase in Naglu activity was detected in MPS IIIB fibroblasts following incubation with Naglu-PTD4X. Future directions will focus on optimizing immunodetection and conducting BBB penetration studies in murine models. / Graduate / 2020-06-21

Introdução: As Doenças lisossômicas (DLs) são condições genéticas, herdadas na sua maioria de forma autossômica recessiva, caracterizadas usualmente pela deficiência de enzimas lisossômicas específicas, envolvidas na síntese, degradação, armazenamento ou transporte de macromoléculas necessárias para o funcionamento normal do organismo. Nas situações mais típicas, o substrato não degradado acumula-se progressivamente nos lisossomos, com repercussões estruturais e funcionais, levando a sinais e sintomas característicos. Os pacientes apresentam um amplo espectro de manifestações clínicas, que podem incluir disfunção de órgãos, anormalidades esqueléticas, envolvimento neuronal, entre outras. O diagnóstico é usualmente obtido pela identificação da deficiência enzimática específica em leucócitos obtidos do sangue periférico, usualmente realizado em laboratórios de referência. O transporte da amostra pode ser um obstáculo quando o serviço requisitante está situado longe do centro de referência ou em outro país, situação em que a amostra de sangue pode chegar ao laboratório já sem condições de ser analisada. Objetivo: Este estudo teve como objetivo principal, tornar disponível um método mais simples, seguro e acessível que utiliza amostras de leucócitos impregnados em papel filtro (LIPF) como uma nova ferramenta para o diagnóstico bioquímico de pacientes com DLs. Métodos: O estudo envolveu amostras de pacientes com diagnóstico previamente confirmado de DLs (amostra de conveniência, por se tratarem de doenças raras, com incidências individuais ao redor de 1:100.000 recém-nascidos vivos). Foram incluídos no estudo os pacientes com diagnóstico já estabelecido de DLs selecionadas (MPS IVA, Doença de Krabbe, Doença de Gaucher e Doença de Pompe), independente do sexo e/ou idade, atendidos no Serviço de Genética Médica do HCPA, que concordaram em participar do estudo. O grupo de referência negativo foi constituído pelas amostras de 50 indivíduos hígidos, adultos, de ambos os sexos. Resultados: Os resultados obtidos nos ensaios enzimáticos de pacientes com MPS IVA, Doença de Krabbe, Doença de Gaucher e Doença de Pompe, indicaram que as enzimas analisadas em amostras de LIPF permitiram a identificação de todos os pacientes, com sensibilidade de 100%. Os testes de estabilidade realizados nas amostras de LIPF indicaram que as amostras, quando mantidas a 4ºC, se mostram estáveis por pelo menos 30 dias. Conclusões: Nas condições utilizadas, amostras de LIPF se mostraram adequadas para a identificação segura de pacientes com MPS tipo IVA, Doença de Krabbe, Doença de Gaucher e Doença de Pompe. As amostras de leucócitos secos em papel filtro são mais estáveis e seguras para o transporte, indicando que possa ser esta uma importante ferramenta para facilitar a identificação de pacientes com DLDs, especialmente daqueles que vivem em áreas que tem dificuldades para a remessa de amostras líquidas para serviços de referência. / Background: Lysosomal Disorders (LDs) are genetic conditions, mostly inherited in autosomal recessive fashion, usually characterized by a deficiency of specific lysosomal enzymes involved in the synthesis, degradation, storage or transportation of macromolecules necessary for normal functioning of the organism. Typically, the non-degraded substrate is progressively accumulated in lysosomes, with structural and functional repercussions, leading to characteristic signs and symptoms. Affected patients present a wide range of clinical manifestations, which may include organ dysfunction, skeletal anomalies, neuronal involvement, etc. The diagnosis is normally made through identification of the specific enzyme deficiency in white blood cells from a sample of peripheral blood, usually performed in reference laboratories. The transporting of a liquid sample can be a problem when the test orderer is located far from the reference center or in a foreign country, as often the blood sample arrives at the laboratory in poor condition and cannot be properly analyzed. Aim: The main aim of this study was to make available a new technique that is simpler, safer and more accessible, using leukocytes impregnated on filter paper (LIFP) as a new tool for the biochemical diagnosis of patients with LSDs. Methods: This study involved samples of patients with previously confirmed diagnosis of selected LSDs (a convenience sample, as these are rare diseases, with individual incidences around 1:100.000 live newborns). Patients with an established diagnosis of MPS IVA, Krabbe Disease, Gaucher’s disease and Pompe disease regardless of sex and/or age, cared for at the Genetics Service of HCPA and who agreed to participate were included in the study. The negative reference group comprised blood samples from 50 healthy adults of both genders. Results: The results obtained in the enzymatic assays of patients with MPS IVA, Krabbe Disease, Gaucher’s disease, and Pompe disease indicated that the analyzed enzymes in LIFP samples allowed the identification of all patients, with sensitivity of 100%. The stability tests performed in LIFP samples indicated that samples, when maintained at 4ºC, were stable for at least 30 days. Conclusions: In the conditions used, LIFP samples were shown to be adequate for a reliable identification of patients with MPS IVA, Krabbe Disease, Gaucher’s disease, and Pompe disease. Blood samples on filter paper are more stable and reliable for transportation, indicating that this may be an important tool to facilitate the identification of patients with LSDs, particularly those living in areas with difficulties for the shipment of liquid samples to reference cervices.

Introdução: As Doenças lisossômicas (DLs) são condições genéticas, herdadas na sua maioria de forma autossômica recessiva, caracterizadas usualmente pela deficiência de enzimas lisossômicas específicas, envolvidas na síntese, degradação, armazenamento ou transporte de macromoléculas necessárias para o funcionamento normal do organismo. Nas situações mais típicas, o substrato não degradado acumula-se progressivamente nos lisossomos, com repercussões estruturais e funcionais, levando a sinais e sintomas característicos. Os pacientes apresentam um amplo espectro de manifestações clínicas, que podem incluir disfunção de órgãos, anormalidades esqueléticas, envolvimento neuronal, entre outras. O diagnóstico é usualmente obtido pela identificação da deficiência enzimática específica em leucócitos obtidos do sangue periférico, usualmente realizado em laboratórios de referência. O transporte da amostra pode ser um obstáculo quando o serviço requisitante está situado longe do centro de referência ou em outro país, situação em que a amostra de sangue pode chegar ao laboratório já sem condições de ser analisada. Objetivo: Este estudo teve como objetivo principal, tornar disponível um método mais simples, seguro e acessível que utiliza amostras de leucócitos impregnados em papel filtro (LIPF) como uma nova ferramenta para o diagnóstico bioquímico de pacientes com DLs. Métodos: O estudo envolveu amostras de pacientes com diagnóstico previamente confirmado de DLs (amostra de conveniência, por se tratarem de doenças raras, com incidências individuais ao redor de 1:100.000 recém-nascidos vivos). Foram incluídos no estudo os pacientes com diagnóstico já estabelecido de DLs selecionadas (MPS IVA, Doença de Krabbe, Doença de Gaucher e Doença de Pompe), independente do sexo e/ou idade, atendidos no Serviço de Genética Médica do HCPA, que concordaram em participar do estudo. O grupo de referência negativo foi constituído pelas amostras de 50 indivíduos hígidos, adultos, de ambos os sexos. Resultados: Os resultados obtidos nos ensaios enzimáticos de pacientes com MPS IVA, Doença de Krabbe, Doença de Gaucher e Doença de Pompe, indicaram que as enzimas analisadas em amostras de LIPF permitiram a identificação de todos os pacientes, com sensibilidade de 100%. Os testes de estabilidade realizados nas amostras de LIPF indicaram que as amostras, quando mantidas a 4ºC, se mostram estáveis por pelo menos 30 dias. Conclusões: Nas condições utilizadas, amostras de LIPF se mostraram adequadas para a identificação segura de pacientes com MPS tipo IVA, Doença de Krabbe, Doença de Gaucher e Doença de Pompe. As amostras de leucócitos secos em papel filtro são mais estáveis e seguras para o transporte, indicando que possa ser esta uma importante ferramenta para facilitar a identificação de pacientes com DLDs, especialmente daqueles que vivem em áreas que tem dificuldades para a remessa de amostras líquidas para serviços de referência. / Background: Lysosomal Disorders (LDs) are genetic conditions, mostly inherited in autosomal recessive fashion, usually characterized by a deficiency of specific lysosomal enzymes involved in the synthesis, degradation, storage or transportation of macromolecules necessary for normal functioning of the organism. Typically, the non-degraded substrate is progressively accumulated in lysosomes, with structural and functional repercussions, leading to characteristic signs and symptoms. Affected patients present a wide range of clinical manifestations, which may include organ dysfunction, skeletal anomalies, neuronal involvement, etc. The diagnosis is normally made through identification of the specific enzyme deficiency in white blood cells from a sample of peripheral blood, usually performed in reference laboratories. The transporting of a liquid sample can be a problem when the test orderer is located far from the reference center or in a foreign country, as often the blood sample arrives at the laboratory in poor condition and cannot be properly analyzed. Aim: The main aim of this study was to make available a new technique that is simpler, safer and more accessible, using leukocytes impregnated on filter paper (LIFP) as a new tool for the biochemical diagnosis of patients with LSDs. Methods: This study involved samples of patients with previously confirmed diagnosis of selected LSDs (a convenience sample, as these are rare diseases, with individual incidences around 1:100.000 live newborns). Patients with an established diagnosis of MPS IVA, Krabbe Disease, Gaucher’s disease and Pompe disease regardless of sex and/or age, cared for at the Genetics Service of HCPA and who agreed to participate were included in the study. The negative reference group comprised blood samples from 50 healthy adults of both genders. Results: The results obtained in the enzymatic assays of patients with MPS IVA, Krabbe Disease, Gaucher’s disease, and Pompe disease indicated that the analyzed enzymes in LIFP samples allowed the identification of all patients, with sensitivity of 100%. The stability tests performed in LIFP samples indicated that samples, when maintained at 4ºC, were stable for at least 30 days. Conclusions: In the conditions used, LIFP samples were shown to be adequate for a reliable identification of patients with MPS IVA, Krabbe Disease, Gaucher’s disease, and Pompe disease. Blood samples on filter paper are more stable and reliable for transportation, indicating that this may be an important tool to facilitate the identification of patients with LSDs, particularly those living in areas with difficulties for the shipment of liquid samples to reference cervices.

Introdução: As Doenças lisossômicas (DLs) são condições genéticas, herdadas na sua maioria de forma autossômica recessiva, caracterizadas usualmente pela deficiência de enzimas lisossômicas específicas, envolvidas na síntese, degradação, armazenamento ou transporte de macromoléculas necessárias para o funcionamento normal do organismo. Nas situações mais típicas, o substrato não degradado acumula-se progressivamente nos lisossomos, com repercussões estruturais e funcionais, levando a sinais e sintomas característicos. Os pacientes apresentam um amplo espectro de manifestações clínicas, que podem incluir disfunção de órgãos, anormalidades esqueléticas, envolvimento neuronal, entre outras. O diagnóstico é usualmente obtido pela identificação da deficiência enzimática específica em leucócitos obtidos do sangue periférico, usualmente realizado em laboratórios de referência. O transporte da amostra pode ser um obstáculo quando o serviço requisitante está situado longe do centro de referência ou em outro país, situação em que a amostra de sangue pode chegar ao laboratório já sem condições de ser analisada. Objetivo: Este estudo teve como objetivo principal, tornar disponível um método mais simples, seguro e acessível que utiliza amostras de leucócitos impregnados em papel filtro (LIPF) como uma nova ferramenta para o diagnóstico bioquímico de pacientes com DLs. Métodos: O estudo envolveu amostras de pacientes com diagnóstico previamente confirmado de DLs (amostra de conveniência, por se tratarem de doenças raras, com incidências individuais ao redor de 1:100.000 recém-nascidos vivos). Foram incluídos no estudo os pacientes com diagnóstico já estabelecido de DLs selecionadas (MPS IVA, Doença de Krabbe, Doença de Gaucher e Doença de Pompe), independente do sexo e/ou idade, atendidos no Serviço de Genética Médica do HCPA, que concordaram em participar do estudo. O grupo de referência negativo foi constituído pelas amostras de 50 indivíduos hígidos, adultos, de ambos os sexos. Resultados: Os resultados obtidos nos ensaios enzimáticos de pacientes com MPS IVA, Doença de Krabbe, Doença de Gaucher e Doença de Pompe, indicaram que as enzimas analisadas em amostras de LIPF permitiram a identificação de todos os pacientes, com sensibilidade de 100%. Os testes de estabilidade realizados nas amostras de LIPF indicaram que as amostras, quando mantidas a 4ºC, se mostram estáveis por pelo menos 30 dias. Conclusões: Nas condições utilizadas, amostras de LIPF se mostraram adequadas para a identificação segura de pacientes com MPS tipo IVA, Doença de Krabbe, Doença de Gaucher e Doença de Pompe. As amostras de leucócitos secos em papel filtro são mais estáveis e seguras para o transporte, indicando que possa ser esta uma importante ferramenta para facilitar a identificação de pacientes com DLDs, especialmente daqueles que vivem em áreas que tem dificuldades para a remessa de amostras líquidas para serviços de referência. / Background: Lysosomal Disorders (LDs) are genetic conditions, mostly inherited in autosomal recessive fashion, usually characterized by a deficiency of specific lysosomal enzymes involved in the synthesis, degradation, storage or transportation of macromolecules necessary for normal functioning of the organism. Typically, the non-degraded substrate is progressively accumulated in lysosomes, with structural and functional repercussions, leading to characteristic signs and symptoms. Affected patients present a wide range of clinical manifestations, which may include organ dysfunction, skeletal anomalies, neuronal involvement, etc. The diagnosis is normally made through identification of the specific enzyme deficiency in white blood cells from a sample of peripheral blood, usually performed in reference laboratories. The transporting of a liquid sample can be a problem when the test orderer is located far from the reference center or in a foreign country, as often the blood sample arrives at the laboratory in poor condition and cannot be properly analyzed. Aim: The main aim of this study was to make available a new technique that is simpler, safer and more accessible, using leukocytes impregnated on filter paper (LIFP) as a new tool for the biochemical diagnosis of patients with LSDs. Methods: This study involved samples of patients with previously confirmed diagnosis of selected LSDs (a convenience sample, as these are rare diseases, with individual incidences around 1:100.000 live newborns). Patients with an established diagnosis of MPS IVA, Krabbe Disease, Gaucher’s disease and Pompe disease regardless of sex and/or age, cared for at the Genetics Service of HCPA and who agreed to participate were included in the study. The negative reference group comprised blood samples from 50 healthy adults of both genders. Results: The results obtained in the enzymatic assays of patients with MPS IVA, Krabbe Disease, Gaucher’s disease, and Pompe disease indicated that the analyzed enzymes in LIFP samples allowed the identification of all patients, with sensitivity of 100%. The stability tests performed in LIFP samples indicated that samples, when maintained at 4ºC, were stable for at least 30 days. Conclusions: In the conditions used, LIFP samples were shown to be adequate for a reliable identification of patients with MPS IVA, Krabbe Disease, Gaucher’s disease, and Pompe disease. Blood samples on filter paper are more stable and reliable for transportation, indicating that this may be an important tool to facilitate the identification of patients with LSDs, particularly those living in areas with difficulties for the shipment of liquid samples to reference cervices.

Notre questionnement trouve sa source dans la mise en place, dans le cadre de la réforme du lycée de 2010, de l'enseignement d'exploration Méthodes et Pratiques Scientifiques (MPS) en Seconde. Cet enseignement pluridisciplinaire, visant à initier les élèves à la démarche scientifique dans le cadre d'un projet, a conduit les enseignants de mathématiques, à interroger et à modifier leurs pratiques. Nous proposons d'analyser ces changements sous l'angle des ressources et du travail des enseignants sur ces ressources. Nous mobilisons les cadres théoriques de la double approche didactique et ergonomique (Robert, 2010a ; Rogalski, 2010) et de l'approche documentaire du didactique (Gueudet et Trouche, 2010). Notre recherche est conduite à partir du suivi de professeurs de mathématiques impliqués en MPS et engagés dans différents collectifs disciplinaires et pluridisciplinaires. Ce suivi est complété par des entretiens avec les collègues des autres disciplines. Nous avons aussi effectué une étude sur l'offre des ressources disponibles pour l'un des thèmes proposés. Cette étude s'inscrit dans le programme de recherche ReVEA (Ressources Vivantes pour l'Enseignement et l'Apprentissage), soutenu par l'ANR. Notre thèse propose de montrer l'impact des ressources et de leur manque depuis la compréhension de la tâche prescrite jusqu'à la tâche effective, ainsi que celui des différents collectifs dans lesquels le professeur de mathématiques est impliqué. Elle questionne également les effets de ce travail sur le développement professionnel concernant en particulier la conception et la mise en œuvre de séances fondées sur des démarches d'investigation. / Our questioning emerges from the establishment of a new teaching, called Scientific Methods and Practices (MPS), as part of the French high school reform in 2010. This multidisciplinary teaching, which aims to initiate pupils to a scientific approach, leds mathematics teachers to question and to change their practices. We propose to analyze these changes in terms of resources and the way teachers work on these resources. We retain two approaches: the double approach didactic and ergonomic of the teaching practices (Robert, 2010a; Rogalski, 2010) and the documentational approach of didactics (Gueudet et Trouche, 2010). Our research is conducted from observations of mathematics teachers involved in MPS and engaged in different disciplinary and multidisciplinary collectives. These observations are supplemented by interviews with colleagues in other disciplines. We also performed a study of resources available for one of the themes proposed. This study is part of the research program ReVEA (Living Resources for Teaching and Learning), supported by the ANR. Our thesis aims to show the impact of resources and lack of resources from understanding the prescribed task to the effective task, and also the impact of the different collectives in which the mathematics teacher is involved. It questions as well the effects of this work on professional development, especially regarding the inquiry-based science teaching.

No description available.

The thesis deals with production planning. It contains theoretical description of methods used for production planning and optimizing. Thesis describes bottleneck problems in production. It offers overview of ways to identify and analyze bottleneck influence to manufacturing process efficiency. Thesis proposes ways to eliminate bottlenecks using various algorithm types. It applies theoretical knowledges from optimization and graph theory to program creation that is focused on order delay and readjustment time minimizing. The program implements genetic algorithm.

Dans le contexte des transports plus électriques, les parties mécaniques tendent à être remplacées par leurs équivalents électriques plus petits. Ainsi, le composant lui-même doit supporter un environnement plus sévère et de lourdes contraintes (haute tension, haute température). Les composants silicium deviennent alors inappropriés. Depuis la commercialisation des premières diodes Schottky en 2001, le carbure de silicium est le matériau reconnu mondialement pour la fabrication de dispositifs haute tension avec une forte intégration. Sa large bande d'énergie interdite et son fort champ électrique critique permettent la conception de transistors à effet de champ avec jonction (JFET) pour les hautes tensions ainsi que les diodes associées. Les structures étudiées dépendent de nombreux paramètres, et doivent ainsi être optimisées. L'influence d'un paramètre ne pouvant être isolée, des méthodes mathématiques ont été appelées pour trouver la valeur optimale. Ceci a conduit à la mise en place d'un critère d'optimisation. Ainsi, les deux grands types de structures de JFET verticaux ont pu être analysés finement. D'une part, la recherche d'une structure atteignant les tensions les plus élevées possible a conduit à l'élaboration d'un procédé de fabrication complexe. D'autre part, un souci de simplification et de stabilisation des procédés de fabrication a permis le développement d'un composant plus simple, mais avec une limite en tension un peu plus modeste. / In the context of more electrical transports, mechanical devices tend to be replaced by their smaller electrical counterparts. However the device itself must support harsher environment and electrical constraints (high voltage, high temperature) thus making existing silicon devices inappropriate. Since the first Schottky diode commercialization in 2001, Silicon Carbide (SiC) is the favorite candidate for the fabrication of devices able to sustain high voltage with a high integration level. Thanks to its wide band gap energy and its high critical field, 4H-SiC allows the design of high voltage Junction Field Effect Transistor (JFET) with its antiparallel diode. Studied structures depends of many parameters, that need to be optimized. Since the influence of the variation of each parameter could not be isolated, we tried to find mathematical methods to emphase optimal values leading to set an optimization criterion. Thus, two main kinds of JFET structure were finely analyzed. In one hand, the aim of the structure that can sustain a voltage as high as possible leads to a complex fabrication process. In the other hand, the care of a simplification and a stabilization of manufacturing process leads to the design of simpler device, but with a bit less sustain capabilities.

Cette thèse étudie dans une perspective comparée les modalités du travail de représentation en matière d’impôt sur la fortune, au Bundestag et à l’Assemblée nationale. S’appuyant sur des approches empiriques complémentaires (entretiens semi-directs, observation non-participante, bases de données prosopographiques, travail d’archive codé), cette enquête entend expliquer la variété des pratiques constatées. La recherche montre que les débats en matière d’impôt sur la fortune se caractérisent par la place centrale prise par les mondes de l’entreprise, faisant émerger le constat d’une forme d’inégalité d’accès à la parole parlementaire pour les groupes sociaux cités au sein des deux Assemblées. L’enquête souligne également que les députés développent des pratiques différenciées entre groupes en utilisant plusieurs registres de représentation visant à justifier et légitimer les différents modes d’investissement observables dans les deux pays. Face à ce constat, cette étude entend proposer un examen des causes des phénomènes observés. Plus d’une trentaine de facteurs explicatifs sont ainsi pris en compte, à des niveaux micro, méso et macrosociologiques (histoire politique, dispositions individuelles, processus de socialisation politique, économique et professionnelle, influence des entourages, effets de positions et de contextes, etc.). La thèse montre alors que les pratiques de représentation sont le résultat de l’articulation d’un système de facteurs qui interdisent de penser ces pratiques de façon réifiée. En cherchant à comprendre la complexité et l’intrication de nombreux éléments intervenant dans les prises de position des élus, la thèse cherche à démêler les fils explicatifs de l’écheveau des pratiques de représentation. / This thesis aims at analysing the methods of representing during wealth tax debates in the French Assemblée nationale and in the German Bundestag. Based on empirical researches (semi-direct interviews, non-participating observations, prosopographic data, coded archival work), this research aspires to explain the variety of identified practices. The research shows on one hand that debates around wealth tax are characterized by the central position of concerns and businesses, highlighting the following acknowledgment: the inequity in accessing speaking time for social groups mentioned in both Parliaments. The thesis also underlines that members of parliament develop differentiated practices between groups by using multiple register of representation, which aim at justifying and legitimating all the different ways of commitment observed in both countries.Facing this observation, the thesis strive to provide a review of the causes of the observed phenomena. More than thirty explanatory factors are taken into consideration on micro, meso and macro sociological levels (political history, individual provisions, political/economical/professional socialisation processes, influence of close circle, effects of positions and environment). The thesis therefore demonstrates that the practices of representation are the result of a very high number of diversifying factors, which prevent from thinking those practices on a reified way. While trying to understand the complexity of new elements intervening in elected representatives’ position statements, the thesis aspires to untangle the explanatory threads of political representation. / Diese Doktorarbeit vergleicht die Modalitäten der politischen Repräsentation in Bezug auf die Vermögensteuer im Bundestag und in der Assemblée nationale. Die Recherche will Erklärungen geben für die Vielfalt der hierbei beobachteten Praktiken. Dafür stützt sie sich auf komplementäre empirische Materialien (Gespräche, Beobachtungen, prosoprographische Daten, codierte Archivarbeit). Die vorliegende Arbeit zeigt, dass die Debatten um die Vermögensteuer durch die zentrale Stellung der Unternehmen geprägt sind. Es existiert eine Form von ungleichem Zugang zur parlamentarischen Rede für die sozialen Gruppen innerhalb der beiden Versammlungen. Oft werden bestimmte soziale Gruppen einfach ignoriert. Die Studie verweist auch darauf, dass die Abgeordneten Praktiken von differenzierter Repräsentation zwischen Gruppen entwickeln, indem sie mehrere Register der Repräsentation benutzen, die darauf aus sind, verschiedene Modi der Investitionen zu rechtfertigen und zu legitimieren- in beiden Ländern. Vor diesem Hintergrund bietet diese Arbeit eine Analyse der Gründe der beobachteten Phänomene an. Dazu werden mehr als dreißig explikative Faktoren unter micro-, meso- und makrosoziologischen Gesichtspunkten untersucht (politische Geschichte, individuelle Dispositionen, Prozesse politischer, ökonomischer und professioneller Sozialisierung, Einflüsse des Umfelds, Einflüsse von institutionellen Positionen und Kontexten usw.). Diese Doktorarbeit will nachweisen, dass die Praktiken der Repräsentation das Ergebnis eines sehr hohen Anteils verschiedener Faktoren sind, die es untersagen diese Praktiken gedanklich zu verfestigen. Indem wir versuchen, die Komplexität und die Verstrickung der zahlreichen Elemente zu verstehen, die in den Stellungnahmen der Abgeordneten zum Ausdruck kommen, will diese Doktorarbeit die erklärenden Fäden des Knäuels der Repräsentationspraktiken entwirren.