A presente dissertação apresenta um estudo comparado entre Mário de Andrade e Bruno de Menezes entre os anos de 1923 e 1930. O objetivo do trabalho é a aproximação de suas trajetórias, compreender suas propostas estéticas sobre o Modernismo, suas pesquisas sobre cultura popular e o apreço por suas cidades. A pesquisa histórica se deu através do método comparativo do estudo da história e parte-se do pressuposto que dois escritores contemporâneos, precursores do movimento modernista em suas cidades São Paulo e Belém, que se encontram em estreita relação no que diz respeito aos temas por eles abordados. Para tanto o presente estudo se fundamenta na pesquisa de seus textos, nas analise de suas atuações como organizadores do Movimento Modernista de 1922, como folcloristas e como historiadores da cidade. Como procedimento metodológico realizou-se uma pesquisa de abordagem bibliográfica, com base nos estudos de Tele Ancona Lopez, Lafetá, Mário de Andrade e Bruno de Menezes. A relação de aproximação entre esses autores no contexto do novo ideal modernista foi, portanto a intenção que norteou este trabalho, ambos encontram-se nos ideais de renovação do pensamento estético de uma geração. Nesses termos, coincidem tanto em relação ao empenho em nutrir e desenvolver os ideais do Movimento modernista como em realçar elementos da cultura popular estabelecendo sua função social. Também compõe essa pesquisa a intenção de buscar nos objetos contemplados uma comparação entre suas experiências e pesquisas sobre a cultura popular, mais especificamente sobre a ideia de manifestação folclórica do Boi-Bumbá desenvolvida por estes autores, a qual rege parte de suas produções e constroem a ideia de modernidade. Além disso, o trabalho trata do relacionamento que ambos tinham com a cidade, de suas posturas que mesclavam observação, crítica e experimentação da vida urbana. Essas três atividades de modernistas, folcloristas e poetas da cidade estavam diretamente relacionadas e comprova que Mário de Andrade e Bruno de Menezes foram além de modernistas, autores modernos e interpretes da historia brasileira / This paper presents a comparative study between Mário de Andrade and Bruno de Menezes between the years 1923 and 1930. The objective is to approach their careers, understand their aesthetic proposals on Modernism, his research on popular culture and appreciation by cities. Historical research was performed using the comparative method to the study of history and we start from the assumption that two contemporary writers, precursors of the modernist movement in their cities Sao Paulo and Belem, which are in close relation with respect to the topics they addressed. For both the present study is based on research of their texts, the analysis of their performances as organizers of the Modernist Movement in 1922, as folklorists and historians as the city. As a methodological procedure was carried out a survey of bibliographic approach, based on studies of Tele Lopez Ancona, Lafetá, Mário de Andrade and Bruno de Menezes. The relationship of proximity between these authors in the context of the new modernist ideal was therefore the intention that guided this work, both are the ideals of renewal of aesthetic thought of a generation. These terms coincide both in relation to commitment to nurture and develop the ideals of the modernist movement as highlight elements of popular culture establishing their social function. He also composes this research the intention of seeking the objects contemplated a comparison between their experiences and research on popular culture, more specifically on the idea of folkloric manifestation of Boi developed by these authors, which governs part of their productions and build the idea of modernity. In addition, the work deals with the relationship they both had with the city, its postures that combined observation, experimentation and critique of urban life. These three modernist, folklorists and poets city activities were directly related and proves that Mário de Andrade and Bruno de Menezes were beyond modernist, modern authors and interpreters of Brazilian history

A presente dissertação apresenta um estudo comparado entre Mário de Andrade e Bruno de Menezes entre os anos de 1923 e 1930. O objetivo do trabalho é a aproximação de suas trajetórias, compreender suas propostas estéticas sobre o Modernismo, suas pesquisas sobre cultura popular e o apreço por suas cidades. A pesquisa histórica se deu através do método comparativo do estudo da história e parte-se do pressuposto que dois escritores contemporâneos, precursores do movimento modernista em suas cidades São Paulo e Belém, que se encontram em estreita relação no que diz respeito aos temas por eles abordados. Para tanto o presente estudo se fundamenta na pesquisa de seus textos, nas analise de suas atuações como organizadores do Movimento Modernista de 1922, como folcloristas e como historiadores da cidade. Como procedimento metodológico realizou-se uma pesquisa de abordagem bibliográfica, com base nos estudos de Tele Ancona Lopez, Lafetá, Mário de Andrade e Bruno de Menezes. A relação de aproximação entre esses autores no contexto do novo ideal modernista foi, portanto a intenção que norteou este trabalho, ambos encontram-se nos ideais de renovação do pensamento estético de uma geração. Nesses termos, coincidem tanto em relação ao empenho em nutrir e desenvolver os ideais do Movimento modernista como em realçar elementos da cultura popular estabelecendo sua função social. Também compõe essa pesquisa a intenção de buscar nos objetos contemplados uma comparação entre suas experiências e pesquisas sobre a cultura popular, mais especificamente sobre a ideia de manifestação folclórica do Boi-Bumbá desenvolvida por estes autores, a qual rege parte de suas produções e constroem a ideia de modernidade. Além disso, o trabalho trata do relacionamento que ambos tinham com a cidade, de suas posturas que mesclavam observação, crítica e experimentação da vida urbana. Essas três atividades de modernistas, folcloristas e poetas da cidade estavam diretamente relacionadas e comprova que Mário de Andrade e Bruno de Menezes foram além de modernistas, autores modernos e interpretes da historia brasileira / This paper presents a comparative study between Mário de Andrade and Bruno de Menezes between the years 1923 and 1930. The objective is to approach their careers, understand their aesthetic proposals on Modernism, his research on popular culture and appreciation by cities. Historical research was performed using the comparative method to the study of history and we start from the assumption that two contemporary writers, precursors of the modernist movement in their cities Sao Paulo and Belem, which are in close relation with respect to the topics they addressed. For both the present study is based on research of their texts, the analysis of their performances as organizers of the Modernist Movement in 1922, as folklorists and historians as the city. As a methodological procedure was carried out a survey of bibliographic approach, based on studies of Tele Lopez Ancona, Lafetá, Mário de Andrade and Bruno de Menezes. The relationship of proximity between these authors in the context of the new modernist ideal was therefore the intention that guided this work, both are the ideals of renewal of aesthetic thought of a generation. These terms coincide both in relation to commitment to nurture and develop the ideals of the modernist movement as highlight elements of popular culture establishing their social function. He also composes this research the intention of seeking the objects contemplated a comparison between their experiences and research on popular culture, more specifically on the idea of folkloric manifestation of Boi developed by these authors, which governs part of their productions and build the idea of modernity. In addition, the work deals with the relationship they both had with the city, its postures that combined observation, experimentation and critique of urban life. These three modernist, folklorists and poets city activities were directly related and proves that Mário de Andrade and Bruno de Menezes were beyond modernist, modern authors and interpreters of Brazilian history

The subject of this dissertation is the publishing of poetry in Sweden 1976–1995. The purpose is to examine the position of poetry in the Swedish book market and in the literary process. It is an empirical and statistical study based primarily on an inventory of the published works. The study shows that the publication of Swedish poetry collections in 1976–1995 consisted of 3 848 titles (new works only), which was more than ever before. Publication was consistent over the period, partly due to the allocation of the literature grant introduced by the Swedish government in 1975, but also to the technical development which made it possible for small and private publishers to release collections of poetry at a lower cost. The main publishers were the general publishing houses of Bonniers, Norstedts and Wahlström & Widstrand, but more than a third of the collections were published by vanity press and self publishers. Publication was strongly concentrated to the capital area. Regardless of the size of the publisher, poetry collections were printed in small numbers and generally sold poorly. Along with the technical development offset-printed books replaced duplicated publications, and more books were hardbound. The publishing houses made bigger efforts than ever before to publish female poets. The number increased over the period, but the men were still in a clear majority by 1995. The women were also largely responsible for rejuvenating the body of authors. The number of debutants was relatively constant during the period. The results in this dissertation indicate a hierarchic order among the publishing houses that determine the conditions for the authors and their works. This is verified through analyses of coverage in the national and regional daily papers, as well as three analyses of the authorships of Yngve Aldhagen, Else-Britt Kjellqvist and Bruno K. Öijer. The dissertation concludes that poetry exists on the publishing lists mainly for symbolical reasons; to publish poetry gives cultural capital to the publishers.

Made available in DSpace on 2014-06-11T19:26:35Z (GMT). No. of bitstreams: 0 Previous issue date: 2009-02-20Bitstream added on 2014-06-13T20:15:16Z : No. of bitstreams: 1 fantin_ep_me_assis.pdf: 822993 bytes, checksum: 7ebe138fa13a3d5d5fe72accc0ddde78 (MD5) / Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES) / Nossa pesquisa centra-se na análise dos cadernos que contêm recortes de jornais selecionados por Bruno Giovannetti, imigrante italiano que chegou ao Brasil na década de 1910 e fixou-se na região da Alta Sorocabana, onde desenvolveu várias atividades. Nesses cadernos, ele procurou, por meio de artigos e notas escritos por ele, e também por outros autores, publicados em diferentes jornais, registrar sua memória. A linha condutora do conteúdo presente nesses cadernos põe em evidência a construção positiva de sua imagem, principalmente no que se refere aos aspectos de sua vida profissional e intelectual. Assim, os cadernos, ao todo seis, podem ser compreendidos como a constituição de uma memória de si, pelos quais ele buscava dar um significado especial à sua vida, cujo registro consistia em provar a si próprio, bem como ao futuro, que ela merecia ser (re)vista, e, assim, lembrada. / This research aims the analysis of the notebooks that contain newspaper clippings selected by Bruno Giovannetti, an Italian immigrant that arrived in Brazil on the decade of 1910 and settled in the Alta Sorocabana region, where he developed many activities. In these notebooks, Giovannetti intended to register his memory, through the articles and notes written by him and by other writers, published on different newspapers. The conductor line of the content present in these notebooks evidences the positive construction of Giovannetti’s image, mainly the aspects of his professional and intellectual life. Thus, the notebooks, on the total of six, can be comprehended as a constitution of self memory. Through these notebooks, Giovannetti aimed to give a special meaning to his life, whose register consisted in proving to himself and to the future that it deserved to be seen (or seen again) and, with this, to be remembered.

[pt] A interferência da mídia no animus dos jurados em decisões proferidas pelo Tribunal do Júri é nítida, principalmente por ter como juízes o próprio povo. Um dos casos mais emblemáticos é o caso Eliza Samúdio, em que uma jovem teria sido assassinada brutalmente a mando do pai do próprio filho, um jogador de futebol famoso. Atuando em tempos e com discursos diferentes, mídia e judiciário seguiram seus caminhos. Caso complexo, repleto de enigmas e o envolvimento de uma pessoa famosa no meio popular apresentou-se perfeito à imprensa por concentrar em si os três assuntos que mais prendem a atenção popular: o crime, esporte e sexo. Além do enigma: onde estariam os restos mortais da jovem? Dia a dia, momento a momento, a imprensa seguia os passos do inquérito policial e do processo penal com o intuito de passar a notícia e com isso perceber os seus lucros, sendo notório que essa cobertura privilegiou a fase de inquérito e do julgamento, sempre enfatizando a personalidade de Bruno Fernandes como fora dos padrões normais. A mídia assim, autoafirmava como autoridade moralizadora, utilizando da narrativa para tanto. Ao fim, viu-se a ratificação de um veredicto já proferido pela mídia muito tempo antes do julgamento, tendo em vista o populismo penal midiático em que Bruno Fernandes fora condenado à reprimenda maior do que no direito se admitiria, principalmente porque o julgamento pelo Tribunal do Júri é feito através do povo, que confirmou o que escutou durante quase três anos. / [en] The interference in the media animus of jurors in decisions made by court s jury is clear, mainly because the judges were their people. One of the most emblematic cases is the Eliza Samúdio, where a young woman had been brutally murdered at the behest of the father of his own son, a famous soccer player. Acting upon different times and speeches, media and judicial branches went their ways. Complex case replete with enigma and the involvement of a famous person in the popular medium presented the perfect press to focus itself on three issues that mostly catches the popular attention, being crime, sport, sex. Besides the enigma: where were the remains of the young woman? Day by day, moment by moment the press followed the steps of the police investigation and criminal procedure in order to pass the news and thus realize their profits, being clear that this coverage favored the stage press inquiries and judgment, always emphasizing the personality of Bruno Fernandes and outside the normal range. The media so, self claimed authority as moralizing, using the narrative to do so. At the end, found itself ratification of a verdict already delivered by the media long before the trial, in view of the penal populism media where Bruno Fernandes was sentenced to reprimand larger than the right would admit, mainly because the trial by jury is made through the people, which confirmed that heard for almost three years.

Made available in DSpace on 2014-08-20T13:25:36Z (GMT). No. of bitstreams: 1 Tese_Marcio_Ramos_Botelho.pdf: 7363575 bytes, checksum: 7093095943e8e185a759d62d75529be8 (MD5) Previous issue date: 2011-02-02 / Dark subsurface horizons with morphological characteristics similar to sombric horizonts have been found in southern Brazil, particularly in the higher areas of the Pelotas Batholith, located in the eastern portion of the Escudo Sul-rio-grandense. The sombric horizon, initially identified by soil scientists who worked in the Belgian Congo, constitutes a diagnostic horizon in soil classification systems of FAO and Soil Taxonomy, but not in the Brazilian system. The conception of sombric horizon in these classification systems is of a free draining subsurface horizon, containing iluvial humus not associated with aluminum or dispersible by sodium, with value and/or lower chroma than the overlying horizon. In the field it can be differentiated from the buried A horizon by monitoring lateral variations and in the laboratory by analyzing thin sections. The mechanisms responsible for the genesis of the sombric horizon are still poorly understood, although most authors agree on the illuvial humus origin and that it constitutes a diagnostic horizon. Considering the need to characterize the occurrence of such horizons in the region and the possible reasons for its genesis this work aimed to describe the occurrence of soil profiles exhibiting dark subsurface horizons with similar morphology to the sombric horizon in the region of Pelotas Batholith and to investigate the organic matter involved in the process. To achieve this goal physical, chemical, morphological, mineralogical and isotopic studies were performed in ten soil profiles with dark subsurface horizons, two of them without the sombric morphological characteristics and another one with morphological evidence of buried A horizon, and two degraded areas. The results showed that: a) the position of the dark subsurface horizons coincide with the A2, AB, BA horizons, top of B horizon or AC/CR; b) the horizons with morphology similar to sombric are not buried horizons, as indicated by particle size analysis, mineralogy, morphology and lateral variations of these horizons in the field, c) the fractionation of humic substances indicated a process of illuviation of organic matter, especially humic acids, for all soil samples, showing that the illuviation of organic material occurs not only in profiles with sombric horizons; d) the absence of the E horizon, associated with the Al + ½ Feo criteria, optical density of oxalate extract, chemical fractionation and significant presence of illuvial subsurface cutans indicate that the podzolization process is only incipient in the soil profiles; d) the C isotopic composition indicate that a part of the dark subsurface horizons have organic matter from C4-type plants (grasslands), differing from to the present. In other profiles, there is a mixture of C3 plants (forests) and C4 plants with little or no variation from the current vegetation, suggesting that the genesis of these horizons is not related only to changes in vegetation; f) a significant part of the soils located on the Pelotas Batholith present are not adequately classified in the Brazilian soil classification system, g) the adoption of the horizon or sombric character and changes in the Argissolos Bruno-Acinzentados suborder in future editions of the Brazilian System of Soil Classification would fit better a significant part of the soil profiles of the study area. / Horizontes subsuperficiais escuros com características morfológicas similares ao sômbrico têm sido encontrados na região Sul do Brasil, particularmente nas áreas mais elevadas do Batólito Pelotas, situado na porção leste do Escudo Sul-rio-grandense. O sômbrico, inicialmente identificado por pedólogos belgas que trabalharam no Congo, constitui um horizonte diagnóstico nos sistemas de classificação de solos da FAO e da Soil Taxonomy, mas não no sistema brasileiro. A concepção de horizonte sômbrico nestes sistemas de classificação é a de um horizonte subsuperficial de drenagem livre, contendo húmus iluvial não associado ao alumínio, nem dispersável pelo sódio, com valor e/ou croma menores do que o horizonte sobrejacente. No campo pode ser diferenciado do horizonte A enterrado pelo acompanhamento das variações laterais ou no laboratório por análise em secções delgadas. Os mecanismos responsáveis pela gênese do horizonte sômbrico são ainda pouco compreendidos, apesar da maioria dos autores concordarem com a origem do húmus iluvial e de constituir horizonte diagnóstico. Diante da necessidade de caracterizar a ocorrência desse tipo de horizonte na região e do entendimento da gênese destes objetivou-se neste trabalho descrever a ocorrência de solos com horizontes de morfologia similar à do sômbrico na região do Batólito Pelotas e investigar o tipo de matéria orgânica envolvido no processo. Para atingir este objetivo foram realizadas análises físicas, químicas, morfológicas, mineralógicas e isotópicas em dez perfis de solos da região com horizontes subsuperficiais escuros, sendo dois deles sem as características morfológicas de sômbrico e outro com indícios morfológicos de horizonte A enterrado, além de duas áreas degradadas. A análise dos resultados permitiu verificar que: a) a posição destes horizontes coincide com os horizontes A2, AB, BA, topo do horizonte B ou ainda AC/CR; (b) os horizontes com morfologia similar a do sômbrico não constituem horizontes enterrados, conforme indicado pela análise granulométrica, mineralogia, morfologia e variações laterais destes horizontes no campo; c) o fracionamento das substâncias húmicas indicou haver um processo de iluviação de matéria orgânica, principalmente de ácidos húmicos, para todos os perfis estudados, mostrando que a iluviação de material orgânico não ocorre somente nos perfis com horizontes ―sômbricos‖; d) a ausência do horizonte E, associado aos critérios de Al + ½ Feo, densidade ótica do extrato de oxalato, fracionamento químico e presença significativa de cutans de iluviação em subsuperfície indicam que o processo de podzolização é apenas incipiente nos perfis; e) a composição isotópica do carbono indica que uma parte dos horizontes subsuperficiais escuros possui matéria orgânica oriunda de plantas do tipo C4 (gramíneas), com variação isotópica em relação à vegetação atual. Em outros, há mistura de plantas C3 (florestas) e C4 com pouca ou nenhuma variação em relação à vegetação atual, sugerindo que a gênese destes horizontes não esteja relacionada somente a mudanças na vegetação; f) parte significativa dos solos do Batólito Pelotas apresenta problemas de enquadramento na atual classificação brasileira de solos; g) a adoção do horizonte ou caráter sômbrico e mudanças na subordem dos Argissolos Bruno-Acinzentados em edições futuras do Sistema Brasileiro de Classificação de Solos enquadraria melhor uma significativa parte dos perfis da área de estudo.

Cette thèse porte sur les livres auto-illustrés réalisés par l'Autrichien Alfred Kubin (1877-1959), le Tchèque Josef Váchal (1884-1969) et le Polonais Bruno Schulz (1892-1942). En rapprochant les œuvres de ces auteurs et illustrateurs relativement méconnus et considérés comme marginaux selon les canons culturels de leur époque, ce travail souhaite démontrer qu'un paradigme se fait jour dans la pratique de l'auto-illustration et l'imaginaire du livre qui lui est associé. En tenant compte du contexte de la culture centre-européenne dans l'entre-deux-guerres et de la dimension technique de l'histoire du livre, dont les enjeux coïncident avec les réflexions contemporaines sur l'originalité et la reproductibilité mécanisée, la thèse définit la démarche d'auto-illustration comme la quête d'un « livre total de pacotille ». Mais il s'agit aussi de lui donner un cadre théorique en se focalisant sur l'analyse des rapports entre le texte et l'image afin de révéler certains traits récurrents qui défient le processus d'illustration classique : l'antériorité ou la simultanéité de l'image, qui permet l'information mutuelle des codes iconique et linguistique sur la page ou la favorise dans le texte, inverse aussi le principe de « transposition » inhérent à l'illustration, voire l'excède pour lui substituer un « dialogisme » intersémiotique. Ces phénomènes confirment l'importance du geste réflexif présidant à l'auto-illustration, qui suppose une hyperconscience du dispositif illustré mais prend aussi en compte l'omniprésence transgressive du créateur, qui envahit les seuils de l’œuvre, se figure en texte et en images, et en auteur, illustrateur, narrateur et personnage(s). / This dissertation deals with self-illustrated books by the Austrian Alfred Kubin (1877-1959), the Czech Josef Váchal (1884-1969) and the Pole Bruno Schulz (1892-1942). Comparing the works of these writers and illustrators, who are not quite famous and were considered to be on the margins of the cultural models of their time, the dissertation shows that a creative paradigm is to be found through self-illustration and the book imagery that goes with it. Taking into account the context of central-european culture during the interwar period, and the technical aspects of book history, which are linked to contemporaneous debates about originality and technical reproductibility, this study defines the production of these writers-ilustrators as a search for a « cheap total bookwork ». It is based on a theoretical framework which focuses on the relations between text and image in order to reveal recurring features which challenge the classical illustrating process : images being produced prior to or at the same time as the text provide for the mutual exchange of properties between words and pictures on the page and in the text itself. This practice also reverses the principle of « transposition » inherent to illustration, or even replaces it with an intersemiotic « dialogism ». These phenomena confirm the importance of the reflexive nature of self-illustration : not only does it require a high level of awareness of the illustrated apparatus but it also explains the transgressive omnipresence of the creator who invades the « paratext » and represents himself in texts and images as writer, illustrator, narrator and character(s).

La dystrophie myotonique de type 1 (DM1) ou maladie de Steinert est la maladie génétique neuromusculaire la plus commune avec une incidence de 1/8000 à travers le monde. Cette maladie multisystémique touche particulièrement les muscles squelettiques (myotonie, faiblesse et perte musculaires) et le coeur qui présente des symptômes variés comme des troubles de la conduction et des arythmies. La DM1 est causée par une expansion instable de répétitions CTG dans la région 3’ non traduite du gène DMPK. Les individus sains possèdent entre 5 et 37 répétitions CTG tandis que les patients DM1 portent entre 50 et plusieurs milliers de répétitions. Il est bien établi que les expansions de répétitions non codantes forment des foci dans les noyaux musculaires où elles séquestrent le facteur d'épissage MBNL1. Toutefois, l'implication de la stabilisation et l'accumulation de CUGBP1 hyperphosphorylé par la PKC dans la maladie est un sujet controversé dans la communauté DM1. Dernièrement, en plus de la rupture de l'équilibre entre MBNL1/CUGBP1, plusieurs mécanismes ont été mis en cause dans la pathogenèse de la DM1. Parmi eux, l'expression perturbée de facteurs de transcription, la maturation altérée de miARNs, l'activation de kinases... chacune de ces altérations menant au final à une perturbation du transcriptome. Afin d'étudier l'effet de la toxicité des répétitions sur les phénotypes et lestranscriptomes, nous avons généré trois lignées de Drosophile inductibles et site-spécifiques exprimant 240, 600 et 960 répétitions de triplets. Nous avons travaillé en parallèle sur une lignée atténuée pour mbl (orthologue de MBNL1) et deux lignées gain de fonction bru -3 (orthologue de CUGBP1). Exprimées dans les muscles somatiques, les répétitions CTG conduisent à une mobilité réduite, le fractionnement des fibres musculaires, une réduction de leur taille et une altération du processus de fusion des myoblastes de manière dépendante de Mbl et Bru-3. En outre, l'expression des répétitions cause une hypercontraction musculaire dépendante de Mbl et due à un mauvais épissage de dSERCA. L'analyse transcriptionnelle comparative réalisée sur les muscles larvaires des différentes conditions pathologiques montre que l'atténuation de mbl reproduit 70-82% des dérégulations transcriptomiques des larves DM1 alors que le gain de fonction bru-3 représente 32-53% des altérations transcriptomiques des lignées DM1. Ainsi Mbl est un facteur clé des dérégulations observées dans les muscles somatiques des lignées DM1. Au contraire, les analyses physiologiques effectuées sur les coeurs adultes suggèrent que Bru-3 est un facteur clé dans la mise en place des phénotypes cardiaques. En effet, d'une part, l'atténuation de mbl dans le coeur cause une cardiomyopathie dilatée, un symptôme rarement diagnostiqué chez les patients. D'autre part, les lignées gain de fonction bru-3 et DM1 présentent de la fibrillation qui évolue avec l'âge ou la taille des répétitions vers un phénotype qui rappelle l'insuffisance cardiaque chez les patients. / Myotonic Dystrophy Type 1 (DM1) or Steinert's disease is the most common genetic neuromuscular disorder affecting 1 out of 8000 people worldwide. This multisystemic disease affects particularly the skeletal muscles (myotonia, muscle weakness and wasting) and the heart, which can exhibit various symptoms like conduction disturbances and arrhythmia (auricular fibrillation and flutter). DM1 is caused by an unstable CTG repeat expansion in the 3' non-translated region of the DMPK gene. In healthy individuals, the number of CTG repeats ranges from 5 to 37 whereas DM1 patients carry from 50 to thousands repeats. It is well established that when expanded non-coding repeats aggregate into foci within muscle nuclei and sequester the MBNL1 splicing factor. However, the involvement of the stabilization and accumulation of CUGBP1 following PKC hyper-phosphorylation in the disease is a controversial matter in the DM1 community. Lately, in addition to the disruption of the balance between MBNL1/CUGBP1, several mechanisms were identified as part of the DM1 pathogenesis. Among them, transcription factors perturbations, altered maturation of miRNA, kinases activation… each of them leading eventually to transcriptomic alterations. In order to investigate the effect of toxic repeat expression on phenotypic and transcriptomic alterations, we generated three inducible site-specific Drosophila lines expressing 240, 600 and 960 triplet repeats. We worked in parallel on a mbl (MBNL1 orthologue) knocked-down line and two bru-3 (CUGBP1 orthologue) gain of function lines. When expressed in somatic muscles, CTG repeats lead to altered motility, fiber splitting, reduced fiber size and affected myoblast fusion process in a Mbl and Bru-3 dependent manner. In addition, toxic repeats cause fiber hyper-contraction in a Mbldependentmanner due to dSERCA mis-splicing. Comparative transcriptional profiling performed on larval muscles of different conditions show that mbl attenuation reproduces 70-82% of DM1 transcriptomic deregulations whereas bru-3 gain of function represents 32-53% of transcritomic alterations. Thus Mbl appears as a key factor of transcripts deregulations observed in DM1 muscles. On the contrary, physiologic analyses performed on adult hearts suggest that Bru-3 is a key factor for cardiac phenotypes. Indeed, on one hand, mbl attenuated flies display dilated cardiomyopathy, a symptom barely diagnosed in patients. On the other hand, bru-3 gain of function line and DM1 lines display fibrillation, which evolves withage or repeat size into a phenotype reminiscent of heart insufficiency in patients.

Cette recherche se constitue comme un parcours généalogique dans la pensée naturelle et politique de Giordano Bruno autour de la notion de « vinculum » ‘lien’ ou ‘relation’, à travers la reconstruction de son origine et de son élaboration à partir des écrits magiques jusqu’aux dialogues italiens et aux premiers ouvrages latins. L’exigence est de retracer dans l’œuvre ainsi que dans l’expérience intellectuelle et biographique du Nolain, quelle est la relation entre philosophie naturelle et politique et comment elle se constitue. En d’autres termes, il s’agit de comprendre si l’analyse du politique pourrait être déliée ou réellement distinguée de l’étude de la nature ou si elle prendrait plutôt son point d’origine et se développerait au cœur de la connaissance de la physis : isoler et analyser quel que soit le rôle et la fonction que la notion de « vinculum » assume entre la réflexion métaphysique et anthropologique de Bruno. Ces questions constituent le problème de fond qui anime ce travail et auquel j’ai essayé de répondre dans ces pages, bien que conscient du caractère jamais définitif et ouvert de cette réponse comme pour chaque recherche. / This research is a genealogic study of the natural and political thought of Giordano Bruno, about the notion of «vinculum», ‘bond’ or ‘relation’, by the reconstruction of its origin and development from the magic writing to the Italians dialogues and the firsts Latins works The necessity of this work is to track down the Bruno’s opera and in his intellectual and biographic experience which is the relation between natural and political philosophy. In others words, the work lies in understanding if is possible to separate and distinguish the analysis of the politic from the nature; or the political thought is originate and it is inseparable from the reflection about the physis: isolating and analyzing which is the role and the function of the notion of «vinculum» between the metaphysical and anthropological reflection of Giordano Bruno. Those questions constitute the problem of this research and to which I try to respond in theses pages, even if conscious of the character never complete of this answer, as well as every research.

L’évocation de la Renaissance italienne des XVe et XVIe siècles est souvent synonyme de la propagation d’une nouvelle pensée de l’homme, exaltant les valeurs oubliées de l’excellence et de la liberté humaines. Chez un philosophe comme Giordano Bruno (1548-1600), la question de la liberté ne se présente pas aussi facilement que chez d’autres grands auteurs des Quattrocento et Cinquecento (tel Marsile Ficin ou Pic de la Mirandole). Sa défiance héroïque envers l’autorité ecclésiastique et son exécution par l’Inquisition, le 17 février 1600, sur le campo dei Fiori, illustrent sa longue lutte pour libérer la philosophie des entraves de la religion révélée. Bruno peut se targuer d’être l’un des premiers penseurs depuis l’Antiquité à intégrer une cosmologie, une physique, une psychologie et une éthique dans un système de philosophie (la nova filosofia). Malgré une terminologie parfois fluctuante et des contradictions souvent apparentes, la philosophie de Bruno possède une réelle cohérence interne et peut être regardée comme annonçant celle de Spinoza. Or à la différence du déterminisme de ce dernier, Bruno soutient que l’homme est doté d’un libre arbitre, s’opposant en cela aux thèses de Luther et abondant dans le sens d’Erasme. Son affirmation d’une liberté humaine intimement liée aux problèmes éthiques et religieux de son époque n’est toutefois pas sans provoquer certaines tensions au regard de sa conception métaphysique d’un univers infini en acte. L’objet de ce travail est d’analyser la thèse brunienne de la compossibilité de la liberté humaine avec la nécessité divine qui s’exprime dans un univers métamorphique et infini, en recherchant, dans une première partie, les sources de son compatibilisme et en interprétant, dans une seconde partie, la manière dont Bruno concilie liberté et nécessité. / The evocation of the Italian Renaissance of the fifteenth and sixteenth centuries is often synonymous with the spread of a new human thought, exalting the forgotten values of human excellence and freedom. For a philosopher like Giordano Bruno (1548-1600), the problem of freedom does not arise as easily as it does for other great authors of Quattrocento and Cinquecento (such as Marsilio Ficino or Pico della Mirandola). His heroic defiance of ecclesiastical authority and his execution by the Inquisition on 17 February 1600 onto the Campo de’ Fiori, exemplifies his long struggle to free philosophy from the trammels of revealed religion. Bruno can claim to be the first thinker since Antiquity to integrate a cosmology, physics, ethics and psychology into a system of philosophy (nova filosofia). Despite sometimes inconsistent terminology and often apparent contradictions, Bruno’s philosophy has a real inner coherence and can be seen as announcing Spinoza’s. However, unlike the latter’s determinism, Bruno maintains that human being is endowed with a free will, opposing Luther’s theses and agreeing with Erasmus. Nonetheless, his affirmation of human freedom, intimately linked to the ethical and religious problems of his time, is not without causing tensions with regard to his metaphysical conception of the actual infinity in the universe. The purpose of this work is to analyse the brunian thesis of the compossibility of human freedom with the divine necessity expressed in a metamorphic and infinite universe, by seeking, in a first part, the sources of its compatibility and by interpreting, in a second part, the way in which Bruno reconciles liberty and necessity.