A Síndrome de Marfan (SMF) é uma doença de tecido conjuntivo, com caráter autossômico dominante, que acomete cerca de 1 em 5.000 indivíduos. As principais manifestações clínicas incluem aneurismas e rompimento da aorta, crescimento excessivo dos ossos, escoliose e deformidades torácicas. Mutações no gene FBN1, que codifica a proteína de matriz extracelular fibrilina-1, foram relacionadas à doença, fazendo com que essa fosse classificada no grupo das fibrilinopatias. Mais de 500 mutações já foram identificadas e, com exceção de um pequeno grupo de mutações recorrentes, as mutações são únicas, sendo encontradas em famílias isoladas. A doença caracteriza-se pela grande variabilidade tanto intra quanto interfamilial, não sendo possível fazer uma correlação precisa entre genótipo e fenótipo. Este trabalho visa discutir os mecanismos responsáveis pela variabilidade clínica inter e intra familiar da SMF através da caracterização qualitativa e quantitativa da variabilidade fenotípica observada no modelo murino para SMF MGΔloxPneo . Neste sentido, caracterizamos o modelo mgΔloxPneo em duas linhagens de camundongos diferentes, C57BL/6 e 129/Sv. Os animais mutantes de ambas as linhagens apresentaram deficiência na deposição de microfibrilas, cifose de coluna, enfisema pulmonar e degeneração da parede aórtica. Contudo, a idade de início dos sinais fenotípicos mostrou-se mais tardia em animais da linhagem C57BL/6 em comparação com os animais 129/Sv, indicando a presença de genes modificadores entre as duas linhagens. Além disso, caracterizamos uma grande variabilidade fenotípica entre os animais 129/Sv mutantes, o que é sugestivo do envolvimento de fatores epigenéticos na gravidade da doença. Finalmente, demonstramos uma forte correlação negativa entre os níveis globais de transcrição do gene Fbn1 e a gravidade do fenótipo. Esses resultados corroboram a hipótese de que o nível de expressão da proteína normal está relacionado com a gravidade do quadro clínico da SMF em humanos. Com base nisso, o trabalho também visa o estudo de novas estratégias terapêuticas para a SMF nesse mesmo modelo. / The Marfan syndrome (MFS) is an autosomal dominant disease of connective tissue, which affects 1 in 5,000 individuals. The main clinical manifestations include aneurysms and aortic disruption, excessive growth of bones, scoliosis and thoracic deformities. Mutations in the FBN1 gene, which encodes the fibrillin-1 protein, were genetically linked to the MFS, classifying this disease in the fibrilinopathies group. Over 500 mutations have been identified and, except for a small group of recurrent mutations, the mutations are unique, being found in unrelated families. The disease is characterized by a wide clinical variability both within and between families, and it is not possible to make a precise genotypephenotype correlation. This work concerns the analysis of the mechanisms associated with the clinical variability present within and between MFS families, by qualitative and quantitative characterization of the phenotypic variability observed in the mgΔloxPneo model for MFS. We characterize the model mgΔloxPneo, in two different mouse strains, the C57BL/6 and the 129/Sv strain. Mutant animals from both strains present defective microfibrillar deposition, emphysema, deterioration of aortic wall and kyphosis. However, the onset of a clinical phenotypes is earlier in the 129/Sv than in C57BL/6 background, indicating the existence of genetic modifiers of MFS between these two mouse strains. In addition, we characterized a wide clinical variability within the 129/Sv heterozygotes, suggesting involvement of epigenetic factors in disease severity. Finally, we show a strong negative correlation between overall levels of Fbn1 expression and the severity of the phenotypes. These results corroborated with studies, using animal models, as well with MFS patients, where the levels of normal fibrillin-1 seem to have the potential to modulate the clinical severity of the disease. In addition, the study also aims to evaluate new treatment possibilities for MFS in this same model.

Kepler is mainly known among historians of science due to his astronomical theories and his approaches to problems having to do with philosophy of science and ontology. This thesis attempts to contribute to Kepler studies by providing a comprehensive discussion of a topic hitherto not really considered, namely Kepler’s theory of the soul, a general theory of knowledge or epistemology whose central problem is what makes knowledge possible—rather than what makes knowledge true, as happens in the case of Descartes’s and Bacon’s epistemologies. Kepler’s theory consists of four issues: the theory of the different sorts of soul—i.e. the human soul, the animal soul, the vegetable soul, and the Earth soul—concerning their faculties, the differences and the resemblances that emerge among them, the relation they maintain with their own bodies and the world, and the distinction soul-world. The thesis discusses these issues from a historical perspective, that is, it reconstructs the way they appear in three periods of Kepler’s career: the period prior to the publication of the Mysterium Cosmographicum, the period going from 1596 to 1611, and the harmonic period. Finally, Kepler’s epistemology is briefly contrasted with Descartes’s and Bacon’s in order to suggest why Kepler’s is philosophically interesting and valuable. / October 2006

In a famous argument at the beginning of the Nicomachean Ethics, Aristotle argues that the function and good of the human being is the "actuality of the soul in accordance with virtue". Presenting a view critical of the widespread intellectualist reading of Aristotle's Ethics, in this thesis I argue that the characteristic function of the human being is constitutive of a distinctly human life as a dynamic formal cause teleologically operative in human awareness. I argue for the validity of my own view in a preliminary way in the introduction by way of Aristotle's critique of the Platonic forms. In the second chapter, I argue that the processes of the non-rational part of the soul are acquired and actively operate once acquired independently of singular dictates of active reason within the individual. By this I mean that the virtues do not obey reason in the sense that they receive individual commands from discursive reason to desire or feel in certain ways. Rather, although the moral virtues are formed gradually by repeated acts of choice, as affective states, they are activated by being affected from without by external stimuli. These external stimuli produce impulses in the soul which are conducive to virtuous action, including a cognitive element: primarily, non-rational and non-discursive evaluative judgments of phantasia, which supply a human agent immediately with the ends of his action and the beginning-points of deliberation. These judgments are the awareness of sensible particulars as pleasant. In the third chapter, I turn to the De Anima in order to illuminate the cognitive conditions of human praxis. Following on the arguments contained in the second chapter, I argue that there are two primary cognitive moments which are necessary conditions of action. While the ends of desire are immediate objects of awareness and move humans as unmoved movers, motivational desires, which move as efficient causes, are initiated by a distinct cognitive power: proclamations to pursue or avoid.

Kepler is mainly known among historians of science due to his astronomical theories and his approaches to problems having to do with philosophy of science and ontology. This thesis attempts to contribute to Kepler studies by providing a comprehensive discussion of a topic hitherto not really considered, namely Kepler’s theory of the soul, a general theory of knowledge or epistemology whose central problem is what makes knowledge possible—rather than what makes knowledge true, as happens in the case of Descartes’s and Bacon’s epistemologies. Kepler’s theory consists of four issues: the theory of the different sorts of soul—i.e. the human soul, the animal soul, the vegetable soul, and the Earth soul—concerning their faculties, the differences and the resemblances that emerge among them, the relation they maintain with their own bodies and the world, and the distinction soul-world. The thesis discusses these issues from a historical perspective, that is, it reconstructs the way they appear in three periods of Kepler’s career: the period prior to the publication of the Mysterium Cosmographicum, the period going from 1596 to 1611, and the harmonic period. Finally, Kepler’s epistemology is briefly contrasted with Descartes’s and Bacon’s in order to suggest why Kepler’s is philosophically interesting and valuable.

Kepler is mainly known among historians of science due to his astronomical theories and his approaches to problems having to do with philosophy of science and ontology. This thesis attempts to contribute to Kepler studies by providing a comprehensive discussion of a topic hitherto not really considered, namely Kepler’s theory of the soul, a general theory of knowledge or epistemology whose central problem is what makes knowledge possible—rather than what makes knowledge true, as happens in the case of Descartes’s and Bacon’s epistemologies. Kepler’s theory consists of four issues: the theory of the different sorts of soul—i.e. the human soul, the animal soul, the vegetable soul, and the Earth soul—concerning their faculties, the differences and the resemblances that emerge among them, the relation they maintain with their own bodies and the world, and the distinction soul-world. The thesis discusses these issues from a historical perspective, that is, it reconstructs the way they appear in three periods of Kepler’s career: the period prior to the publication of the Mysterium Cosmographicum, the period going from 1596 to 1611, and the harmonic period. Finally, Kepler’s epistemology is briefly contrasted with Descartes’s and Bacon’s in order to suggest why Kepler’s is philosophically interesting and valuable.

Made available in DSpace on 2014-12-17T14:20:32Z (GMT). No. of bitstreams: 1 CristinaSF_TESE.pdf: 2605520 bytes, checksum: d9ce69b5b8d10f4d603e310b65d8a8c8 (MD5) Previous issue date: 2014-05-05 / Coordena??o de Aperfei?oamento de Pessoal de N?vel Superior / No contexto de p?s-independ?ncia dos pa?ses da regi?o norte ocidental do continente africano e do posicionamento cr?tico e discursivo dos sujeitos frente ?s imagens euroc?ntricas que culminou com a constitui??o das cinematografias africanas, destaca-se a obra do realizador Moustapha Alassane. Com uma pr?tica voltada para uma concep??o particular das imagens em movimento, o autor nigerino reconstr?i o trajeto do encanto pela imagem ? magia do movimento, criando sua lanterna m?gica, animando figuras e impress?es de seu cotidiano, a partir do simples tra?o do desenho ? modelagem e cria??o de marionetes tridimensionais. Um discurso que se faz gesto pela bricolagem de elementos que est?o a seu alcance. E o gesto ir?nico do realizador transforma o cen?rio de um vilarejo no interior do N?ger em um filme de faroeste, uma oportunidade para se apropriar do mito que ? associado ao da cria??o do cinema no Ocidente. Este trabalho toma como refer?ncia os estudos de autores da di?spora, da cr?tica p?s-colonial e de te?ricos africanos. Dessa forma analiso a obra cinematogr?fica e a trajet?ria f?lmica do realizador nigerino pela via de acesso da chegada das cinematografias africanas ao Brasil, levando em conta o car?ter transnacional dos processos sociais contempor?neos, (re)aproximando-nos do continente africano e da reflex?o de combate ao racismo como um sistema-mundo

Submitted by Automa??o e Estat?stica (sst@bczm.ufrn.br) on 2018-03-12T20:00:44Z No. of bitstreams: 1 JoanaVieiraViana_DISSERT.pdf: 5476101 bytes, checksum: 49c78720e218dc3dcc01a5f43e59b371 (MD5) / Approved for entry into archive by Arlan Eloi Leite Silva (eloihistoriador@yahoo.com.br) on 2018-03-16T18:09:10Z (GMT) No. of bitstreams: 1 JoanaVieiraViana_DISSERT.pdf: 5476101 bytes, checksum: 49c78720e218dc3dcc01a5f43e59b371 (MD5) / Made available in DSpace on 2018-03-16T18:09:10Z (GMT). No. of bitstreams: 1 JoanaVieiraViana_DISSERT.pdf: 5476101 bytes, checksum: 49c78720e218dc3dcc01a5f43e59b371 (MD5) Previous issue date: 2016-08-31 / A presente pesquisa tem como objetivo investigar a utiliza??o das t?cnicas de anima??o como linguagem na forma??o do professor de teatro e nas atividades em sala de aula do Ensino Fundamental 1. Tem como refer?ncia as experi?ncias vivenciadas na disciplina Teatro de Formas Animadas, na UFRN, no semestre de 2014.1, ministrada pelo Professor Dr. S?vio Ara?jo, na qual a pesquisadora realizou doc?ncia assistida e o relato e analise, a partir da sua pr?tica docente junto ?s turmas do Ensino Fundamental 1, da Escola Municipal Jos? Peregrino de Carvalho, em Jo?o Pessoa, PB, nos anos de 2015 e 2016. Observa-se nos dois espa?os, seja para alunos de gradua??o como para crian?as, que s?o enfrentados desafios referentes ao planejamento e sele??o de conte?dos, estrat?gias de motiva??o das turmas e melhor forma de fazer com que os alunos tenham autonomia e discernimento para desenvolverem seus potenciais art?sticos e pessoais. Com rela??o ? forma??o dos professores, ao definir conceitualmente e ao se estabelecer escolhas terminol?gicas e metodol?gicas, relacionadas ao que se entende por teatro de anima??o, ator animador, objeto, animar, e, principalmente, a partir da rela??o do di?logo estabelecida entre o ator, o animador e o objeto, delineia-se qual caminho percorrer no vasto universo do teatro de anima??o. Ressaltando-se as reflex?es sobre a cria??o art?stica como parte fundamental no processo de ensino-aprendizagem e sua rela??o com um melhor aproveitamento por parte dos alunos. Da mesma forma, com rela??o ao trabalho desenvolvido no ensino fundamental 1, a cria??o de cenas configura-se como uma potente estrat?gia metodol?gica na constru??o do conhecimento, embora uma s?rie de desafios sejam enfrentados diariamente no ambiente escolar. / This study aims investigate the utilization of theatre animation techniques as language in the drama teacher formation and in the elementary classroom activities. For this purpose, two experiences are taken as references. The first one is based in the internship as teacher assistant in the Dr. Savio Araujo?s classes of Theatre of Animated Forms in the first semester of 2014 in the Federal University of Rio Grande do Norte. The second is derivate of the researcher's work as teacher of arts in the Municipal School Jos? Peregrino de Carvalho of elementary level, in the city Jo?o Pessoa, PB, during the years of 2015 and 2016. In the both cases, as for undergraduate students as elementary school children, it is observed that there are challenges related to the planning and selection of contents, to the strategies to motivate the classes and the best way to make the students have autonomy and discretion to develop their artistic and personal potential. Regarding the formation of the teacher, the way to go through the vast universe of animation theatre is lineated by the conceptual definitions and terminological and methodological choices related of the assumed meaning for animation theatre, animator actor, object, give life, and mainly from the relationship of the dialogue established between the actor, the animator and the object. They are highlighted reflections about the artistic creation as fundamental part in the teaching-apprenticeship process and its relationship with the improvement by the students. Similarly, in relation to work in primary school 1, scenes creation is configured as a powerful methodological strategy in the construction of knowledge, although a number of challenges are faced daily in the school locus.

This thesis asks for the role of signs in Aristotle's theory of knowledge. The great part of semiotic literature is restricted on the interpretation of the works De Interpretatione, which explains the theory of symbols, and Analytica Priora, which explains the theory of signs. In Aristotle's words, theory of signs is based on the theory of knowledge submitted in De Anima. However, semioticians are not able to define the exact part of this work which is being linked, therefore they only neglect this link. The aim of this thesis is to find how much the conception of knowledge submitted in De Anima works or does not work with the notion of representation, and which role Aristotle's notions of sign (sémeion) and symbol (symbolon) take in that issue. Keywords: Aristotle, De Interpretatione, Analytica Priora, De Anima, history of semiotics, knowledge, signs.

El propósito de esta tesina es analizar la repercusión del arquetipo de la madre como elemento esencial en el personaje de Pedro Páramo, en la obra Pedro Páramo, publicada en 1955, por el escritor mexicano Juan Rulfo. Para realizar este estudio, se ha elegido la teoría psicoanalítica de Carl Gustav Jung como base con la que estudiaremos dicho personaje. Esta teoría será utilizada también como herramienta de análisis y como prisma, a través del cual analizaremos los arquetipos que la figura de la madre representa: los mismos que Pedro Páramo buscaba en Susana San Juan. Por otro lado, miraremos más de cerca los elementos femeninos de la naturaleza, la tierra, analizándolos desde la perspectiva femenina.A través de nuestro análisis hemos podido comprobar la importancia del arquetipo de la madre y el anima en el desarrollo del personaje de Pedro Páramo, sobre quien recae la incompleta imagen del arquetipo de la madre como factor determinante de su conducta. Por último hemos llegado a entender la desilusión de Pedro Páramo, al vengarse de Comala, cuando se da cuenta de que fracasa en la integración con su anima.

Dans la question 10 du livre II de son commentaire au traité De l’âme d’Aristote, Nicole Oresme soulève la question suivante : est-ce que le sens se trompe relativement à son objet propre? S’attaquant à la problématique de l’illusion sensible, il soutient qu’il y a effectivement place pour l’erreur dans la connaissance sensible puisque les facultés sensitives de l’âme ont la capacité de former des jugements. Influencé par le modèle perspectiviste de la vision, Oresme met l’accent sur le rôle des sens internes, plutôt que les sens externes, dans la perception. Ce mémoire de maîtrise a pour but d’élucider les notions de « concept » (conceptus), « jugement » (iudicium) et « discours » (discursus) telles qu’Oresme les emploie dans le deuxième livre de ses Quaestiones De anima et est accompagné d’une traduction des questions I.4, II.8-15 et II.21 des Quaestiones De anima. / In question 10 of book II of his commentary on Aristotle’s De anima, Nicole Oresme asks whether the senses can be deceived about their proper objects. Tackling the issue of sense illusion, he maintains that there is indeed error in sense cognition since the sensitive faculties of the soul can form judgments. Influenced by the perspectivist account of vision, Oresme highlights the role of the internal senses, rather than the external senses, in perception. This master’s thesis aims to elucidate the notions of « concept » (conceptus), « judgment » (iudicium) and « discourse » (discursus) as Oresme uses them in book II of his Quaestiones De anima and includes a French translation of questions I.4, II.8-15 and II.21 of the Quaestiones De anima.