A Doença Pulmonar Obstrutiva Crônica (DPOC) é caracterizada pela limitação persistente de trocas gasosas, usualmente progressiva e associada a uma resposta inflamatória crônica exacerbada das vias aéreas a partículas e gases nocivos. Apesar de prevenível e tratável, não se logrou até o presente uma terapêutica eficaz, que resulte na cura da doença. Neste cenário, a terapia celular apresenta-se como uma alternativa terapêutica potencialmente promissora em DPOC, bem como em outras doenças pulmonares degenerativas e de caráter inflamatório. Porém, vários aspectos da terapia celular carecem de um melhor entendimento. Um dos principais desafios ao sucesso da terapia celular são as baixas taxas de sobrevivência das células transplantadas. O Inibidor do Ativador de Plasminogênio 1 (Plasminogen Activator Inhibitor 1 - PAI-1) pode representar um potencial mediador da sobrevivência de células estromais mesenquimais (CTM) pós-transplante, pois tem sido proposto que anticorpos neutralizadores do PAI-1 auxiliam no aumento da sobrevivência de CTM no tecido-alvo da terapia celular. Desta forma, a diminuição dos níveis de PAI-1 possui um potencial terapêutico interessante, ao modular os principais processos envolvidos na criação de um ambiente pouco propício ao \"homing\" celular durante o processo de injúria. A diminuição dos níveis de PAI-1 é promovida, pela sinvastatina, fármaco da família das estatinas. Desta forma, objetivou-se com este trabalho analisar os efeitos da sinvastatina sobre a expressão do PAI-1, bem como sua influência na sobrevivência das células infundidas para terapia celular de enfisema pulmonar em modelo murino. Camundongos da linhagem FVB foram submetidos à instilação intranasal de elastase para indução de enfisema pulmonar e, posteriormente, tratados com CTM do tecido adiposo e sinvastatina. Os resultados mostraram que, quanto aos aspectos morfológicos e funcionais, considerando-se a análise conjunta de ambos os pulmões, não houve diferença estatisticamente significativa entre os grupos submetidos à instilação intranasal de elastase e submetidos à terapia celular com CTM tratados ou não com sinvastatina. Quando porém os pulmões foram analisados individualmente constatou-se que não houve diferença estatisticamente significativa entre os grupos controle e os resultados referentes ao lado direito do pulmão dos animais tratados com elastase e que receberam sinvastatina e infusão de CTM. Diferenças anatômicas entre os lados direito e esquerdo do pulmão, levaram a uma maior deposição de células no lado direito, como evidenciado pelos resultados obtidos nos ensaios de bioluminescência. Pode-se, portanto, inferir que a recuperação morfológica no lado direito do pulmão de animais com DPOC/enfisema poderia ser decorrente de um efeito regenerativo parácrino das CTM associadas à sinvastatina. / Chronic Obstructive Pulmonary Disease - COPD is characterized by the persistent limitation of gas exchange, is usually progressive, and associated to a chronic augmented inflammatory response of the airways to particles and noxious gases. Despite preventable e treatable, an effective, curative therapeutic approach is yet to be achieved. In this context, cell therapy presents itself as a promising therapeutic approach for COPD and other pulmonary inflammatory and degenerative diseases. However, many aspects of cell therapy with stem cells remain unclear. One of the major challenges to the success of cell therapy are the low survival rates of transplanted cells. The Plasminogen Activator Inhibitor 1 (PAI-1) is a potential mediator of the survival of mesenchymal stromal cells (MSC) after transplantation, since PAI-1 neutralizing antibodies have been shown to increase the survival rate of MSC in the target tissue of cell therapy. Thus, the decreased levels of PAI-1 has an interesting therapeutic potential to modulate key processes involved in creating an inhospitable environment, during the process of injury, to the homing of transplanted cells. Decreased levels of PAI-1 are promoted by simvastatin, a drug of the statins family. Thus, the goal of this work was to evaluate the effect of simvastatin in vivo on the expression of PAI-1, as well as its influence on the survival rate of infused cells in mice model of cell therapy for pulmonary COPD/emphysema. FVB mice were submitted pulmonary emphysema induction by means of intranasal instillation of elastase and than treated with adipose-derived mesenchymal stem cells and simvastatin. The results regarding morphological and functional aspects, when considering the analisys of both lungs, presented no statistically significant difference among the groups submitted to intranasal instillation of elastase and cell therapy with MSC, treated or not with simvastatin. However, when the lungs where analyzed individually, it was found that there was no statistically significant difference between the control group and the results regarding the right lung of animals treated with elastase and that received simvastatin and MSC infusion. Anatomical differences between the right and left sides of the lung lead to a higher deposition of cells in the right side, as observed in the bioluminescence assays. Thus, it is possible to infer that the morphological recovery in the right side of the lung of animals with DPOC/emphysema could be due to a regenerative paracrine effect of mesenchymal stem cells associated with simvastatin.

Les dysplasies frontonasales (DFN) sont un groupe de malformations rares de la face résultant d’une anomalie de développement du processus frontonasal, et se manifestant cliniquement par l’association variable d’une fente faciale médiane, d’un hypertélorisme, et d’anomalies nasales. Elles s’intègrent généralement dans un cadre syndromique, la plupart étant peu spécifiques et non caractérisées. Une douzaine d’entités ont été décrites ; les bases moléculaires sont connues pour sept d’entre elles seulement. Pour les autres entités, les hypothèses initiales étaient celles de pathologies autosomiques dominantes liées à des mutations de novo.Ce travail s’est fondé sur une cohorte de 80 patients présentant une DFN, recrutés au niveau national et international, avec l’objectif d’identifier les bases moléculaires de plusieurs entités de DFN. Une caractérisation phénotypique a d’abord été effectuée. Deux stratégies moléculaires ont ensuite été poursuivies en parallèle : une approche « phenotype-first », visant à étudier les patients classés par cohortes homogènes cliniquement, et une approche « genotype-first », visant à réaliser des études moléculaires chez des patients présentant un tableau aspécifique.Lorsque le diagnostic était celui d’un syndrome dont le gène causal était connu, nous avons réalisé un séquençage ciblé dudit gène (EFNB1, ZSWIM6). Nous avons effectué un séquençage haut-débit d’exome (SHD-E) chez 11 patients présentant un syndrome de Pai (5 en trio, 5 en solo, un en profondeur sur tissu atteint en paire), trois patients avec syndrome oculoauriculofrontonasal (en trio), et respectivement un patient avec syndrome oculocérébrocutané et syndrome de Teebi (en trio). Nous avons réalisé un séquençage haut-débit de génome (SHD-G) chez 3 patients avec un syndrome de Pai, ainsi qu’une patiente avec syndrome oculoauriculofrontonasal. Enfin, nous avons séquencé les gènes ALX1, ALX3 et ALX4 chez 13 individus avec DFN aspécifique ; réalisé un SHD-E en profondeur chez une patiente avec DFN et hypomélanose d’Ito, ainsi que trois SHD-E en trio chez des patients avec DFN aspécifique ou non classée.Nous avons réalisé un travail de caractérisation phénotypique des patients de la cohorte, décrivant les diagnostics différentiels principaux et les chevauchements phénotypiques. Nous avons décrit une nouvelle entité de DFN, identifiée chez 4 de nos patients, sans base moléculaire à l’heure actuelle. Nous avons confirmé les diagnostics cliniques lorsque le gène causal était connu, chez 5 patients. Nous avons retrouvé des variants candidats dans deux gènes de la voie du TGFβ chez quatre patients avec syndrome de Pai : un de novo dans le gène TGFBRAP1, deux de novo et un hérité d’un parent asymptomatique dans le gène PCSK7. Nous avons identifié les mutations dans TFE3 comme étant à l’origine d’un syndrome neurocutané de mosaïcisme pigmentaire chez une patiente et 6 patients additionnels. Enfin, nous avons identifié un variant dans le gène POLR2A chez un fœtus avec DFN aspécifique. Au total, 34 individus avec un syndrome connu et 34 avec une DFN aspécifique restent à l’heure actuelle sans piste moléculaire identifiée.Au total, ce travail a permis de démontrer l’intérêt d’une meilleure connaissance clinique de ces syndromes rares, pour le diagnostic et le conseil génétique. Il a permis l’individualisation et la description de deux syndromes pouvant s’accompagner d’une DFN – dont un à l’échelle moléculaire –, et la démonstration du rôle du gène TFE3 dans le développement. Enfin, des hypothèses sont émises concernant les résultats incertains et négatifs : celles d’un oligogénisme, d’une anomalie épigénétique, d’une mutation post-zygotique ou de l’influence de l’environnement. / Frontonasal dysplasias (FND) are a group of rare facial malformations due to an abnormal development of the frontonasal process, clinically resulting in the variable association of median facial cleft, hypertelorism and nasal anomalies. Most of them are syndromic, but non-specific and not characterized. A dozen entities have been described; molecular bases are known for only seven of them. Regarding the other entities, the hypothesis of dominant disorders due to de novo mutations had been raised.This work was based on a cohort of 80 patients presenting with FND, nationally and internationaly recruited with the goal of identifying molecular bases of FND entities. We first phenotypically characterized the individuals. Then two molecular strategies were performed in parallel: a “phenotype-first” approach, aiming to study clinically homogeneous cohorts of patients, and a “genotype-first” approach, aiming to perform molecular studies on patients with an aspecific phenotype.When the causative gene for the disorder was known, we performed targeted sequencing of the gene (EFNB1, ZSWIM6). We performed whole-exome sequencing (WES) in 11 patients presenting with Pai syndrome (5 trio WES, 5 solo WES, one deep-sequencing pair-WES on affected tissue), 3 individuals presenting with oculoauriculofrontonasal syndrome (OAFNS) (trio WES), and respectively one patient with oculocerebrocutaneous syndrome and Teebi syndrome (both trio WES). We performed whole-genome sequencing (WGS) on 3 patients with Pai syndrome and one patient with OAFNS. Finally, we performed ALX1, ALX3 and ALX4 genes sequencing in 13 individuals with aspecific FND; deep-WES in one patient with FND and Ito hypomelanosis; as well as 3 trio WES in individuals with aspecific or non characterized FND.We achieved a phenotypic characterization of the patients from the cohort, describing major differential diagnosis and clinical overlaps. We described a new FND entity, identified in 4 individuals, with no molecular basis so far. We confirmed the clinical diagnosis when the causative gene was known, for 5 patients. We identified candidate variants in two genes from the TGFβ pathway in four patients with Pai syndrome: one de novo variant in the TGFBRAP1 gene, two de novo and one inherited from an asymptomatic parent in the PCSK7 gene. We identified mutations in TFE3 as causative for a neurocutaneous syndrome of pigmentary mosaicism in a female patient and six additional individuals. Finally, we identified a variant in the POLR2A gene in a fetus with aspecific FND. A total of 34 individuals with a known syndrome and 34 with an aspecific FND still have no identified molecular basis so far.In conclusion, this work allowed proving the importance of a better clinical knowledge of these rare disorders, in terms of diagnosis and genetics counseling. It allowed the delineation of two syndromes with FND – one at a molecular level –, and the demonstration of a role for TFE3 in development. Finally, four hypotheses are raised regarding the negative or uncertain results: oligogenism, epigenetic anomaly, post-zygotic mutation or environmental influence.

Ce travail de thèse a consisté à améliorer certaines propriétés de revêtements fonctionnels à base de polychlorure de vinyle (PVC) plastifié et de polyamide-imide (PAI) par incorporation de nanocharges inorganiques préformées, lamellaires ou divisées. La compatibilisation des nanocharges avec la matrice dans laquelle elles ont été incorporées s’est avérée indispensable pour obtenir des films nanocomposites avec une distribution homogène et un état de dispersion le plus fin possible. Différentes stratégies de compatibilisation ont été étudiées, comme la physisorption, la chimisorption, l’intercalation ou encore la chélation d’agents compatibilisants judicieusement choisis et adaptés à chacun des systèmes. Les nouvelles nanocharges ainsi modifiées ont été caractérisées en vue de leur introduction dans la matrice. Les films nanocomposites « compatibilisés » ont été élaborés en voie solvant et/ou par polymérisation in-situ, suivie d’une gélification physique pour le PVC ou d’une réticulation chimique pour le PAI. La caractérisation morphologique des films, réalisée par DRX et MEB/MET, ainsi que les propriétés thermiques et thermomécaniques des films, évaluées par ATG, DSC et DMA, mettent en évidence l’importance de deux paramètres : la chimie de surface des nanocharges, à l’origine des interactions interfaciales nanocharge/polymère, et le procédé d’élaboration du nanocomposite. / This study aims at improving some properties of functional PVC- and PAI- based coatings by adding preformed inorganic lamellar or spherical nanofillers. The compatibilization of nanofiller with the polymer matrix in which they are introduced, is required in order to obtain nanocomposite films with an homogeneous distribution and a dispersion state as fine as possible. Different compatibilization strategies, well-suited for each system, have been studied: compatibilizer physisorption, chemisorption, intercalation or chelation. The new modified nanofillers have been characterized before their introduction into the matrix. Various strategies have been considered to obtain the “compatibilized” nanocomposite films such as the solution mixing and/or the in-situ polymerization, followed by a physical gelation or curing step for PVC- or PAI-based nanocomposites, respectively. The morphological characterization of the films, through XRD and SEM/TEM analysis, and the thermal and thermomecanical properties, evaluated by TGA, DSC and DMA, underlined the importance of two parameters: the nanofiller surface chemistry, responsible for the nanofiller/polymer interfacial interactions, and the elaboration process of the nanocomposite.

INTRODUCTION - Plasminogen activator inhibitor type-1 (PAI-1) has a known relationship with obesity and more specifically with central obesity. Traditionally the physiological contribution of PAI-1 is seen as an indicator of fibrinolysis with increased PAI-1 levels contributing to decreased fibrinolysis. In more recent years, assays have been developed that not only uses proxy markers, such as PA-1, which is considered to be representative of fibrinolysis , but global assays that report on the global fibrinolytic potential of an individual, often reported as clot lysis time (CLT). Investigations into the relationship of CLT with obesity are scarce. Preliminary evidence shows that the relationship of CLT with obesity may differ from that of PAI-1 with obesity although in depth investigations in this regard are lacking. Therefore, the aim of this study was to investigate the association between fibrinolysis markers (PAI-1act and CLT) and various markers of body composition in the South African Prospective Urban and Rural Epidemiology (PURE) data collected during 2010. METHODS - Data collected in the PURE study in 2010 were cross-sectionally analysed. The participants (n = 1288) were apparently healthy black South-African men and women 35 years and older, residing in urban and rural settlements in the North-West Province. Experimental methods included anthropometric measurements such as height, weight, hip circumference, waist circumference, skinfolds (triceps, chest, abdominal, thigh and supra iliac skinfolds) and body composition measurements by means of air-displacement plethysmography and biolelectrical impedance analysis. Laboratory analysis of fibrinolysis markers, PAI-1act and CLT were also performed. MAIN FINDINGS - In men, similarities were seen regarding the relationship between PAI-1act and body composition markers and the relationships observed between CLT and body composition markers. In contrast, in the women more and stronger associations were observed between CLT and body composition markers compared to that observed between PAI-1act and body composition markers. CLT showed a linear relationship with body composition markers where PAI-1act levels plateaued at higher body composition categories. Possible reasons for the observed differences may be related to differences in adipose tissue distribution and sequence of accumulation between men and women. PAI-1 is associated with visceral adipose tissue (VAT) where high amounts of stromal cells are found. In men preferential accumulation of VAT may explain similarities in the relationship of PAI-1act with body composition and that of CLT with body composition. Proportionally less VAT, but more subcutaneous adipose tissue in women may explain the observed increase in CLT compared to PAI-1act levels that plateaued over body composition tertiles and categories. CONCLUSION - PAI-1act has a stronger association with central obesity while CLT has a stronger association with total body fat. In women PAI-1act and CLT showed different associations with body composition markers, whereas associations of PAI-1act and CLT with body composition were similar in men. PAI-1act is strongly influenced by type of body fat accumulation whereas CLT is associated with obesity independent of type and sequence of body fact accumulation. Significant associations observed between CLT and body composition variables are, therefore, at least in part, independent of PAI-1act. Additional factors such as, thrombin activatable fibrinolysis inhibitor (TAFI), α-2-antiplasmin, plasminogen, prothrombin and fibrin clot structure that influence CLT and are also related to obesity may additionally contribute to the link between CLT and obesity. / MSc (Dietetics), North-West University, Potchefstroom Campus, 2014

INTRODUCTION - Plasminogen activator inhibitor type-1 (PAI-1) has a known relationship with obesity and more specifically with central obesity. Traditionally the physiological contribution of PAI-1 is seen as an indicator of fibrinolysis with increased PAI-1 levels contributing to decreased fibrinolysis. In more recent years, assays have been developed that not only uses proxy markers, such as PA-1, which is considered to be representative of fibrinolysis , but global assays that report on the global fibrinolytic potential of an individual, often reported as clot lysis time (CLT). Investigations into the relationship of CLT with obesity are scarce. Preliminary evidence shows that the relationship of CLT with obesity may differ from that of PAI-1 with obesity although in depth investigations in this regard are lacking. Therefore, the aim of this study was to investigate the association between fibrinolysis markers (PAI-1act and CLT) and various markers of body composition in the South African Prospective Urban and Rural Epidemiology (PURE) data collected during 2010. METHODS - Data collected in the PURE study in 2010 were cross-sectionally analysed. The participants (n = 1288) were apparently healthy black South-African men and women 35 years and older, residing in urban and rural settlements in the North-West Province. Experimental methods included anthropometric measurements such as height, weight, hip circumference, waist circumference, skinfolds (triceps, chest, abdominal, thigh and supra iliac skinfolds) and body composition measurements by means of air-displacement plethysmography and biolelectrical impedance analysis. Laboratory analysis of fibrinolysis markers, PAI-1act and CLT were also performed. MAIN FINDINGS - In men, similarities were seen regarding the relationship between PAI-1act and body composition markers and the relationships observed between CLT and body composition markers. In contrast, in the women more and stronger associations were observed between CLT and body composition markers compared to that observed between PAI-1act and body composition markers. CLT showed a linear relationship with body composition markers where PAI-1act levels plateaued at higher body composition categories. Possible reasons for the observed differences may be related to differences in adipose tissue distribution and sequence of accumulation between men and women. PAI-1 is associated with visceral adipose tissue (VAT) where high amounts of stromal cells are found. In men preferential accumulation of VAT may explain similarities in the relationship of PAI-1act with body composition and that of CLT with body composition. Proportionally less VAT, but more subcutaneous adipose tissue in women may explain the observed increase in CLT compared to PAI-1act levels that plateaued over body composition tertiles and categories. CONCLUSION - PAI-1act has a stronger association with central obesity while CLT has a stronger association with total body fat. In women PAI-1act and CLT showed different associations with body composition markers, whereas associations of PAI-1act and CLT with body composition were similar in men. PAI-1act is strongly influenced by type of body fat accumulation whereas CLT is associated with obesity independent of type and sequence of body fact accumulation. Significant associations observed between CLT and body composition variables are, therefore, at least in part, independent of PAI-1act. Additional factors such as, thrombin activatable fibrinolysis inhibitor (TAFI), α-2-antiplasmin, plasminogen, prothrombin and fibrin clot structure that influence CLT and are also related to obesity may additionally contribute to the link between CLT and obesity. / MSc (Dietetics), North-West University, Potchefstroom Campus, 2014

Inhibitors belonging to the serpin (serine protease inhibitor) family control proteases involved in various physiological processes. All serpins have a common tertiary structure based on the dominant b-sheet A, but they have different inhibitory specificity. The specificity of a serpin is determined by the Pl-Pl’ peptide bond acting as a bait for the target protease which is made up of an exposed reactive centre loop (RCL). The serpin plasminogen activator inhibitor type-1 (PAI-1) is the main physiological inhibitor of urokinase-type and tissue-type plasminogen activators (uPA and tPA, respectively). Elevated plasma levels of PAI-l have been correlated with a higher risk of deep venous thrombosis, and PAI-1 is a risk factor for recurrent myocardial infarction. Furthermore, PAI-1 has a role in cell migration and has been suggested to regulate tumor growth and angiogenesis. PAI-1 is unique among the serpins in that it can spontaneously and rapidly convert into its latent form. This involves full insertion of the RCL into b-sheet A. There were two partially overlapping goals for this thesis. The first was to use latent PAI-1 as model for development of a fluorescence-based method, Donor-Donor Energy Migration for intramolecular distance measurements. The second goal was to use DDEM, together with other biochemical methods, to reveal the structure of the PAI-1/uPA complex, the conformation of the RCL in active PAI-1, and molecular determinants responsible for the conversion of PAI-1 from the active to the latent form. The use of molecular genetics for introduction of fluorescent molecules enables the use of DDEM to determine intramolecular distances in a variety of proteins. This approach can be applied to examin the overall molecular dimensions of proteins and to investigate structural changes upon interactions with specific target molecules. In this work, the accuracy of the DDEM method has been evaluated by experiments with the latent PAI-1 for which X-ray structure is known. Our data show that distances approximating the Förster radius (57±1 Å) obtained by DDEM are in good agreement (within 5.5 Å) with the distances obtained by X-ray crystallography. The molecular details of the inhibitory mechanism of serpins and the structure of the serpin/protease complex have remained unclear. To obtain the structural insights required to discriminate between different models of serpin inhibition, we used fluorescence spectroscopy and cross-linking techniques to map sites of PAI-1/uPA interaction, and distance measurement by DDEM to triangulate the position of the uPA in the complex. The data have demonstrated clearly that in the covalent PAI-1/uPA complex, the uPA is located at the distal end of the PAI-1 molecule relative to the initial docking site. This indicates that serpin inhibition involves reactive center cleavage followed by full loop insertion, whereby the covalently linked protease is translocated from one pole of the inhibitor to the opposite one. To search for molecular determinants that could be responsible for conversion of PAI-1 to the latent form, we studied the conformation of the RCL in active PAI-1 in solution. Intramolecular distance measurements by DDEM, the newly a developed method based on probe quenching and biochemical methods revealed that the RCL in PAI-1 is located much closer to the core of PAI-1 than has been suggested by the recently resolved X-ray structures of stable PAI-1 mutants, and it can be partially inserted. This possibly explains for the ability of PAI-1 to convert spontaneously to its latent form.

[pt] Este estudo teve como objetivo buscar, em alguns textos de Freud, a visão do autor a respeito da figura paterna e de sua relevância para a subjetivação do sujeito. São enfatizados os desenvolvimentos teóricos sobre a formulação e a importância do complexo de Édipo, tanto o masculino como o feminino, para a formação do superego, bem como a importância do narcisismo para a construção do eu e para o processo de identificação. São enfocadas, ainda, as mudanças ocorridas no ambiente social, desde a época de Freud aos dias atuais, que propiciaram questões acerca da família, com alterações na relação homem/mulher e na função de cada gênero, provocando o declínio da figura paterna devido às novas configurações familiares. Além disso, são apresentadas as novas abordagens sobre paternidade, função paterna e lugar do pai no contexto das múltiplas configurações familiares contemporâneas. / [en] This study aimed at searching in some of Freud s writings the author s view of the paternal figure and its relevance to the individual s subjectivity. We emphasize the theoretical developments regarding the formulation and the importance of the Oedipus complex - both male and female - to the formation of the superego, as well as the importance or narcissism in the I construction and in the identification process. We also highlight the changes occurred in social environment, from Freudian to present time, which had raised questions about the family, with changes in the relationship between men and women, and in the functions of each gender, causing the decline of paternal figure in new family configurations. In addition, we present new approaches on paternity, paternal function and father s place in the context of multiple family configurations in contemporaneity.

[pt] As imensas modificações sociais ocorridas nos últimos tempos, tais como a possibilidade do divórcio e de vários recasamentos, assim como da união estável; a existência de relacionamentos não reconhecidos juridicamente, como a união de homossexuais; e as várias descobertas biotecnológicas, têm- se refletido nas famílias, provocando uma enorme alteração estrutural nas mesmas. Nas novas famílias, vínculos fortes se formam, entre pessoas que não são biologicamente ligadas e não têm vínculo jurídico reconhecido. A noção que a Lei traz de família, como sendo composta por um homem e uma mulher, unidos por matrimônio ou união estável, e os filhos a eles ligados por laços de sangue ou pela adoção, já está superada, e se faz necessária uma maior compreensão a respeito dessas novas relações e desses novos vínculos, para que se consiga, através do sistema legislativo e jurídico, atender ao melhor interesse da criança e do adolescente, princípio adotado em nível internacional como orientador em assuntos que envolvem os menores. O tema eleito para investigação foram os vínculos psicossociais existentes entre pessoas sem ligação biológica ou jurídica, entre as quais exista uma relação tal que, durante a infância/adolescência de uma delas, a outra tenha exercido funções maternas, paternas ou fraternas. Foi objetivo deste estudo descortinar a realidade desses vínculos e o nível de importância e influência que têm nas vidas e na constituição da subjetividade das crianças e adolescentes, buscando facilitar, desse modo, a compreensão dessas relações. Foram entrevistadas treze pessoas, divididas em sete duplas: duas duplas maternofiliais, duas duplas paterno-filiais, duas duplas fraternas e uma dupla homoparental-filial feminina, membros de famílias em que existiam, desde a mais tenra idade de pelo menos um deles, a convivência com outro com quem não havia nem vínculo consangüíneo, nem adotivo, mas que desempenhava, em relação ao primeiro, funções maternas, paternas ou fraternas. Da análise das entrevistas e de seu confronto com a revisão da literatura, restou fortalecida a convicção de que a genética e a legislação pouco ou nada têm a ver com a questão da família, e que vínculos familiares estão muito além da consangüinidade, sendo formados a partir das experiências e vivências compartilhadas e das funções exercidas perante os demais membros do grupo familiar. / [en] The major social changes having occurred in recent times, such as the possibility of divorce and multiple remarriages, the advent of stable unions or the existence of relationships which have no legal standing, such as those between homosexual couples; coupled with advances in biotechnology, have had a profound effect on and caused a major structural upheaval in the family. In the new family strong bons are formed between people who have no biological ties and who have no legally-recognised relationship. The notion that the law has had of the family - as being made up of one man and one woman, united by marriage or stable union, along with their children, whether these are their own or they have been adopted - is outdated and greater comprehension is required of these new relationships and ties, in order for the legislative and judicial system to be able to really serve the best interests of the child and adolescent - an internationallyadopted principle in matters involving minors. The question chosen for investigation was the psycho-social ties between persons who had neither blood ties nor legally-recognised relationships, and between whom there was a relation such as that during the childhood or the adolescence of one of them, the other had played the role of mother, father or brother. The aim of the study was to investigate the reality of these ties and the degree of importance and influence they have on the life and the formation of the child/adolescent´s subjective makeup, in order to facilitate the understanding of these relationships. Thirteen people were interviewed, divided into of seven pairs: two mother- and-child pairs, two father-and-child pairs, two sibling pairs and one homo- parent-child pair, members of families where one of them lived with the other since infancy, and with whom there were neither blood nor adoptive ties, but who nevertheless played the role of either the mother, father or brother to the other. Analysis of the interview material along with a reexamination of the literature supports the idea that neither genetics nor the law has much to do with family matters; that family ties go far beyond mere consanguinity and that the formation of such bonds is based on common life experiences and the role played with respect to the remaining family members.

Made available in DSpace on 2016-08-10T10:37:31Z (GMT). No. of bitstreams: 1 Valquiria Guimaraes Duarte.pdf: 1197381 bytes, checksum: 82ead4bf7879228c2588ffc51316ae4f (MD5) Previous issue date: 2004-07-15 / This study analysis the relevance of the expressions of culture through the pilgrimage of the oxcart drives that leaves the city of Mossâmedes and the surrounding areas towards the Feast of the Eternal Divine Father, held at the shrine in Trindade, state of Goiás in the beginning of July, showing how their agents are articulated in their own way of construting, reinforcing and renewing their religious and social identity. Getting to know the cult the Holy Trinity or, as it is called by the pilgrims, the Divine Eternal Father , is to immerse oneself in a multifaceted complexity, encompassing all its meanings. Restoring part of this diversity through the yearly pilgrimage of the oxcart drives necessarily implies embracing the discourse of the different groups represented in the church, or on their way there: pilgrims, priests, the people in charge if managing the sacred. These presences restore and revitalize the memory of the saint, in order to do this, they immerse themselves in the diversified interplay between the historical fact and the myth. The ethnographic analysis of the pilgrimage explains certain aspects of the systems related to the representation, beliefs, values expressed not only through discourses of the oxcart drivers, but mainly through their ritual actions. The pilgrimage reveals itself as a contemporary purpose of continuing the elements of the rural tradicional culture and the popular Catholicism, and at the same time it is presented as a possibility of dialoguing with determined aspects of the urban culture. / Este estudo analisa a relevância das expressões da cultura através da romaria de carreiros, que sai da cidade de Mossâmedes e arredores em direção à Festa do Divino Pai Eterno - que acontece no santuário de Trindade, estado de Goiás no início do mês de Julho, mostrando como se articulam seus agentes no modo de construir, reforçar e renovar sua identidade religiosa e social. Conhecer o culto à Santíssima Trindade, ou como é denominado pelos romeiros o Divino Pai Eterno , é imergir em uma complexidade multifacetada em seus sentidos. Restituir parte dessa diversidade através da romaria dos carreiros necessariamente implica abranger discursos de diferentes grupos representados no santuário ou a caminho dele: romeiros, clérigos, administradores do sagrado. Estas presenças restituem e revitalizam a memória do santo; para isto entregam-se ao jogo variegado da história e do mito. A análise etnográfica trata de explicar certos aspectos do sistema de representação, crenças, valores expressos não somente através dos discursos dos carreiros, mas principalmente através de suas ações rituais. A romaria se revela como uma proposta contemporânea de continuidade de elementos das culturas tradicionais rurais e do catolicismo popular, ao mesmo tempo se apresenta como uma possibilidade de dialogar com certos aspectos das culturas urbanas.

Made available in DSpace on 2016-07-27T13:48:53Z (GMT). No. of bitstreams: 1 Joao Otavio Martins.pdf: 627917 bytes, checksum: aa97401db6b119fc346cd79fb3cdbd9c (MD5) Previous issue date: 2001-06-26 / O santuário, cercado por uma realidade diversificada é o centro das atenções. Por resguardar a imagem da Santíssima Trindade, Pai, Filho e Espírito Santo coroando a Virgem Maria, o santuário acolhe milhares de peregrinos que chegam de todos os cantos do Brasil e do mundo agradecendo benefícios recebidos e fazendo pedidos segundo necessidades diversas. A reprodução social é constatada na peregrinação entre o profano e o sagrado. Numa cultura familiar que resiste aos valores novos, mas que é influenciada e transformada. Num contexto da modernidade leva-se ao objetivo de tornarem-se evidentes os significados da tradição. Pela experiência do autor na prática e teoria no assunto religião torna-se plausível a escolha do tema: os peregrinos do Divino Pai Eterno e do objeto de estudo: os carreiros e a reprodução da tradição. O autor pertence ao grupo dos missionários redentoristas, uma congregação religiosa requisitada no final do século XIX na Alemanha para cristianizar as romarias em Trindade. Para obter-se uma compreensão mais abrangente e original do assunto delimitado realizou-se uma pesquisa participante. A coleta de dados foi obtida através da filmagem, fotografias e depoimentos Numa dinâmica de transformação os valores tradicionais se mantêm, até mesmo ameaçados pelas mudanças ocorridas e influenciados pelo capitalismo industrial que proporcionou o aparecimento de novas técnicas e uma pluralidade de valores novos. A peregrinação e a festa culminam no santuário, mostrando a sua realidade interna rica em símbolos.