998 resultados para Guillaume de Lorris


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Mémoire numérisé par la Direction des bibliothèques de l'Université de Montréal.

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Common features such as elastic fibre destruction, mucoid accumulation, and smooth muscle cell apoptosis are co-localized in aneurysms of the ascending aorta of various aetiologies. Recent experimental studies reported an activation of TGF-beta in aneurysms related to Marfan (and Loeys-Dietz) syndrome. Here we investigate TGF-beta signalling in normal and pathological human ascending aortic wall in syndromic and non-syndromic aneurysmal disease. Aneurysmal ascending aortic specimens, classified according to aetiology: syndromic MFS (n = 15, including two mutations in TGFBR2), associated with BAV (n = 15) or degenerative forms (n = 19), were examined. We show that the amounts of TGF-beta 1 protein retained within and released by aneurysmal tissue were greater than for control aortic tissue, whatever the aetiology, contrasting with an unchanged TGF-beta 1 mRNA level. The increase in stored TGF-beta 1 was associated with enhanced LTBP-I protein and mRNA levels. These dysiregulations of the extracellular ligand are associated with higher phosphorylated Smad2 and Smad2 mRNA levels in the ascending aortic wall from all types of aneurysm. This activation correlated with the degree of elastic fibre fragmentation. Surprisingly, there was no consistent association between the nuclear location of pSmad2 and extracellular TGF-beta 1 and LTBP-I staining and between their respective mRNA expressions. In parallel, decorin. was focally increased in aneurysmal media, whereas biglycan was globally decreased in aneurysmal aortas. In conclusion, this study highlights independent dysregulations of TGF-beta retention and Smad2 signalling in syndromic and non-syndromic aneurysms of the ascending aorta. Copyright (C) 2009 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.

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Histopathological alterations in human aneurysms and dissections of the thoracic ascending aorta include areas of mucoid degeneration within the medial layer, colocalized with areas of cell disappearance and disruption of extracellular matrix elastic and collagen fibers. We studied the presence of matrix metalloproteinases in relation to their capacity to diffuse through the tissue or to be retained in areas of mucoid degeneration in aneurysms and dissections of the ascending aorta. Ascending aortas from 9 controls, 33 patients with aneurysms, and 14 with acute dissections, all collected at surgery, were analyzed. The morphological aspect was similar whatever the etiology or phenotypic expression of the pathological aortas, involving areas of extracellular matrix breakdown and cell rarefaction associated with mucoid degeneration. Release of proMMP-2, constitutively expressed by smooth muscle cells, was not different between controls and aneurysmal aortas, whereas the aneurysmal aortas released more of the active form. Release of pro and active MMP-9 was also similar between controls and aneurysmal aortas. Immunohistochemical staining of MMP-2 and MMP-9 was weak in both control and pathological aortas. In contrast, released MMP-7 (matrilysin) and MMP-3 (stromelysin-1) could not be detected in conditioned media but were present in tissue extracts with no detectable quantitative difference between controls and pathological aortas. Immunohistochemical staining of MMP-7 and MMP-3 revealed their retention in areas of mucoid degeneration, and semiquantitative evaluation of immunostaining showed more MMP-7 in pathological aortas than in controls. In conclusion, areas of mucoid degeneration, the hallmark of aneurysms, and dissections of thoracic ascending aortas, whatever their etiology, are not inert and can retain specific proteases. (c) 2009 Elsevier Inc. All rights reserved.

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Fibrinolytic activity is associated with presence of cystic medial degeneration in aneurysms of the ascending aorta Aims: Thoracic ascending aortic aneurysms (TAA) are characterized by elastic fibre breakdown and cystic medial degeneration within the aortic media, associated with progressive smooth muscle cell (SMC) rarefaction. The transforming growth factor (TGF)-beta/Smad2 signalling pathway is involved in this process. Because the pericellular fibrinolytic system activation is able to degrade adhesive proteins, activate matrix metalloproteinase (MMP), induce SMC disappearance and increase the bioavailability of TGF-beta, the aim was to investigate the plasminergic system in TAA. Methods and results: Ascending aortas [21 controls and 19 TAAs (of three different aetiologies)] were analysed. Immunohistochemistry showed accumulation of t-PA, u-PA and plasmin in TAAs, associated with residual SMCs. Overexpression of t-PA and u-PA was confirmed by reverse transcription-polymerase chain reaction (RT-PCR), immunoblotting and zymography on TAA extracts and culture medium conditioned by TAA. Plasminogen was present on the SMC surface and inside cytoplasmic vesicles, but plasminogen mRNA was undetectable in the TAA medial layer. Plasmin-antiplasmin complexes were detected in TAA-conditioned medium and activation of the fibrinolytic system was associated with increased fibronectin turnover. Fibronectin-related material was detected immunohistochamically in dense clumps around SMCs and colocalized with latent TGF-beta binding protein-1. Conclusions: The fibrinolytic pathway could play a critical role in TAA progression, via direct or indirect impact on ECM and consecutive modulation of TGF-beta bioavailability.

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Duchenne muscular dystrophy (DMD) is a severe, progressive disease first described by Meryon in 1852 and later by Guillaume Duchene. It is the most common and severe form of childhood muscular dystrophy, affecting 1 in 3500 live male births. Is caused by an X—linked recessive genetic disorder resulting in a deficiency of the dystrophin protein, responsible for linking contractile proteins to the sarcolemma. Diagnosis is not always easy and the first symptoms are often related to weakness and difficulty or delay in acquiring the ability to perform simple activities. Progressive weakness leads to the use of compensatory strategies in order to maintain the ability to walk and perform other activities. Respiratory muscles are also affected and the complications resulting from its impairments are frequently the cause of early death of these patients. The advances in DMD management has increased life expectancy of these children with the need for adequate care in adulthood. DMD manifestations include muscle weakness, contractures, respiratory and cardiac complications. Some authors also refer that one-third of patients have difficulties with learning and delayed global development because the gene that encodes dystrophyn expresses various dystrophin isoforms that are found in Schwann and Purkinje celis in the brain. Body functions and structure impairments like muscle weakness, contractures and reduced range of motion lead to limitations in activities, i.e., impairments affect the performance of tasks by the individual. In a physiotherapist’s point of view analysing these limitations is mandatory because physiotherapy’s final purpose is to restore or preserve the ability to perform ADL and to improve quality of life.

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Double Degree. A Work Project presented as part of the requirements for the Award of a Master Degree in Management from the NOVA – School of Business and Economics and a Master Degree in Business Engineering from Louvain School of Management

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Gravity Recovery and Climate Experiment (GRACE) mission is dedicated to measuring temporal variations of the Earth's gravity field. In this study, the Stokes coefficients made available by Groupe de Recherche en Géodésie Spatiale (GRGS) at a 10-day interval were converted into equivalent water height (EWH) for a ~4-year period in the Amazon basin (from July-2002 to May-2006). The seasonal amplitudes of EWH signal are the largest on the surface of Earth and reach ~ 1250mm at that basin's center. Error budget represents ~130 mm of EWH, including formal errors on Stokes coefficient, leakage errors (12 ~ 21 mm) and spectrum truncation (10 ~ 15 mm). Comparison between in situ river level time series measured at 233 ground-based hydrometric stations (HS) in the Amazon basin and vertically-integrated EWH derived from GRACE is carried out in this paper. Although EWH and HS measure different water bodies, in most of the cases a high correlation (up to ~80%) is detected between the HS series and EWH series at the same site. This correlation allows adjusting linear relationships between in situ and GRACE-based series for the major tributaries of the Amazon river. The regression coefficients decrease from up to down stream along the rivers reaching the theoretical value 1 at the Amazon's mouth in the Atlantic Ocean. The variation of the regression coefficients versus the distance from estuary is analysed for the largest rivers in the basin. In a second step, a classification of the proportionality between in situ and GRACE time-series is proposed.

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O Centro de Endemismo Belém (CEB) -região biogeográfica localizada entre os rios Tocantins e Pindaré- é a área mais desmatada da Amazônia. Estudar a biota do solo dos remanescentes florestais e de áreas em regeneração (capoeiras) do CEB é fundamental para promover práticas agrícolas mais sustentáveis e orientar ações de restauração de áreas degradadas. Dessa forma, o objetivo desse estudo foi identificar os principais determinantes da composição e da diversidade dos grandes táxons de macrofauna do solo (uso atual do solo, núcleos geográficos, histórico de uso, freqüência de fogo) em uma cronoseqüência de capoeiras e florestas no CEB. A macrofauna foi amostrada em oito áreas de capoeiras baixas (4-7 anos), seis áreas de capoeiras médias (11-15 anos), sete áreas de capoeiras altas (20-40 anos), oito áreas de remanescentes de florestas e três áreas de pastagem. Sua composição e diversidade foi estudada através de Análises de Componentes Principais e Entre-classes. Um total de 9.225 indivíduos pertencentes a 29 grandes taxóns (ordens ou famílias) foram coletados nas 32 parcelas amostradas. A composição da macrofauna se mostrou sensível ao efeito do histórico de uso do solo e identificou comunidades significativamente distintas entre as micro-regiões, havendo nessa escala efeito da cronosequência de capoeiras e florestas. A intensidade do uso do solo levou a redução da abundância e da riqueza de predadores, indicando alterações significativas no funcionamento do solo. Esses resultados mostram um efeito duradouro da degradação sobre a macrofauna do solo e conseqüentemente sobre suas funções ecológicas.

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As florestas ripárias provêem serviços ecossistêmicos essenciais à humanidade, mas estão sendo degradadas em um ritmo acelerado na Amazônia. Esse estudo teve como objetivo quantificar os estoques de carbono acima e abaixo do solo de florestas ripárias com quatro níveis de degradação (muito alta, alta, média e baixa) em duas microbacias (Rio Pepital e Rio Grande) em Alcântara (Maranhão), na Amazônia Oriental. Foram instaladas 24 parcelas permanentes de 1.000 m2, onde a vegetação (árvores, arbustos, herbáceas, lianas e palmeiras), a necromasa (serapilheira, árvores mortas, galhos) e o solo (0-20 cm e raízes) foram amostrados. A biomassa foi estimada mediante modelos alométricos (vegetação com DAP > 1 cm e árvores mortas) e de forma destrutiva (herbáceas, serapilheira, galhos finos, raízes). O estoque total de carbono nas florestas ripárias mais conservadas variou entre 88 e 202 Mg(C) ha-1. A degradação reduziu significativamente o estoque de carbono em todos os compartimentos (até 97% na biomassa viva acima do solo, até 91% de necromassa e até 47% no solo). Nas áreas conservadas, a biomassa viva acima do solo tem a maior participação no estoque total de carbono (> 70%), principalmente devido às árvores de grande porte. O estoque de carbono nos diferentes compartimentos estão fortemente relacionados entre si e também com a abertura do dossel. A incorporação desses resultados em modelos regionais de carbono pode auxiliar na implementação e revisão do Código Florestal Brasileiro, em particular na restauração das florestas ripárias onde a agricultura está consolidada.

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We identify in this paper two conditions that characterize the domain of single-peaked preferences on the line in the following sense: a preference profile satisfies these two properties if and only if there exists a linear order $L$ over the set of alternatives such that these preferences are single-peaked with respect L. The first property states that for any subset of alternatives the set of alternatives considered as the worst by all agents cannot contains more than 2 elements. The second property states that two agents cannot disagree on the relative ranking of two alternatives with respect to a third alternative but agree on the (relative) ranking of a fourth one. Classification-JEL: D71, C78