Primary cilia are nonmotile, hair-shaped organelles that extend from the basal body in the centrosome. The present study is the first investigation of this organelle in the oligodendrocyte lineage in vivo. I used immunohistochemical approaches in normal and cilia-deficient mutant mice to study cilia in relation to oligodendrogenesis and myelination. Primary cilia immunoreactive for Arl13b and ACIII were commonly present in NG2+ oligodendrocyte progenitor cells (OPCs), in which cilia-associated pathways control proliferation, differentiation, and migration. The loss of primary cilia is generally associated with enhanced Wnt/β-catenin signaling, and Wnt/β-catenin signaling has been shown to promote myelin gene expression. I examined whether the lack of cilia in the oligodendrocyte lineage is associated with elevated Wnt/β-catenin activity. I found that absence of a primary cilium was associated with with higher levels of TCF3, and with β-galactosidase in Axin2-lacZ Wnt reporter mice. This evidence supports the proposal that cilia loss in oligodendrocytes leads to enhanced Wnt/β-catenin activity, which promotes myelination. Cilia are dependent on the centrosome, which assembles microtubules for the cilium, the cytoskeleton, and the mitotic spindle. Centrosomes are the organizing center for microtubule assembly in OPCs, but this function is decentralized in oligodendrocytes. I found that the intensity of centrosomal pericentrin was reduced in oligodendrocytes relative to OPCs, and γ-tubulin was evident in centrosomes of OPCs but not in mature oligodendrocytes. These decreases in centrosomal proteins might contribute to functional differences between OPCs and oligodendrocytes. The importance of cilia in the oligodendrocyte lineage was examined in Tg737orpk mice, which have a hypomorphic IFT88 mutation resulting in decreased cilia numbers and lengths. These mice showed marked, differential decreases in numbers of oligodendrocytes and myelin, yet little or no change in OPC populations. It appears that sufficient cells were available for maturation, but lineage progression was stalled. There were no evident effects of the mutation on Wnt/β-catenin. Factors that might contribute to the abnormalities in the oligodendrocyte lineage of Tg737orpk mice include decreased cilia-dependent Shh mitogenic signaling and dysregulation in cilia-associated pathways such as Notch and Wnt/β-catenin.

Les cellules épithéliales nasales humaines (CENH) sont des acteurs importants de la physiopathologie de la polypose naso-sinusienne (PNS), le plus souvent la PNS est primitive (étiologie inconnue), plus rarement, elle est secondaire à la mucoviscidose (PNS CF) ou la dyskinésie ciliaire primitive (PNS DCP). L'objectif de cette thèse est d'identifier des protéines différentiellement exprimées dans les PNS primitives et secondaires par approche protéomique puis de les caractériser par des techniques classiques. Dans cette étude, nous avons utilisé des cultures primaires (interface air/liquide) de CENH, dérivant de déchet per-opératoire (PNS) ou de brossages (muqueuse saine). Le marquage iTRAQ suivie d'une analyse par nano-LC-MALDI-TOF-TOF à en particulier montré : 1) une augmentation de six marqueurs du stress du réticulum endoplasmique (RE) dans la PNS primitive et la PNS CF, 2) une altération du métabolisme du glucose dans la PNS CF (sous-expression de 6 protéines dont la pyruvate kinase, enzyme clef de la glycolyse) et 3) une sous expression de 12 protéines des filaments acto-myosines dans la PNS DCP (dont 3 tropomyosines). Nous avons caractérisé le stress du RE dans la PNS primitive, montrant qu'il est induit par le biais d'une sensibilité des CENH à un stress oxydatif probablement d'origine mitochondrial et qu'il participe activement à l'inflammation (sécrétion d'IL-8 et de LTB4). Ces observations nous permettent de proposer que, dans la PNS primitive, il existe un cercle vicieux entre stress du RE, stress oxydatif et inflammation et que la sensibilité au stress oxydatif observée apparaît comme une nouvelle cible thérapeutique dans le traitement de la PNS. / Human nasal epithelial cells (HNEC) play an important role in nasal polyposis(NP). NP have generally an unknown etiology, less frequently, NP is secondary to cystic fibrosis (CF NP) or primary ciliary dyskinesia (PCD NP). The aim of this thesis is to identify differentially expressed proteins in NP, CF NP and PCD NP using proteomic approach then to characterize them by classical methodes. In this study, we used primary culture (air/liquid)of HNEC derived from NP patients (surgery waste) or healthy patients (brushing). The iTRAQ labeling folowing by nano-LC-MALDI-TOF-TOF analysis shown in particular :1)an increased expression of six endoplasmic reticulum (ER) stress markers in NP and CF NP, 2)an alteration of glucose metabolism (decreased expression of six proteins with in particular the pyruvate kinase, glycolysis key enzyme) and 3)an decreased expression of 12 acto-myosin filament proteins (in particular three tropomyosins). We caracterized the ER stress in NP showing that ER stress is activated by an oxydative stress susceptibility of NP HNEC probably of mitochondrial origin and that it directly participate to inflammation (IL-8 and LTB4 secretions). Altogether, our results underline a vicious circle that exists between oxidative stress, ER stress and inflammation.The oxydative stress susceptibility observed in NP may represent new therapeutic target in such a pathology.

Made available in DSpace on 2016-08-19T18:20:36Z (GMT). No. of bitstreams: 1 NAILA ARRAES DE ARAUJO.pdf: 3510798 bytes, checksum: 89545fa06219ca0586c927136b354d6a (MD5) Previous issue date: 2008-11-11 / Coordenação de Aperfeiçoamento de Pessoal de Nível Superior / The lacustrine area of Penalva, in the Baixada Maranhense region, is formed by the lakes Cajari, Capivari, Lontra and Formoso, which encompass a diversified ciliary vegetation. Those environments, subject to the influence of seasonal floods, provide a variety of habitats for shelter, reproduction and feeding of fish species. Some of the fish species, as they feed from fruits and seeds from ciliary plant species they help in the process of seed dispersion, contributing to the reproductive success of the plants. This research aimed at studying the relationship between the ichthyologic fauna of the lacustrine area of Penalva and the ciliary vegetation and its implications in the sustainability of the regional fishing. More specifically, to analyze the relationship among the different plant species and the reproductive period, feeding habits and shelter of the fish species, investigating which species are dependent on the ciliary vegetation and which species function as seed dispersers. The methodology included, in a first phase, interviews with fishermen (key informants), by using semi-structured questionnaires, to record the traditional knowledge on fish and vegetation. In a second phase, monthly collections of fish specimens from the lakes Cajari and Capivari were carried out in the period April, 2007 to June, 2008; following the collections, the specimens were taken to laboratory analyses. The results showed that fishermen possess a vast knowledge on the relationship between the ichthyofauna and the regional ciliary vegetation, making clear the dependence between fish and plants. In this study eleven fish species were identified as seed dispersers of eleven ciliary plant species. In the rainy season, when extensive vegetation areas are flooded, the biggest number of fish specimens was recorded with full stomachs, as well as the biggest number of males and females in the maturation phase, confirming the importance of the ciliary forests for the ichthyofauna. The ciliary forests of the lacustrine area of Penalva have been suffering along the years from deforestation and burning that also affect fishing and local man. The effects from vegetation suppression can be already felt with the decrease of the size and quantity of fish species and individuals, according to local people. It is clear, therefore, the relationship between the reduction of the ciliary vegetation and the signs of fishing decline in the region. If control measures and conservation actions are not taken, sustainability of regional fishing can be seriously compromised. / A região lacustre de Penalva, na Baixada Maranhense, é formada pelos lagos Cajari, Capivari, da Lontra e Formoso, que dão base em seus entornos, a uma diversificada vegetação ciliar. Esses ambientes, sujeitos às influências de inundações sazonais, propiciam uma variedade de habitats para abrigo, reprodução e alimentação de peixes. Algumas espécies de peixes ao se alimentarem de frutos e sementes das espécies vegetais ciliares ajudam no processo de dispersão das sementes, contribuindo para o sucesso reprodutivo das plantas. Esta pesquisa teve como objetivo estudar a relação da fauna ictiológica da região lacustre de Penalva com a vegetação ciliar e suas implicações na sustentabilidade da pesca regional. Mais especificamente, analisar a relação entre as diferentes espécies vegetais e o período reprodutivo, alimentação e abrigo dos peixes, investigando quais as espécies de peixes dependentes da vegetação ciliar e quais as espécies dispersoras de sementes. A metodologia incluiu, em uma primeira fase, entrevistas com pescadores informantes-chaves, utilizando-se questionários semi-estruturados, para resgate e uso do conhecimento tradicional sobre peixes e vegetação. Em uma segunda fase, foram realizadas coletas mensais de peixes nos lagos Cajari e Capivari, no período de abril de 2007 a junho de 2008, seguidas de análises laboratoriais. Os resultados mostraram que os pescadores possuem conhecimentos apurados sobre a relação da ictiofauna e a vegetação ciliar regional e evidenciaram a dependência entre peixes e plantas. Neste estudo foram identificadas onze espécies de peixes dispersoras de sementes de onze espécies vegetais ciliares. Na estação chuvosa, quando grandes áreas de vegetação encontram-se inundadas, foi registrado o maior número de peixes com estômagos cheios e o maior número de machos e fêmeas em estágio de maturação, confirmando a importância da mata ciliar para a ictiofauna. As áreas de vegetação ciliar da região lacustre de Penalva vêm sofrendo ao longo dos anos, com desmatamentos e queimadas que afetam também a pesca e o homem local. Os efeitos da supressão da vegetação já se fazem sentir com a diminuição do tamanho e quantidade de peixes, segundo o conhecimento local. É clara, pois, a relação entre a redução da vegetação ciliar e os sinais de declínio da pesca na região. Se medidas de controle e conservação não forem adotadas, a tendência é a de comprometimento da sustentabilidade da pesca regional.

<p>Ultrastructural studies of airway epithelium in airway disease are important for diagnosis and understanding the underlying pathology which helps clinicians to improve the patients' treatment.</p><p>Airway biopsies from a 5-month old boy with respiratory problems and gastro-oesophageal reflux were studied by transmission electron microscopy (TEM). The tracheal columnar cells showed accumulation of lamellar bodies, indicative of lysosomal storage disease. The patient was diagnosed with Gaucher disease type 2.</p><p>Shedding of airway epithelial cells is commonly found in asthma. The attachment of these cells to the basal lamina was investigated by TEM of biopsies from patients with asthma and healthy controls. The contact area between columnar cells and basal lamina in asthmatics was significantly less than in controls. Attachment of columnar cells to the basal lamina occurs mainly indirectly, via desmosomal attachment to basal cells. </p><p>Primary ciliary dyskinesia (PCD) is a congenital disease. It is important to differentiate PCD from acquired (secondary) ciliary dyskinesia (SCD). The number of dynein arms determined by TEM was 1.5 and 1.4 for outer and inner dynein arms, respectively in PCD, versus 7.9 and 5.2 for controls and 8.1 and 5.9 in SCD. Compared to PCD patients, SCD patients have more structurally abnormal cilia. A significant difference was found in orientation of the central microtubule pair between PCD and SCD, but also overlap. </p><p>Leukotriene receptor antagonists are a new treatment for asthma. Both corticosteroids and montelukast caused apoptosis and necrosis of airway epithelial cells, and reduced the expression of intercellular adhesion molecule-1. Treatment of cells with tumor necrosis factor-α or interferon-γ reduced the fraction of the lateral cell membrane occupied by desmosomes and this effect was counteracted by corticosteroids. </p>

Ultrastructural studies of airway epithelium in airway disease are important for diagnosis and understanding the underlying pathology which helps clinicians to improve the patients' treatment. Airway biopsies from a 5-month old boy with respiratory problems and gastro-oesophageal reflux were studied by transmission electron microscopy (TEM). The tracheal columnar cells showed accumulation of lamellar bodies, indicative of lysosomal storage disease. The patient was diagnosed with Gaucher disease type 2. Shedding of airway epithelial cells is commonly found in asthma. The attachment of these cells to the basal lamina was investigated by TEM of biopsies from patients with asthma and healthy controls. The contact area between columnar cells and basal lamina in asthmatics was significantly less than in controls. Attachment of columnar cells to the basal lamina occurs mainly indirectly, via desmosomal attachment to basal cells. Primary ciliary dyskinesia (PCD) is a congenital disease. It is important to differentiate PCD from acquired (secondary) ciliary dyskinesia (SCD). The number of dynein arms determined by TEM was 1.5 and 1.4 for outer and inner dynein arms, respectively in PCD, versus 7.9 and 5.2 for controls and 8.1 and 5.9 in SCD. Compared to PCD patients, SCD patients have more structurally abnormal cilia. A significant difference was found in orientation of the central microtubule pair between PCD and SCD, but also overlap. Leukotriene receptor antagonists are a new treatment for asthma. Both corticosteroids and montelukast caused apoptosis and necrosis of airway epithelial cells, and reduced the expression of intercellular adhesion molecule-1. Treatment of cells with tumor necrosis factor-α or interferon-γ reduced the fraction of the lateral cell membrane occupied by desmosomes and this effect was counteracted by corticosteroids.

While prolonged nearwork is considered to be an environmental risk factor associated with myopia development, an underlying genetic susceptibility to nearwork-induced accommodative adaptation may be one possible mechanism for human myopia development. As the control of accommodation by the autonomic system may be one such genetically predetermined system, this research sought to investigate whether an anomaly of the autonomic control of accommodation may be responsible for myopia development and progression. The emphasis of this work was determining the effect of altering the sympathetic input to the ciliary muscle on accommodation responses such as tonic accommodation and nearwork-induced accommodative adaptation in myopes and non-myopes. The first study of the thesis was based on observations of Gilmartin and Winfield (1995) which suggested that a deficit in the sympathetic inputs to the ciliary muscle may be associated with a propensity for myopia development. The effect of ß-antagonism with timolol application on accommodation characteristics was studied in different refractive error groups. Our results support the previous findings that a deficit of sympathetic facility during nearwork was not a feature of late-onset myopia. However it was found that classifying myopes according to stability of their myopia and their ethnic background was important and this allowed differentiation between accommodation responses and characteristics of the ciliary muscle autonomic inputs, with the greatest difference observed between Caucasian stable myopes and Asian progressing myopes. Progressing myopes, particularly those with an Asian background, demonstrated enhanced susceptibility to nearwork-induced accommodative adaptation and this was suggested to result from a possible parasympathetic dominance and a relative sympathetic deficit to the ciliary muscle. In contrast, stable myopes, particularly those with an Asian background, demonstrated minimal accommodation changes following nearwork (counter-adaptation in some cases), and increased accommodative adaptation with ß-antagonism, suggesting sympathetic dominance as the possible autonomic accommodation control profile. As ethnic background was found to be an important factor, a similar study was also conducted in a group of Hong Kong Chinese children to investigate if enhanced susceptibility to nearwork-induced changes in accommodation may explain in part the high prevalence of myopia in Hong Kong. Despite some minor differences in methodology between the two studies, the Hong Kong stable myopic children demonstrated counter-adaptive changes and greater accommodative adaptation with timolol, findings that were consistent with those of the adult Asian stable myopes. Both Asian progressing myopic children and adults also showed greater accommodative adaptation than the stable myopes and similar response profiles following ß-adrenergic antagonism. Thus a combination of genetically predetermined accommodation profiles that confer high susceptibility and extreme environmental pressures is a likely explanation for the increase in myopia over the past decades in Asian countries. The hypothesis that a sympathetic deficit is linked to myopia was also investigated by comparing the effect of â-stimulation with salbutamol, a ß-agonist, on accommodation with that of ß-antagonism using timolol. It was hypothesized that salbutamol would have the opposite effect of timolol, and that it would have a greater effect on subjects who demonstrated greater accommodative adaptation effects, i.e. the progressing myopes, compared to those who showed minimal changes in accommodation following nearwork. Consistent with the hypothesis, the effect of sympathetic stimulation with salbutamol application was only evident in the progressing myopes whom we hypothesized may have a parasympathetic dominance and a relative sympathetic deficit type of autonomic imbalance while it did not further enhance the rapid accommodative regression profile demonstrated by the stable myopes. Characteristics of the convergence system and the interaction between accommodation and convergence were also investigated in the Hong Kong children. No significant differences in response AC/A ratios between the emmetropic, stable and progressing myopic children were found and it was concluded that elevated AC/A ratios were not associated with higher myopic progression rate in this sample of Hong Kong children. However, ß-adrenergic antagonism with timolol application produced a greater effect on accommodative convergence (AC) in stable myopic children who presumably have a more adequate, robust sympathetic input to the ciliary muscle, but had little effect on AC of progressing myopic children. This finding again points to the possibility that the autonomic control of the accommodation and convergence systems may be different between stable and progressing myopia. The primary contribution of this study to the understanding of myopia development is that differences in the autonomic control of the ciliary muscle may be responsible for producing anomalous accommodation responses. This could have significant impact on retinal image quality and thus results in myopia development. This knowledge may be incorporated into computer models of accommodation and myopia development and provides scope for further investigation of the therapeutic benefits of autonomic agents for myopia control.

Axonal degeneration is a major pathological feature of the central nervous system (CNS) inflammatory demyelinating disease multiple sclerosis (MS). This axonal degeneration has major consequences, as functional axonal regeneration in the CNS is largely absent. Cumulative axonal degeneration is the likely cause of the majority of progressive MS-related disability, and therefore, the need for novel neuroprotective therapies for MS exists. Experimental autoimmune encephalomyelitis (EAE), an animal model of MS pathology, also produces axonal injury. In particular, the optic nerve and spinal cord are key sites of neuroinflammation in mouse EAE. By utilizing this model, the short term and long term effects of the putative neuroprotective cytokine, leukaemia inhibitory factor (LIF), were investigated in the optic nerve and spinal cord utilising a number of outcome measures of axonal dysfunction. These included MRI measures of water diffusivity along (ADC ||) and across (ADC┴) the optic nerves, serum levels of phosphorylated neurofilament heavy chain subunit (pNF-H) and histological morphometric measures. LIF treatment reduced EAE grade and pNF-H plasma levels, decreased ADC┴, but had no effect on ADC ||, axon counts or inflammatory infiltration. / In contrast, genetic deletion of LIF and its sister cytokine ciliary neurotrophic factor (CNTF), not only increased EAE grade and pNF-H levels, but also decreased optic nerve ADC|| and optic nerve and spinal cord axon densities. After reviewing current literature, we hypothesize that the target cell for endogenously upregulated LIF in EAE may be the neuron or axon, whereas the target cell for exogenously administered therapeutic LIF may be another cell type, possibly infiltrating macrophages and activated microglial cells. LIF antagonist treatment did not have any affect on EAE grade, pNF-H levels or MRI parameters. This lack of effect may be due to the inability of the LIF antagonist to enter the CNS, supporting the hypothesis that endogenous LIF has a centrally acting mechanism.

Orientador: Antonio Carlos Boschero / Dissertação (mestrado) - Universidade Estadual de Campinas, Instituto de Biologia / Made available in DSpace on 2018-08-17T15:59:13Z (GMT). No. of bitstreams: 1 Santos_GustavoJorge_M.pdf: 2550633 bytes, checksum: 914361a57f5efd5794bcee99ff8ce2a4 (MD5) Previous issue date: 2011 / Resumo: Diabetes Mellitus (DM) é uma síndrome metabólica, de etiologia múltipla, caracterizada por hiperglicemia crônica, decorrente da falta de insulina, às vezes, associado à resistência dos tecidos periféricos a esse hormônio. O DM1 é caracterizado pela infiltração de macrófagos e linfócitos do tipo T-CD4+ e T-CD8+ no pâncreas, devido a uma falha do reconhecimento no sistema autoimune, que desencadeiam processos inflamatórios com liberação de óxido nítrico (NO), de radicais livres e de citocinas tais como: interleucina-1? (IL-1 ?) e Interferon- ? (IFN- ?). Essas citocinas pró-inflamatórias ativam mecanismos que levam a morte celular por apoptose, com perda da massa funcional das células beta. Esses mecanismos podem ser reproduzidos in-vitro pela exposição de células beta a essas citocinas ou à Aloxana. O CNTF é uma citocina de sobrevivência neuronal, e em células beta age sobre o controle glicêmico inibindo secreção de insulina e promovendo sobrevivência de ilhotas. A AMPK é uma proteína quinase que em células beta atua como um sensor do estado energético celular e, quando as concentrações intracelulares de ATP diminuem, a AMPK é ativada estimulando geração de ATP e inibindo o consumo desse nucleotídeo. Em ilhotas, a AMPK desempenha função importante na regulação da secreção de insulina sendo que a inibição de AMPK protege as células beta da apoptose mediada por citosinas (IL-1? e IFN-?) ou induzidas por células T do tipo CD8+ e CD4+. Diante do exposto, conclui-se que CNTF e AMPK desempenham funções importantes e correlatas nas células beta pancreáticas e, podem ser considerados alvos terapêuticos para o tratamento do DM tipo I. Contudo, a interação entre esses dois fatores (CNTF e AMPK) em células secretoras de insulina permanece desconhecida. Assim, o objetivo deste trabalho foi investigar a possível papel da interação entre CNTF e AMPK na morte celular induzida pelos agentes diabetogênicos IL-1? e Aloxana. Nossos resultados indicaram que: A Aloxana e a IL-1? dependem da via da AMPK para induzir apoptose em células MIN6; O CNTF modula a via da AMPK em células MIN6 e ilhotas de camundongos Swiss neonato. O CNTF foi capaz de impedir a morte celular induzida por Aloxana e por IL-1? através da downregulation da via da AMPK em células MIN6. / Abstract: Diabetes Mellitus (DM) is a metabolic syndrome of multiple etiologies, resulting from the lack of insulin sometime associated with an increase in the resistance to the hormone by insulin-target tissues. DM1 is characterized by the infiltration of macrophages and T-type CD4 + and T-CD8 + cells in the pancreas, due to a failure of the autoimmune system, causing inflammation and leading to the release of nitric oxide (NO), free radicals, and cytokines such as interleukin-1? (IL-1?) and interferon-? (IFN-?). These pro-inflammatory cytokines activate pro-apoptotic mechanisms, triggering beta cell death apoptosis, leading to a loss in functional beta cell mass. These mechanisms may be reproduced in vitro with exposure of beta cells to pro-inflammatory cytokines such as IL-1? or to Alloxan. The cytokine CNTF is a neuronal survival factor. Besides, CNTF modulates glycemia, inhibiting insulin secretion and promoting islet cells survival. AMPK is a protein kinase that acts on pancreatic beta cells as a sensor of the cellular energy state, and is activated when the cellular ATP concentrations decrease, stimulating ATP generation and inhibiting ATP consumption. In islets, AMPK plays an important role in regulating insulin secretion and inhibition of AMPK protects beta cells from apoptosis mediated by either cytokines (IL-1? and IFN-?) and/or induced T cell CD8 + and CD4 +. AIMS: Given that, both CNTF and AMPK play important role in beta cells and may be used as therapeutic targets for the treatment of DM1. However, the interaction between these two factors (CNTF and AMPK) in pancreatic beta cells remains unknown. Thus, the objective of this work was to investigate the relationship between interaction of AMPK and CNTF on pancreatic beta cell death, induced by Alloxan or IL-1?. Our results indicated that both Alloxan and IL-1? are dependent of AMPK pathway to induce apoptosis in MIN6 cells; CNTF inhibits AMPK pathway in MIN6 cells as well as in islets of newborn Swiss mice; CNTF prevents beta cell death, induced by Alloxan and IL-1?, through downregulation of AMPK pathway in MIN6 cells. / Mestrado / Fisiologia / Mestre em Biologia Funcional e Molecular

Coordenação de Aperfeiçoamento de Pessoal de Nível Superior / The intensive and disordered use of the soil has been taking many ecosystems to the degradation, besides the ciliary ones that, in spite of they be considered by the legislation as Permanent Preservation Areas, they have been suffering constant antropic pressures and every time larger, and as consequence occur its degradation. In this wey, the present study has as objective evaluate the antropic pressure impacts on the ciliary forest of an urban-rural tributary on the watershed of the Vacacaímirim, Santa Maria - RS, seeking to diagnose the degradation degree and to indicate a subsequent recovery. The study was accomplished on the ciliary forest of the Lagoão do Ouro stream, tributary of 3th order of the Vacacaí-mirim river. The fountain is located in urban area, traveling several towns, the Campus of UFSM, besides an extensive rural area. The predominant soils are classified as: Argissolos Bruno-Acinzentados, Vermelho-Amarelos and Vermelhos , besides Planossolos Háplicos , being these of low natural fertility and quite susceptible to water erosion. Studies of surveying of the degradation were accomplished, in 12 sampling plots of 300 m2 each demarcated along of the margin of the Lagoão do Ouro stream, with the collection of the following information: species floristic surveying of natural occurrence and its regeneration potential, presence of exotic species, evaluation of the bank of soil seeds in two collection times, soil use, soil covering on surface level and by the canopy of the trees and surveying of the erosion (current and potential). The main soil chemical properties (analysis completes of routine) and physics (density, porosity, texture and structure) were appraised also, in each one of the sampling plots. The correlations of the environmental characteristics and antropics with the current erosion and potential erosive were unfolded in direct and indirect effects. After evaluation diagnostic of the degradation, the experiment of recovery of the ciliary ecosystem was preceded, through the revegetation with native species and natural regeneration, besides the soil bioengineering use for erosion control in the stream margin. The floristic surveying showed there to be degradation of the ciliary forest, evidenced by the low number of native species, for the bad distribution of the species in the different strata and for the great presence of exotic species. Just the species that presented larger frequency and that appear in the three floristic strata appraised possess potential of natural regeneration. The bank of soil seeds also showed there to be degradation of the ciliary forest, evidenced by the low number of native arboreal species and for the great number of exotic species and herbaceous invaders. Just the native arboreal species observed in the evaluation and that presented larger frequency possesses potential of regeneration natural through bank of soil seeds. There is correlation of the environmental and antropic factors with the erosion of the soil and with the erosive potential. Antropic interventions are necessary to recovery of the ciliary forest of the Lagoão do Ouro stream, in what concerns to the introduction of species that don't possess potential of natural regeneration and to soil bioengineering works in the margins with erosion more accentuated. The native species used in the recovery of the ciliary forest are appropriate for such an objective, presenting mortality percentage inside of acceptable limits and good initial growth. The soil bioengineering is a cheap and efficient technique in the control of the erosion of the margins of water courses. The experimental methodology of recovery, adopted, can be expanded in the Vacacaí-mirim watershed, mainly in the places where the stream margins are in advanced degradation level. / O uso intensivo e desordenado do solo tem levado muitos ecossistemas à degradação, inclusive os ciliares que, apesar de serem considerados pela legislação como Áreas de Preservação Permanente, têm sofrido pressões antrópicas constantes e cada vez maiores, tendo como consequência a sua degradação. Sendo assim, o presente estudo tem como objetivo avaliar os impactos da pressão antrópica sobre a mata ciliar de um tributário urbano-rural na microbacia hidrográfica do Vacacaímirim, Santa Maria - RS, visando diagnosticar seu grau de degradação e indicar uma posterior recuperação. O estudo foi realizado na mata ciliar da Sanga Lagoão do Ouro, tributário de 3ª ordem do rio Vacacaí-mirim. Sua nascente localiza-se em área urbana, percorrendo várias vilas, o Campus da UFSM, além de uma extensa área rural. Os solos predominantes classificam-se como: Argissolos Bruno-Acinzentados, Vermelho-Amarelos e Vermelhos, além de Planossolos Háplicos, sendo estes de baixa fertilidade natural e bastante suscetíveis a erosão hídrica. Foram realizados estudos de levantamento da degradação, em 12 parcelas de 300 m2 cada, demarcadas ao longo da margem da Sanga Lagoão do Ouro, através da coleta das seguintes informações: levantamento florístico de espécies de ocorrência natural e seu potencial de regeneração, presença de espécies exóticas, avaliação do banco de sementes do solo em duas épocas de coleta, uso do solo, cobertura do solo em nível de superfície e de copada e levantamento da erosão (atual e potencial). Foram também avaliadas as principais propriedades químicas (análise completa de rotina) e físicas (densidade, porosidade, textura e estrutura) do solo, em cada uma das parcelas. As correlações das características ambientais e antrópicas com a erosão atual e potencial erosivo foram desdobradas em efeitos diretos e indiretos. Após avaliação diagnóstica da degradação, procedeu-se com experimento de recuperação do ecossistema ciliar, através da revegetação com espécies nativas e regeneração natural, além do uso de bioengenharia de solos para controle da erosão na margem da sanga. O levantamento florístico mostrou haver degradação da mata ciliar, evidenciada pelo baixo número de espécies nativas, pela má distribuição das espécies nos diferentes estratos e pela grande presença de espécies exóticas. Possuem potencial de regeneração natural apenas as espécies que apresentaram maior frequência e que aparecem nos três estratos florísticos levantados. O banco de sementes do solo também mostrou haver degradação da mata ciliar, evidenciada pelo baixo número de espécies arbóreas nativas e pelo grande número de espécies exóticas e herbáceas invasoras. Possuem potencial de regeneração natural via banco de sementes do solo apenas as espécies arbóreas nativas observadas na avaliação e que apresentaram maior frequência. Há correlação dos fatores ambientais e antrópicos com a erosão do solo e com o potencial erosivo. São necessárias intervenções antrópicas na recuperação da mata ciliar da Sanga Lagoão do Ouro, no que concerne à introdução de espécies que não possuem potencial de regeneração natural e a obras de bioengenharia de solos nas margens com erosão mais acentuada. As espécies nativas usadas na recuperação da mata ciliar são adequadas para tal objetivo, apresentando porcentagem de mortalidade dentro de limites aceitáveis e bom crescimento inicial. A bioengenharia de solo é uma técnica barata e eficiente no controle da erosão das margens de cursos d água. A metodologia experimental de recuperação adotada pode ser expandida na microbacia hidrográfica do Vacacaí-mirim, principalmente nos locais onde as margens da sanga se encontram em estágio avançado de degradação.

Primary ciliary dyskinesia is a rare hereditary disorder with impairment of cilia characterized by chronic cough with sputum, bronchiectasis or pneumonia. Regular exercise training should affect pulmonary function, promote mucociliary clearance and improve quality of life. The aim of this thesis is to compare the aerobic fitness of patients with PCD with a control group.