Mitochondrial dysfunction in fatty acid β-oxidation disorders

Mitochondria are central organelles for cell survival with particular relevance in energy production and signalling, being mitochondrial fatty acid β–oxidation (FAO) one of the metabolic pathways harboured in this organelle. FAO disorders (FAOD) are among the most well studied inborn errors of metabolism, mainly due to their impact in health. Nevertheless, some questions remain unsolved, as their prevalence in certain European regions and how pathophysiological determinants combine towards the phenotype. Analysis of data from newborn screening programs from Portugal and Spain allowed the estimation of the birth prevalence of FAOD revealing that this group of disorders presents in Iberia (and particularly in Portugal) one of the highest European birth prevalence, mainly due to the high birth prevalence of medium chain acyl-CoA dehydrogenase deficiency. These results highlight the impact of this group of genetic disorders in this European region. The characterization of mitochondrial proteome, from patients fibroblasts with FAOD, namely multiple acyl-CoA dehydrogenase deficiency (MADD) and long chain acyl-CoA dehydrogenase deficiency (LCHADD), provided a global perspective of the mitochondrial proteome plasticity in these disorders and highlights the main molecular pathways involved in their pathogenesis. Severe MADD forms show an overexpression of chaperones, antioxidant enzymes (MnSOD), and apoptotic proteins. An overexpression of glycolytic enzymes, which reflects cellular adaptation to energy deficiency due to FAO blockage, was also observed. When LCHADD fibroblasts were analysed a metabolic switching to glycolysis was also observed with overexpression of apoptotic proteins and modulation of the antioxidant defence system. Severe LCHADD present increased ROS alongside with up regulation of MnSOD while moderate forms have lower ROS and down-regulation of MnSOD. This probably reflects the role of MnSOD in buffering cellular ROS, maintain them at levels that allow cells to avoid damage and start a cellular response towards survival. When ROS levels are very high cells have to overexpress MnSOD for detoxifying proposes. When severe forms of MADD were compared to moderate forms no major differences were noticed, most probably because ROS levels in moderate MADD are high enough to trigger a response similar to that observed in severe forms. Our data highlights, for the first time, the differences in the modulation of antioxidant defence among FAOD spectrum. Overall, the data reveals the main pathways modulated in FAOD and the importance of ROS levels and antioxidant defence system modulation for disease severity. These results highlight the complex interaction between phenotypic determinants in FAOD that include genetic, epigenetic and environmental factors. The development of future better treatment approaches is dependent on the knowledge on how all these determinants interact towards phenotype.!

Competitive destination analysis in Southern European countries

Purpose – The purpose of this paper is to analyse the competitiveness of the European Union Member States of Southern Europe (France, Greece, Italy, Portugal and Spain) as tourist destinations for European Union Member States of Central and Northern Europe (Austria, Belgium, Denmark, Finland, Germany, The Netherlands, Ireland, Sweden and the UK). Design/methodology/approach – Application of the market share analysis tool, initially developed by Faulkner, using secondary data from Eurostat – statistical office of the European communities. Findings – The results obtained show that France, Greece, Italy, Portugal and Spain present distinct levels of competitiveness for the various generating countries, with changes having occurred in the period between 1999 and 2007. Originality/value – The paper offers refreshment of Faulkner's tool and an insight into tourist flows in Europe as a tool for tourism and hospitality managers.

Loss of Aggressiveness of Phytophthora cinnamomi (Beta-Cinnamomin Silenced Strain) in the Infection of Castanea sativa

Several forest species are severely affected by Phytophthora cinnamomi. The contribution of this oomycete to forest decline and dieback has been broadly reported. In particular, it is consensual that it is the causal agent of ink disease in Castanea sativa. It has been associated with the severe decline of Quercus species, namely the Q. suber and Q. ilex dieback in Portugal and Spain, and has been responsible for the infection of numerous native species and crops. This pathogen persists in the soil or on plant material in the form of chlamydospores allowing the infection of living root tissues when environmental conditions are favorable. © Microscopy Society of America 2012.

Variety of Media Systems in Third-Wave Democracies

The number of comparative studies in the field of political communication increased considerably after Daniel Hallin and Paolo Mancini's publication of comparing Media systems. In this book, four dimensions are used to distinguish between the media environments in western countries around the year 2000: press market development, parallelism between parties and media outlets, state intervention in the realm of media, and levels of journalist professionalization. The authors conclude that in western Europe and North America three types of media systems coexisted: a polarized pluralist model (in southern Europe), a democratic corporatist model (in scandinavia and some western European countries), and a liberal model (Canada, USA, Ireland, and the UK). Within this framework, both Portugal and Spain are described as polarized pluralist media systems, given their weak press markets and low patterns of journalistic professionalization, as well as strong state intervention in the realm of media and parallelism between media outlets and political parties.

Les régimes providentiels à l'ère post-industrielle: reconfiguration ou résilience ?

L'approche des régimes providentiels élaborée par Esping-Andersen dans les années 1990 présente un grand intérêt dans l'appréhension des lignes directrices de la protection sociale: elle met en relief le jeu d'interrelations entre différents piliers de bien-être (l'État, le marché et la famille) dans la production et la distribution du bien-être. Esping-Andersen a identifié à cet effet trois types de régimes providentiels: les régimes social-démocrate, libéral et conservateur (qui correspondent respectivement aux pays nordiques, anglo-saxons et d'Europe continentale) auxquels certains observateurs ont rajouté le type latin, qui correspond aux pays d'Europe du Sud (Espagne, Grèce, Italie, Portugal). Ces régimes se sont consolidés dans un contexte industriel d'après-guerre et on peut se demander s'ils peuvent tenir la route à l'ère post-industrielle. En effet, le jeu d'interrelations entre l'État, le marché et la famille est appelé à connaître des reconfigurations, pour répondre plus adéquatement aux divers risques nouveaux qu'encourent les individus. La résilience des régimes providentiels est donc mise à l'épreuve et doit composer avec de nouvelles réalités sociales et économiques qui peuvent l'amener vers des terrains qui lui étaient jusque là inconnus. Notre examen s'intéresse à l'évolution des régimes providentiels à l'ère post-industrielle. Nous cherchons à caractériser et à différencier vingt pays de l'OCDE sur la base d'indicateurs quantitatifs de dépenses publiques et de situations socio-économiques couvrant la période de 1985 aux années 2000. Au moyen de l'analyse factorielle des correspondances et de l'analyse de classification hiérarchique, nous avons pu dégager des regroupements de pays qui correspondent assez fidèlement à la typologie d'Esping-Andersen et à ses développements subséquents et à mettre en relief différentes formes de protection sociale à l'ère post-industrielle: soit "l'activation laissée au marché" dans les pays anglo-saxons, "l'activation comme projet en devenir" dans les pays d'Europe continentale et, finalement, "la faible référence à l'activation" dans les pays d'Europe du Sud.

The dark side of the Mediterranean : expression of fear from the inquisition to the present

Inside the stones of its most famous buildings, Évora keeps mysteries and secrets which constitute the most hidden side of its cultural identity. A World Heritage site, this town seems to preserve, in its medieval walls, a precious knowledge of the most universal and ancient human emotion: fear. Trying to transcend many of its past and future fears, some of its historical monuments in Gothic style were erected against the fear of death, the most terrible of all fears, which the famous inscription, in the Bones Chapel of the Church of São Francisco, insistently reminds us, through the most disturbing words: “Nós ossos que aqui estamos pelos vossos esperamos”. If the first inquisitors worked in central Europe (Germany, northern Italy, eastern France), later the centres of the Inquisition were established in the Mediterranean regions, especially southern France, Italy, Portugal, and Spain. Consequently, the roots of fear in Évora are common to other towns, where the Inquisition developed a culture of fear, through which we can penetrate into the dark side of the Mediterranean, where people were subjected to the same terrifying methods of persecution and torture. This common geographical and historical context was not ignored by one of the most famous masters of American gothic fiction, Edgar Allan Poe. Through the pages of The Pit and the Pendulum, readers get precise images of the fearful instruments of terror that were able to produce the legend that has made the first grand inquisitor, Tomas de Torquemada, a symbol of ultimate cruelty, bigotry, intolerance, and religious fanaticism, which unfortunately are still the source of our present fears in a time when religious beliefs can be used again as a motif of war and destruction. As Krishnamurti once suggested, only a fundamental realization of the root of all fear can free our minds.

Marketing de moda : a percepção da marca Zara

No contexto complexo dos mercados de consumo, entender e dominar todas as bases e práticas de marketing tornou-se essencial para a sobrevivência de qualquer marca/empresa. No mercado global da moda, a transformação ainda é mais acelerada em resultado da evolução das necessidades e desejos dos consumidores e do fácil acesso à informação. A partir da pesquisa bibliográfica especificamos e analisamos complexo mundo da moda, o conceito de marketing de moda e os factores estratégicos necessários para compreender a sobrevivência e crescimento de uma marca de moda no mercado actual, compreendendo a capacidade do sector da moda em adaptar-se às constantes mudanças do mercado e da sociedade, sem deixar de corresponder às expectativas e necessidades dos consumidores. Aprofundaremos esta problemática explorando o caso da marca de moda Zara, reconhecida e aplaudida pelo seu êxito global, realizando um estudo de caso, com o objectivo de compreender a percepção dos diferentes consumidores em resultado das estratégias de comunicação e de marketing da marca. O do nosso estudo empírico é compreender se a marca age correctamente ou não ao aplicar as mesmas estratégias em dois países distintos, Portugal e Espanha, que considera como domésticos. Em resultado do estudo realizado, constatámos que apesar da percepção da marca ser algo semelhante, as divergências no que toca no factor preço e influência dele, misturado com as diferenças socioeconómicas e de disponibilidade económica de ambos os países, provocam uma discrepância de opiniões e percepções.Concluímos a nossa dissertação com a apresentação de sugestões relativas ao marketing mix que visam um melhor ajustamento entre a percepção da imagem de marca da Zara e a estratégia de marketing seguida por esta marca de moda.

Intra-Euro rebalancing is inevitable, but insufficient. Bruegel Policy Contribution 2012/15, August 2012

Greece, Portugal and Spain face a serious risk of external solvency due to their close to minus 100 percent of GDP net negative international investment positions, which are largely composed of debt. The perceived inability of these countries to rebalance their external positions is a major root of the euro crisis. Intra-euro rebalancing through declines in unit labour costs (ULC) in southern Europe, and ULC increases in northern Europe should continue, but has limits because: The share of intra-euro trade has declined. Intra-euro trade balances have already adjusted to a great extent. The intra-euro real exchange rates of Greece, Portugal and Spain have also either already adjusted or do not indicate significant appreciations since 2000. There are only two main current account surplus countries, Germany and the Netherlands. A purely intra-euro adjustment strategy would require too-significant wage increases in northern countries and wage declines in southern countries, which do not seem to be feasible. Before the crisis, the euro was significantly overvalued despite the close-to balanced current account position. The euro has depreciated recently, but more is needed to support the extra-euro trade of southern euro-area members. A weaker euro would also boost exports, growth, inflation and wage increases in Germany, thereby helping further intra-euro adjustment and the survival of the euro.

Smart choices for growth. Bruegel Policy Contribution 2012/21, November 2012

Recovery in Greece, Italy, Portugal and Spain is held back in part by structural barriers. Overcoming these requires structural reform and public investment. Given the limited availability of political and financial capital, prioritising reform efforts and spending is important, but difficult. The different success factors for individual sectors are complementary. Using the example of the high-tech industry, we make the case that only investing in one success factor (eg broadband infrastructure) without having a sufficient endowment of others (eg education) is unlikely to make the sector successful. One consequence of the complementarity of the different success factors is that public investment and reform efforts should be fine-tuned in order to match the endowment of other factors. This might imply an increase in efforts to tackle several structural barriers at the same time, but it might also imply reducing investment in less promising fields. This in turn requires strategic thinking about whether it is worthwhile pursuing development strategies that require investment in many success factors but that do not promise much success. Such a strategic approach to public investment and reform efforts might make the allocation of scarce public financial and political capital more efficient.

Análise econômica das políticas de incentivos à cultura no Brasil

O Brasil conta hoje com uma legislação que o coloca seguramente entre os melhores países, senão o melhor, para se realizar projetos culturais. Ao menos na teoria. Essa dissertação tem como objetivo trilhar o caminho das leis de incentivo, desde a sua criação, com a Lei Sarney até a Lei Rouanet, analisando inclusive as leis estaduais e municipais, que também surgiram como respostas a descontinuidades no processo. O mercado cultural brasileiro é caracterizado por externalidades que dificultam sua viabilidade, fazendo necessária a intervenção governamental. A política cultural brasileira tem como principal instrumento as leis de incentivo à cultura, que utilizam a renúncia fiscal para atrair o capital privado. São formalizadas parcerias onde as empresas privadas patrocinam projetos de interesse do governo e, como contrapartida, recebem o direito de deduzir esse valor, integral ou parcialmente, de seus impostos. Contudo, esse modelo de contrato de parceria é mal formulado, e traz perdas para a sociedade. O risco é assumido integralmente pelo Estado, o que acarreta problemas sérios de Moral Hazard. Além disso, devido às diferentes características dos projetos, o modelo acaba também por gerar problemas de Seleção Adversa. Para uma melhor comparação e análise da política cultural brasileira, foram levantados casos internacionais - Estados Unidos, Inglaterra, Portugal e Espanha. Levando-se em conta as análises e críticas levantadas, serão sugeridas alternativas para o modelo de contrato adotado pelo governo para incentivo à cultura, e formas alternativas de financiamento ao setor cultural, de forma a assegurar um melhor retorno para a sociedade sem deixar de cumprir o papel de fomentar o setor e corrigir as externalidades presentes.

Um imperialismo possível: fluxos migratórios e estratégias colonialistas na Europa mediterrânea (1870-1914)

Este artigo analisa algumas das diferentes implicações do fluxo migratório nos principais países fornecedores de contingentes, durante o período conhecido como new emigration: Itália, Espanha e Portugal. Considerando suas especificidades, o objetivo é identificar as estratégias de cada um para transformar esse movimento em fator de desenvolvimento econômico: conquista de mercados, criação e fomento de colônias, incorporação e tutela das remessas dos emigrados. Reino em formação e maior fonte de emigrantes, a Itália seguiu naturalmente os caminhos que identificavam a emigração como um dos agentes de prosperidade da nação, contrastando com Portugal e Espanha.

Corpo e fronteira: o diário de Samuel Fritz e a conquista do espaço amazônico

Considering that the Jesuitical tradition which Father Samuel Fritz belonged, has a clear political and institutional dimension that reveals itself in the missionary initiative placed since the Trento Council, his journal is a experience story as missionary at Maynás region during the period from 1686 until 1725. In his narrative, a series of data related to the conquer of Amazonia, conflicts among the Iberic Kingdoms and french, dutches and british, transformation of culture and space close the period of the Madrid Deal. I´ll explore the men and space relationship, in this case, the missionary in his special practice, therefore an effective and geometrical politic for border control was only applied at 1750 with reformist governments and that Amazônia was, until now, an object of autonomous initiatives, not being until now a priority focused state politics action like the ones in the central regions (silver mines) and that the missionary action of Samuel Fritz represented ant that moment represented the most important border advance to the Spanish Kingdom, coinciding with the end of the borders previously set in Madrid and Santo Idelfonso, I´ll put the question of how and with which politics the experience of Fritz in Maynás could represent an advance about Amazônia space. Then I´ll approach the problem about three aspects that are chapters: The first one was focused to the Iberic Kingdoms atlantic politics and the internal geopolitical relationships they created as the centre and the border emerging a new order; in the second chapter I studied the special transformation cause by the encounter and conflicts between the Indian and European order generating a new organization; in the third chapter I´ll examined the political border of the state and the emergency of the missionary body as an institution, with the tradition and missionary action as support, or not, to the exploration of the east border of Spanish America influencing the delimitation process of the border between Portugal and Spain

Genetic population data of 12 STR loci of the PowerPlex (R) Y system in the state of São Paulo population (Southeast of Brazil)

Allele frequency distributions and population data for 12 Y-chromosomal short tandem repeats (STRs) included in the PowerPlex (R) Y Systems (Promega) were obtained for a sample of 200 healthy unrelated males living in S (a) over tildeo Paulo State (Southeast of Brazil). A total of 192 haplotypes were identified, of which 184 were unique and 8 were found in 2 individuals. The average gene diversity of the 12 Y-STR was 0.6746 and the haplotype diversity was 0.9996. Pairwise analysis confirmed that our population is more similar with the Italy, North Portugal and Spain, being more distant of the Japan. (c) 2007 Elsevier B.V. All rights reserved.

Análise discursiva de dicionários bilíngues Português-Espanhol

Pós-graduação em Estudos Linguísticos - IBILCE

Prevalence of celiac disease among blood donors in Sao Paulo - the most populated city in Brazil

OBJECTIVE: Celiac disease is a permanent enteropathy caused by the ingestion of gluten, which leads to an immune-mediated inflammation of the small intestine mucosa. The prevalence of celiac disease varies among different nations and ethnic backgrounds, and its diversity is determined by genetic and environmental factors. Sao Paulo city is one of the largest cities in the world, with a vast population and an important history of internal migratory flow from other Brazilian regions, as well as immigration from other, primarily European, countries, resulting in significant miscegenation. The aim of the present study was to estimate the prevalence of adults with undiagnosed celiac disease among blood donors of Sao Paulo by collecting information on the ancestry of the population studied. METHODS: The prevalence of celiac disease was assessed by screening for positive IgA transglutaminase and IgA endomysium antibodies in 4,000 donors (volunteers) in the Fundacao Pro-Sangue Blood Center of Sao Paulo, Sao Paulo, Brazil. The antibody-positive subjects were asked to undergo a small bowel biopsy. RESULTS: Of the 4,000 subjects, twenty-four had positive tests, although both antibody tests were not always concordant. For example, ten subjects were positive for IgA tissue transglutaminase only. In twenty-one positive patients, duodenal biopsies were performed, and the diagnosis of celiac disease was confirmed in fourteen patients (Marsh criteria modified by Oberhuber). In this group, 67% claimed to have European ancestry, mainly from Italy, Portugal and Spain. CONCLUSION: The prevalence of celiac disease is at least 1: 286 among supposedly healthy blood bank volunteers in Sao Paulo, Brazil.