Screening of ARHSP-TCC Patients Expands the Spectrum of SPG11 Mutations and Includes a Large Scale Gene Deletion

Autosomal recessive spastic paraplegia with thinning of corpus callosum (ARHSP-TCC) is a complex form of HSP initially described in Japan but subsequently reported to have a worldwide distribution with a particular high frequency in multiple families from the Mediterranean basin. We recently showed that ARHSP-TCC is commonly associated with mutations in SPG11/KIAA1840 on chromosome 15q. We have now screened a collection of new patients mainly originating from Italy and Brazil, in order to further ascertain the spectrum of mutations in SPG11, enlarge the ethnic origin of SPG11 patients, determine the relative frequency at the level of single Countries (i.e., Italy), and establish whether there is one or more common mutation. In 25 index cases we identified 32 mutations; 22 are novel, including 9 nonsense, 3 small deletions, 4 insertions, 1 in/del, 1 small duplication, 1 missense, 2 splice-site, and for the first time a large genomic rearrangement. This brings the total number of SPG11 mutated patients in the SPATAX collection to 111 cases in 44 families and in 17 isolated cases, from 16 Countries, all assessed using homogeneous clinical criteria. While expanding the spectrum of mutations in SPG11, this larger series also corroborated the notion that even within apparently homogeneous population a molecular diagnosis cannot be achieved without full gene sequencing. (C) 2008 Wiley-Liss, Inc.

Electronic properties of a methane-water solution

Electronic properties of a methane-water solution were investigated by a sequential quantum mechanical/molecular dynamics approach. Upon hydration methane acquires an induced dipole moment of similar to 0.5 +/- 0.2 D. This is related to polarisation effects and to weak methane-water hydrogen bond interactions. From gas phase to solution, the first vertical excitation and ionisation energies of methane are red-shifted by 0.45 +/- 0.25 and 0.87 +/- 0.40 eV, respectively. We also report results for the dynamic polarisability of methane in water. In comparison with water, no difference was found for the average monomeric dipole moment of water molecules in close interaction with methane. (c) 2011 Elsevier B.V. All rights reserved.

Osmotin from Calotropis procera latex: New insights into structure and antifungal properties

This study aimed at investigating the structural properties and mechanisms of the antifungal action of CpOsm, a purified osmotin from Calotropis procera latex. Fluorescence and CD assays revealed that the CpOsm structure is highly stable, regardless of pH levels. Accordingly, CpOsm inhibited the spore germination of Fusarium solani in all pH ranges tested. The content of the secondary structure of CpOsm was estimated as follows: alpha-helix (20%), beta-sheet (33%), turned (19%) and unordered (28%). RMSD 1%. CpOsm was stable at up to 75 degrees C, and thermal denaturation (T(m)) was calculated to be 77.8 degrees C. This osmotin interacted with the negatively charged large unilamellar vesicles (LUVs) of 1-palmitoyl-2-oleoyl-sn-glycero-3-phospho-rac-1-glycerol (POPG), inducing vesicle permeabilization by the leakage of calcein. CpOsm induced the membrane permeabilization of spores and hyphae from Fusarium solani, allowing for propidium iodide uptake. These results show that CpOsm is a stable protein, and its antifungal activity involves membrane permeabilization, as property reported earlier for other osmotins and thaumatin-like proteins. (C) 2011 Elsevier B.V. All rights reserved.

Cearania heterophylla gen. nov et sp nov., a fossil gymnosperm with affinities to the Gnetales from the Early Cretaceous of northern Gondwana

Vegetative and fertile shoots of a shrub-like seed plant from the late Aptian Crato Formation of Brazil are described as Cearania heterophylla Kunzmann, Mohr and Bernardes-de-Oliveira, gen. nov. et sp. nov. Anatomical details of the axes, epidermal features and separate ovulate and pollen producing organs indicate the gymnospermous nature of this plant. The vascular tissue of the axes includes tracheids with bordered pits and fiber tracheids. Vegetative shoots comprising at least three branching orders bear opposite-decussately arranged ovate to lanceolate, dorsiventrally flattened, parallelodromous, rather thick leaves that vary tremendously in size. The amphistomatic leaves bear (brachy-)paracytic stomatal complexes arranged in simple longitudinal files. The ovulate structure is interpreted as a terminally attached single globular ovule/seed surrounded by at least five to six lanceolate bracts. A terminally attached pollen-cone like structure grows on a lateral leafy shoot. The unusual character combination may indicate that the fossils belong to a hitherto unknown group with affinities to ephedroid Gnetales. Sterile shoots formerly often described as Podozamites, Nageiopsis or Lilites that are at least partly congeneric with C. heterophylla Kunzmann, Mohr and Bernardes-de-Oliveira, gen. nov. et sp. nov. had a wide geographic distribution during the Early Cretaceous. (C) 2009 Elsevier B.V. All rights reserved.

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.

Micro evidence on brazilian price stickiness and its consequences for sectoral real exchange rate and inflation persistence

The purpose of this thesis is to investigate the price-setting behavior in Brazil and, in particular, the effects on inflation and good-level real exchange rate persistence. This thesis is composed by three Chapters. In the first Chapter, we present the main stylized facts about the behavior of retail prices in Brazil using micro data from the CPI index computed by the Fundação Getulio Vargas. Moreover we construct time series of price-setting statistics and relate them to macroeconomic variables using regression analyses. In Chapter 2, we investigated the relevance of heterogeneity in countries price stickiness on good-level real exchange rate persistence, considering a newly constructed panel data set of relative prices of 115 common products between the U.S. and Brazil. Chapter 3 is devoted to the relation between sectoral price stickiness and inflation persistence.

Periodontal diseases in a representative urban population in south Brazil

Background and Aims: There is little information about the epidemiology and risk factors of periodontal diseases in Latin America in general, and Brazil in particular. The principal aims of this study were to: 1) describe the prevalence and severity of periodontal attachment loss and gingival recession, and to assess the contribution of demographic, behavioral, and environmental exposures to the occurrence of periodontal disease outcomes in a sample representative of the urban population in the state of Rio Grande do Sul in south Brazil; and 2) report the epidemiology and risk indicators of aggressive periodontitis in this population. Methods: A representative sample consisting of 1,586 subjects 14-103 years of age (mean 38 y) and comprising 45.3% males and 54.7% females was selected using a multi-stage, probability, cluster sampling strategy. The subjects were interviewed using a structured questionnaire and underwent a full-mouth, six sites per tooth clinical examination in a mobile examination center. Results: Moderate and severe clinical attachment loss and gingival recession were widespread among adults in this population. The prevalence and extent of attachment loss ³5 and ³7 mm were 79% and 52% subjects, and 36% and 16% teeth; and for gingival recession ³3 mm and ³5 mm were 52% and 22% subjects, and 17% and 6% teeth, respectively. Aggressive periodontitis was diagnosed in 5.5% of subjects, which is significantly higher than the reported prevalence in most other populations. Among the main risk indicators for chronic as well as aggressive destructive periodontal diseases were: older age, low socioeconomic status, dental calculus, and smoking. Cigarette smoking accounted for an important part of periodontal disease burden, particularly in adults, and should be considered an important target in any prevention strategy aimed at reducing the burden of periodontal diseases. Partial recording methods consistently underestimated the prevalence of attachment loss in the population, and the extent of underestimation was dependent on the type of system used and the threshold of attachment loss. Conclusions: Destructive periodontal diseases are prevalent in this Brazilian population. Suitable disease prevention and health promotion programs should be established to improve the periodontal health in this population.

Essays on the credit channel of monetary policy: a case study for Brazil

The onset of the financial crisis in 2008 and the European sovereign crisis in 2010 renewed the interest of macroeconomists on the role played by credit in business cycle fluctuations. The purpose of the present work is to present empirical evidence on the monetary policy transmission mechanism in Brazil with a special eye on the role played by the credit channel, using different econometric techniques. It is comprised by three articles. The first one presents a review of the literature of financial frictions, with a focus on the overlaps between credit activity and the monetary policy. It highlights how the sharp disruptions in the financial markets spurred central banks in developed and emerging nations to deploy of a broad set of non conventional tools to overcome the damage on financial intermediation. A chapter is dedicated to the challenge face by the policymaking in emerging markets and Brazil in particular in the highly integrated global capital market. This second article investigates the implications of the credit channel of the monetary policy transmission mechanism in the case of Brazil, using a structural FAVAR (SFAVAR) approach. The term “structural” comes from the estimation strategy, which generates factors that have a clear economic interpretation. The results show that unexpected shocks in the proxies for the external finance premium and the credit volume produce large and persistent fluctuations in inflation and economic activity – accounting for more than 30% of the error forecast variance of the latter in a three-year horizon. Counterfactual simulations demonstrate that the credit channel amplified the economic contraction in Brazil during the acute phase of the global financial crisis in the last quarter of 2008, thus gave an important impulse to the recovery period that followed. In the third articles, I make use of Bayesian estimation of a classical neo-Keynesian DSGE model, incorporating the financial accelerator channel developed by Bernanke, Gertler and Gilchrist (1999). The results present evidences in line to those already seen in the previous article: disturbances on the external finance premium – represented here by credit spreads – trigger significant responses on the aggregate demand and inflation and monetary policy shocks are amplified by the financial accelerator mechanism. Keywords: Macroeconomics, Monetary Policy, Credit Channel, Financial Accelerator, FAVAR, DSGE, Bayesian Econometrics

The implementation of corporate governance in Germany and Brazi l: a comparative case study

The recent promotion of best corporate governance standards by several different government institutions and non-for profit organizations resulted in the implementation of more sophisticated governance mechanisms. As consequence to the separation of ownership and control the concept of agency theory arose. Agency theory argues that without out proper control mechanism managers would behave exploit owners due to information asymmetry. Regulators have promoted corporate governance mechanisms in order to address this issue. This paper aims to contrast the implementation of best corporate governance practices in Germany and Brazil on the example of two practical examples. With this purpose in mind, this paper analyzed two companies listed in the main stock exchange in Germany and Brazil throughout a period of 5 years. In order to measure the degree of corporate governance practices implemented 3 different parameters have been chosen. In line with great part of the literature the parameters considered to be relevant are; composition, procedures and deviation from the local corporate governance code. The comparison of the data revealed that board composition in the two analyzed companies is similar regarding the proportion of independent representatives but does distinguish in size. While committees are related to the same topics it can be implied that Natura’s board is more involved in the actual management of the company. Lastly, Beiersdorf has been able to comply to a larger extend with the recommendations of the local German code than Natura to the recommendations published by Brazilian code of the IBGC.

The Brazilian and the french private equity sectors from 2006 to present: a comparative analysis

Private equity, ou o ato de fundos ou investidores de investir em empresas não cotadas em bolsa pública, assumiu uma importância crescente no mundo financeiro nos últimos anos. De fato, enquanto o surgimento de um setor de private equity (PE) tem sido um grande fenômeno em mercados emergentes desde meados dos anos 2000, a crise financeira mundial enfraqueceu private equity no mundo desenvolvido. Assim, esta pesquisa vai se concentrar em dois países com dinâmicas supostamente muito diferentes em relação a este sector: França e Brasil. O objetivo será o de discernir padrões gerais de comportamento em ambos os sectores de PE durante todo o período compreendido 2006-2013, e tentar determinar em que medida eles são comparáveis. Utilizando a literatura como fonte conceitual para o quadro comparativo a ser desenvolvido, será analisado se as condições do mercado e do ambiente institucional evoluíram durante o período estudado na França e no Brasil, se comparar, e se eles impactaram o nível de atividade de private equity - oferta e demanda de fundos - em ambos os países. Para identificar esses padrões, a pesquisa contará com uma análise de dados exploratória qualitativa, com base em um quadro dos determinantes do setor de PE identificados e retirados da literatura acadêmica. Esta pesquisa trazera sua contribuição para o trabalho acadêmico existente sobre private equity, graças à sua natureza comparativa e para a sua conclusão sobre a relevância dos determinantes acima mencionados sobre a atividade de private equity na França e no Brasil.

Differences in the perception of interest representation: a comparision of Brasília and Brussels lobbying activity

The research topic of this paper is focused on the analysis of how trade associations perceive lobbying in Brussels and in Brasília. The analysis will be centered on business associations located in Brasília and Brussels as the two core centers of decision-making and as an attraction for the lobbying practice. The underlying principles behind the comparison between Brussels and Brasilia are two. Firstof all because the European Union and Brazil have maintained diplomatic relations since 1960. Through these relations they have built up close historical, cultural, economic and political ties. Their bilateral political relations culminated in 2007 with the establishment of a Strategic Partnership (EEAS website,n.d.). Over the years, Brazil has become a key interlocutor for the EU and it is the most important market for the EU in Latin America (European Commission, 2007). Taking into account the relations between EU and Brazil, this research could contribute to the reciprocal knowledge about the perception of lobby in the respective systems and the importance of the non-market strategy when conducting business. Second both EU and Brazilian systems have a multi-level governance structure: 28 Member States in the EU and 26 Member States in Brazil; in both systems there are three main institutions targeted by lobbying practice. The objective is to compare how differences in the institutional environments affect the perception and practice of lobbying, where institutions are defined as ‘‘regulative, normative, and cognitive structures and activities that provide stability and meaning to social behavior’’ (Peng et al., 2009). Brussels, the self-proclaimed "Capital of Europe”, is the headquarters of the European Union and has one of the highest concentrations of political power in the world. Four of the seven Institutions of the European Union are based in Brussels: the European Parliament, the European Council, the Council and the European Commission (EU website, n.d.). As the power of the EU institutions has grown, Brussels has become a magnet for lobbyists, with the latest estimates ranging from between 15,000 and 30,000 professionals representing companies, industry sectors, farmers, civil society groups, unions etc. (Burson Marsteller, 2013). Brasília is the capital of Brazil and the seat of government of the Federal District and the three branches of the federal government of Brazilian legislative, executive and judiciary. The 4 city also hosts 124 foreign embassies. The presence of the formal representations of companies and trade associations in Brasília is very limited, but the governmental interests remain there and the professionals dealing with government affairs commute there. In the European Union, Brussels has established a Transparency Register that allows the interactions between the European institutions and citizen’s associations, NGOs, businesses, trade and professional organizations, trade unions and think tanks. The register provides citizens with a direct and single access to information about who is engaged in This process is important for the quality of democracy, and for its capacity to deliver adequate policies, matching activities aimed at influencing the EU decision-making process, which interests are being pursued and what level of resources are invested in these activities (Celgene, n.d). It offers a single code of conduct, binding all organizations and self-employed individuals who accept to “play by the rules” in full respect of ethical principles (EC website, n.d). A complaints and sanctions mechanism ensures the enforcement of the rules and addresses suspected breaches of the code. In Brazil, there is no specific legislation regulating lobbying. The National Congress is currently discussing dozens of bills that address regulation of lobbying and the action of interest groups (De Aragão, 2012), but none of them has been enacted for the moment. This work will focus on class lobbying (Oliveira, 2004), which refers to the performance of the federation of national labour or industrial unions, like CNI (National Industry Confederation) in Brazil and the European Banking Federation (EBF) in Brussels. Their performance aims to influence the Executive and Legislative branches in order to defend the interests of their affiliates. When representing unions and federations, class entities cover a wide range of different and, more often than not, conflicting interests. That is why they are limited to defending the consensual and majority interest of their affiliates (Oliveira, 2004). The basic assumption of this work is that institutions matter (Peng et al, 2009) and that the trade associations and their affiliates, when doing business, have to take into account the institutional and regulatory framework where they do business.

Is what you say what you do? Analyzing and comparing the effect of buyer-supplier relationship in the Brazilian and Chinese supplier selection criteria

Choosing properly and efficiently a supplier has been challenging practitioners and academics since 1960’s. Since then, countless studies had been performed and relevant changes in the business scenario were considered such as global sourcing, quality-orientation, just-in-time practices. It is almost consensus that quality should be the selection driver, however, some polemical findings questioned this general agreement. Therefore, one of the objectives of the study was to identify the supplier selection criteria and bring this discussion back again. Moreover, Dickson (1966) suggested existing business relationship as selection criterion, then it was reviewed the importance of business relationship for the company and noted a set of potential negative effects that could rise from it. By considering these side effects of relationship, this research aimed to investigate how the relationship could influence the supplier selection and how its harmful effects could affect the selection process. The impact of this phenomenon was investigated cross-nationally. The research strategy adopted was a controlled experiment via vignette combined with discrete choice analysis. The data collections were performed in China and Brazil. By examining the results, it could be drawn five major findings. First, when purchasers were asked to declare their supplier selection priorities, quality was stated as the most important independently of country and relationship. This result was consistent with diverse studies since 60’s. However, when purchasers were exposed to a multi-criteria trade-off situation, their actual selection priorities deviate from what they had declared. In the actual decision-making without influence of buyer-supplier relationship, Brazilian purchasers focused on price and Chinese buyers prioritized delivery then price. This observation reinforced some controversial prior studies of Verma & Pullman (1998) and Hirakubo & Kublin (1998). Second, through the introduction of the buyer-supplier relationship (operationalized via relational capital) in the supplier selection process, this research extended the existing studies and found that Brazilian buyers still focused on price. The relationship became just another criterion for supplier selection such as quality and delivery. However, from the Chinese sample, the results suggested that quality was totally discarded and the decision was majorly made through price and relationship. The third finding suggested that relational capital could legitimate the quality and sustainability of the supplier and replaces these selection criteria and made the decisional task less complex. Additionally, with the relational capital, the decision-makings were associated to few biases such as availability cognition, commitment, confirmatory and perceived biases. By analyzing the purchasers’ behavior, relational capital inducted buyers of both countries to relax in their purchasing requirements (quality, delivery and sustainability) leading to potential negative effects. In the Brazilian sample, the phenomenon of willing to pay a higher price for a lower quality offer demonstrated to be a potential counterproductive and suboptimal decision. Finally, the last finding was associated to the cultural effect on the buyers’ decisions. From the outcome, it is possible to observe that if a purchaser’s cultural background is more relation-oriented, the more he will tend to use relational capital as a decision heuristic, thus, the purchaser will be more susceptible to the potential relationship’s side effects

Reading comprehension in primary school: textbooks, curriculum and assessment

This study describes the reading comprehension processes present in the most widely sold textbooks at the fourth grade level in Portugal and discusses how they compare to international assessments of reading literacy. We adopted the Progress of International Reading Literacy Study framework to categorize the questions in the textbooks. Our analyses revealed that they focus heavily on the retrieval of explicitly stated information to the detriment of higher level comprehension skills. Portuguese fourth grade textbooks rarely challenge students to make connections between their knowledge and the ideas in the texts and to adopt a critical and evaluative reading stance. This is in sharp contrast to what students are asked to do in the Progress of International Reading Literacy Study, conducted every five years since 2001, and it may help explain the poor results Portuguese students have in national assessment and in PISA. The findings are discussed in light of the curriculum frameworks currently adopted in Portugal and suggestions are made as to how we can improve reading literacy achievement.

Impacto das tecnologias digitais nas bibliotecas universitárias: reflexões sobre o tema

MOREIRA, Luciana Moreira; SILVA, Armando Malheiro da. Impacto das tecnologias digitais nas bibliotecas universitarias: reflexões sobre o tema. Informaçao e sociedade: estudos. Joao Pessoa, v.19, n.3, p. 125-132,2009.

Chronic Diseases: Chronic Diseases and Development 2 Health, agricultural, and economic effects of adoption of healthy diet recommendations

Transition to diets that are high in saturated fat and sugar has caused a global public health concern as the pattern of food consumption is a mayor modifiable risk factor for chronic non-communicable diseases Although agri food systems are intimately associated with this transition, agriculture and health sectors are largely disconnected in their priorities policy, and analysis with neither side considering the complex inter relation between agri trade patterns of food consumption health, and development We show the importance of connection of these perspectives through estimation of the effect of adopting a healthy diet on population health, agricultural production trade the economy and livelihoods, with a computable general equilibrium approach on the basis of case studies from the UK and Brazil we suggest that benefits of a healthy diet policy will vary substantially between different populations, not only because of population dietary intake but also because of agricultural production trade and other economic factors