Characterisation of six novel A-subunit mutations leading to congenital factor XIII deficiency and molecular analysis of the first diagnosed patient with this rare bleeding disorder

In 1960, the first case report on factor XIII deficiency was published describing a seven-year-old Swiss boy with a so far unknown bleeding disorder. Today, more than 60 mutations in the factor XIIIA- and B-subunit genes are known leading to congenital factor XIII deficiency. In the present study, we describe six novel mutations in the factor XIII A-subunit gene. Additionally, we present the molecular characterisation of the first described patient with congenital factor XIII deficiency. The six novel mutations include a small deletion, Glu202 delG, leading to a premature stop codon and truncation of the protein, and a splice site mutation at the exon 10/intron 10 boundary, +1G/A, giving rise to an incorrect spliced mRNA lacking exons 10 and 11. The remaining four mutations are characterised by the single amino acid changes Met159Arg, Gly215Arg, Trp375Cys, and His716Arg, and were expressed in COS-1 cells. Antigen levels and activity of the mutants were significantly reduced compared to the wild-type. The patient described in 1960 also shows a single amino acid change, Arg77Cys. Structural analysis of all mutant enzymes suggests several mechanisms leading to destabilisation of the protein.

The Lord of Heaven in the Inner Chambers: Jesuits, Women, and Domestic Christianity (ca. 1580-1690)

The study focuses on gender norms and practices in Chinese Christian communities established by Jesuit missionary activity during the long seventeenth century. It analyzes how European and Chinese gender norms and practices affected each other in the context of the Sino-Western cultural contact initiated by the missionaries. The thesis consists of two parts. First, it analyzes the ways in which European Jesuits engaged with Chinese gender relations in the course of their mission in China. The study demonstrates that the Jesuits’ adoption of the Chinese scholar-gentry’s habitus entailed a partial adaptation to Confucian gender norms. The latter placed great emphasis on gender segregation and therefore discouraged direct communication between missionaries and Chinese women. This resulted in the emergence of organizational and devotional arrangements of Christian communities specific to China. Second, the study discusses Chinese Christian women's religious culture that emerged in the absence of a strong missionary presence among female devotees. It points out that Chinese Christian women created their own ritual culture and religious sociability in the domestic context, and that they actively took part in shaping Chinese Christianity as masters of domestic rituals.

The Performance of Domestic and Foreign Banks: The Case of Korea and the Asian Financial Crisis

This paper considers the performance of banks, domestic and foreign, in Korea prior to, during, and immediately after the Asian financial crisis, examining how the profitability of those banks differed and identifying factors that explain why those differences existed. The performance of Korean banks deteriorated dramatically in 1998 with most banks recovering somewhat in 1999. Foreign banks did not experience the same negative effect on their returns on assets and equity as a rule. Several standard findings emerge. For example, equity to assets correlates positively with domestic, but not foreign, bank performance, as measured by the returns on assets and equity, even when the government recapitalized institutions that were performing quite badly. Also, foreign-currency deposits significantly and negatively correlate with domestic Korean bank performance, although only in the post-crisis period for regional banks. In sum, the domestic Korean banks suffered more severely from the Asian financial crisis than foreign banks.

The Limited Influence of the European Union in Armenia and Azerbaijan: A Domestic Explanation. EU Diplomacy Paper 9/2015

This paper seeks to explain why the European Union (EU) has had limited influence in Armenia and Azerbaijan in the framework of the European Neighbourhood Policy (ENP). Combining approaches from external governance, norm diffusion and structural foreign policy, it offers an explanation based on domestic factors in the two countries: the political regime, state capacity, political structures, domestic incentives and the perceived legitimacy of EU rules. Although willingness to reform appears to exist in Armenia, such willingness remains constrained by the country’s vulnerable geopolitical location and high dependence on Russia. By contrast, none of the domestic preconditions for EU influence identified by the analytical framework were found in Azerbaijan. The author argues that the Eastern Partnership has not properly addressed the extent to which the clan structures feed into informal political practices and enforce the sustainability of an existing regime in both countries, and that, in addition, the EU has underestimated the multipolar environment which the two countries have to operate in, making it unlikely that the current policy can reach its objectives in Armenia and Azerbaijan.

Reinventing Liberty : Nation, Commerce and the British Historical Novel from Walpole to Scott /

Sir Walter Scott is often regarded as the first historical novelist. Reinventing Liberty challenges this view by returning us to the rich range of historical fiction written in the late 18th and early 19th century. For the first time placing these works in the context of British politics and British history writing, this book redefines the historical novel, revealing a genre which seeks to manage political change through historiographical experimentation. It explores how historical novelists participated in a contentious debate concerning the nature of commercial modernity, the formulation of political progress and British national identity. Ranging across well-known writers, like William Godwin, Horace Walpole and Frances Burney, to lesser-known figures, such as Cornelia Ellis Knight and Jane Porter, Reinventing Liberty uncovers how history becomes a site to rethink Britain as ‘land of liberty’. Reading Scott in relation to this tradition, Reinventing Liberty demonstrates the genre’s troubled role in the construction of the myth of Britain as a nation of gradual, safe political change.

Domestic abuse of adults with disabilities : report to the Illinois General Assembly.

Required by the Domestic Abuse of Disabled Adults Intervention Act ("act"), P.A. 87-658.

Air pollution exposure and novel biomarkers of inflammation and cardiac stress in the Multi-Ethnic Study of Atherosclerosis and Air Pollution (MESA Air)

Thesis (Master's)--University of Washington, 2016-06

The impact on domestic productivity of inward investment in the UK

One of the basic tenets of UK industrial policy, that attracting inward investment into the UK stimulates domestic productivity growth, is examined. A model of productivity growth is developed for the indigenous sector of UK manufacturing, linking domestic productivity growth to theoretical explanations of inward investment. The paper demonstrates that inward investment does stimulate productivity growth in the domestic sector of around 0.75 per cent per annum. However, this cannot be attributed to investment or output spillovers, but is a result of the productivity advantage exhibited by the foreign firms.

A study in the application of domestic solar assisted heat pumps for heating and cooling

In the present work, the more important parameters of the heat pump system and of solar assisted heat pump systems were analysed in a quantitative way. Ideal and real Rankine cycles applied to the heat pump, with and without subcooling and superheating were studied using practical recommended values for their thermodynamics parameters. Comparative characteristics of refrigerants here analysed looking for their applicability in heat pumps for domestic heating and their effect in the performance of the system. Curves for the variation of the coefficient of performance as a function of condensing and evaporating temperatures were prepared for R12. Air, water and earth as low-grade heat sources and basic heat pump design factors for integrated heat pumps and thermal stores and for solar assisted heat pump-series, parallel and dual-systems were studied. The analysis of the relative performance of these systems demonstrated that the dual system presents advantages in domestic applications. An account of energy requirements for space and hater heating in the domestic sector in the O.K. is presented. The expected primary energy savings by using heat pumps to provide for the heating demand of the domestic sector was found to be of the order of 7%. The availability of solar energy in the U.K. climatic conditions and the characteristics of the solar radiation here studied. Tables and graphical representations in order to calculate the incident solar radiation over a tilted roof were prepared and are given in this study in section IV. In order to analyse and calculate the heating load for the system, new mathematical and graphical relations were developed in section V. A domestic space and water heating system is described and studied. It comprises three main components: a solar radiation absorber, the normal roof of a house, a split heat pump and a thermal store. A mathematical study of the heat exchange characteristics in the roof structure was done. This permits to evaluate the energy collected by the roof acting as a radiation absorber and its efficiency. An indication of the relative contributions from the three low-grade sources: ambient air, solar boost and heat loss from the house to the roof space during operation is given in section VI, together with the average seasonal performance and the energy saving for a prototype system tested at the University of Aston. The seasonal performance as found to be 2.6 and the energy savings by using the system studied 61%. A new store configuration to reduce wasted heat losses is also discussed in section VI.

Effectiveness of Olympic sponsorship by foreign and domestic companies: the influential role of consumer ethnocentrism

This study investigates Chinese consumers' responses to foreign and domestic sponsors engaged in the Beijing Olympic Games. It identifies direct causal relationships between consumer ethnocentrism, attitudes towards the sponsor and product judgement. Findings reveal that event involvement mediates the positive relationship between consumer ethnocentrism and attitudes towards the domestic sponsor. Attitudes towards foreign sponsors are found to be a significant mediator in the relationship between consumer ethnocentrism and judgements of the sponsors' products. Theoretical and managerial implications are discussed.

Domestic Politics in the Dominican Republic after the Earthquake in Haiti

Despite historical tensions between the Dominican Republic and Haiti, Dominicans appear to have put aside their resentment in favor of supporting Haitians after the earthquake that devastated the neighbor nation in January 2010. Over the past several months, there has been unprecedented cooperation between the Dominican Republic and Haiti, with little evidence of a negative impact on domestic politics in the Dominican Republic. In fact, the high ratings of President Leonel Fernández and the results of the May Parliamentary elections may suggest that how the Fernández administration handled the Haitian crisis did not have a negative impact on citizens’ perception. However, the issue of Haitian immigration remains very sensitive in the Dominican Republic, and has the potential to become the major concern on the domestic political front. As of June 2010, the Haitian crisis seemed to have little or no impact on Dominican politics, as the following points indicate: The May 16, 2010 Parliamentary elections increased President Fernández political party to 31 out of 32 Senate seats, and 105 out of 183 Chamber of Deputies seats; this is a total increase of 18 seats from the previous term. Polls indicate that President Fernández has a 54 percent approval rating. Polls also indicate that Haiti is not among the most pressing issues of concern to Dominican citizens. Instead, 65 percent of the population identifies drug trafficking and corruption as the greatest concerns. The immigration debate will remain the major consideration in domestic politics in the Dominican Republic; 62.4 percent of Dominicans polled think that the military should be strengthened along the DR-Haitian border.

The World Trade Organization, competition policy, and developing countries: A cross -country examination of domestic policy actions

This dissertation addresses the following research question: in a particular policy area, why do countries that display unanimity in their international policy behavior diverge from each other in their domestic policy actions? I address this question in the context of the divergent domestic competition policy actions undertaken by developing countries during the period 1996-2007, after these countries had quite conspicuously displayed near-unanimity in opposing this policy measure at the World Trade Organization (WTO). This divergence is puzzling because (a) it does not align with their near-unanimous behavior at the WTO over competition policy and (b) it is at variance with the objectives of their international opposition to this policy at the WTO. Using an interdisciplinary approach, this dissertation examines the factors responsible for this divergence in the domestic competition policy actions of developing countries. ^ The theoretical structure employed in this study is the classic second-image-reversed framework in international relations theory that focuses on the domestic developments in various countries following an international development. Methodologically, I employ both quantitative and qualitative methods of analysis to ascertain the nature of the relationship between the dependent variable and the eight explanatory variables that were identified from existing literature. The data on some of the key variables used in this dissertation was uniquely created over a multi-year period through extensive online research and represents the most comprehensive and updated dataset currently available. ^ The quantitative results obtained from logistic regression using data on 131 countries point toward the significant role played by international organizations in engineering change in this policy area in developing countries. The qualitative analysis consisting of three country case studies illuminate the channels of influence of the explanatory variables and highlight the role of domestic-level factors in these three carefully selected countries. After integrating the findings from the quantitative and qualitative analyses, I conclude that a mix of international- and domestic-level variables explains the divergence in domestic competition policy actions among developing countries. My findings also confirm the argument of the second-image-reversed framework that, given an international development or situation, the policy choices that states make can differ from each other and are mediated by domestic-level factors. ^

‘Tenshillingland’: Community and Commerce, Myth and Madness in the Modern Scottish Novel.

While ‘community’ as a concept has come under increasing attack in a neoliberal era, it has remained in Scotland a mythic, though not unexamined, signifier of resistance to perceived threats to national identity. Community, central to the Scottish novel since the Kailyard, continues to be a prevalent theme in the many important novels of the twentieth and twenty-first centuries explored here. Yet, while often disturbingly oppressive in tenor, many of these representations of community actually attack the myth of Scottish communalism to critique, and often expose as forms of madness, the conventional values of social class, capitalism, patriarchy, and religion.

Forms of the Postmodern Historical Novel : Christoph Ransmayr, Daniel Kehlmann, Wolfgang Hildesheimer

L’émergence du postmodernisme aux Etats-unis, mouvement esthétique rejetant les dogmes modernistes, date des années 1960. En s’imposant, durant les dernières décennies du 20ème siècle, comme le paradigme esthétique de l’architecture, des arts et de la littérature, le postmodernisme a également créé les conditions propres à une renaissance du roman historique. Cependant, la fiction historique postmoderne constitue maintenant une nouvelle forme du genre basée sur la parodie, l’ironie et le scepticisme envers les discours dominants. Cette nouvelle forme ne se limite plus à la présentation des récits dans un cadre historique réaliste. Elle remet plutôt en question la validité et par conséquent la nature même du discours historique, problématisant et mettant ainsi à l’avant le processus d’interprétation et de reconstruction du passé. Dans cette optique, la fiction historique contemporaine reflète les débats actuels sur les formes de l’historiographie, débats lancés par Hayden White. Dans les années 1980, la fiction historique a de nouveau fleuri dans l'espace culturel allemand tout comme ailleurs. Le présent mémoire analyse des formes postmodernes de la fiction historique en se basant sur trois romans historiques de langue allemande parus entre 1981 et 2005: Die Schrecken des Eises und der Finsternis de Christoph Ransmayr, Die Vermessung der Welt de Daniel Kehlmann et Marbot : Eine Biographie de Wolfgang Hildesheimer. L’analyse s'appuie sur divers modèles de la fiction postmoderne, en particulier sur le schéma de catégorisation du roman historique élaboré par Ansgar Nünning. Le mémoire montre dans quelle mesure ces romans appliquent des moyens stylistiques typiques pour le postmodernisme et portent un regard critique ou comique sur l’histoire et la culture allemande et autrichienne.

Forms of the Postmodern Historical Novel : Christoph Ransmayr, Daniel Kehlmann, Wolfgang Hildesheimer

L’émergence du postmodernisme aux Etats-unis, mouvement esthétique rejetant les dogmes modernistes, date des années 1960. En s’imposant, durant les dernières décennies du 20ème siècle, comme le paradigme esthétique de l’architecture, des arts et de la littérature, le postmodernisme a également créé les conditions propres à une renaissance du roman historique. Cependant, la fiction historique postmoderne constitue maintenant une nouvelle forme du genre basée sur la parodie, l’ironie et le scepticisme envers les discours dominants. Cette nouvelle forme ne se limite plus à la présentation des récits dans un cadre historique réaliste. Elle remet plutôt en question la validité et par conséquent la nature même du discours historique, problématisant et mettant ainsi à l’avant le processus d’interprétation et de reconstruction du passé. Dans cette optique, la fiction historique contemporaine reflète les débats actuels sur les formes de l’historiographie, débats lancés par Hayden White. Dans les années 1980, la fiction historique a de nouveau fleuri dans l'espace culturel allemand tout comme ailleurs. Le présent mémoire analyse des formes postmodernes de la fiction historique en se basant sur trois romans historiques de langue allemande parus entre 1981 et 2005: Die Schrecken des Eises und der Finsternis de Christoph Ransmayr, Die Vermessung der Welt de Daniel Kehlmann et Marbot : Eine Biographie de Wolfgang Hildesheimer. L’analyse s'appuie sur divers modèles de la fiction postmoderne, en particulier sur le schéma de catégorisation du roman historique élaboré par Ansgar Nünning. Le mémoire montre dans quelle mesure ces romans appliquent des moyens stylistiques typiques pour le postmodernisme et portent un regard critique ou comique sur l’histoire et la culture allemande et autrichienne.