ADCK3, an ancestral kinase, is mutated in a form of recessive ataxia associated with coenzyme Q(10) deficiency

Muscle coenzyme Q(10) (CoQ(10) or ubiquinone) deficiency has been identified in more than 20 patients with presumed autosomal-recessive ataxia. However, mutations in genes required for CoQ(10) biosynthetic pathway have been identified only in patients with infantile-onset multisystemic diseases or isolated nephropathy. Our SNP-based genome-wide scan in a large consanguineous family revealed a locus for autosomal-recessive ataxia at chromosome 1q41. The causative mutation is a homozygous splice-site mutation in the aarF-domain-containing kinase 3 gene (ADCK3). Five additional mutations in ADCK3 were found in three patients with sporadic ataxia, including one known to have CoQ(10) deficiency in muscle. All of the patients have childhood-onset cerebellar ataxia with slow progression, and three of six have mildly elevated lactate levels. ADCK3 is a mitochondrial protein homologous to the yeast COQ8 and the bacterial UbiB proteins, which are required for CoQ biosynthesis. Three out of four patients tested showed a low endogenous pool of CoQ(10) in their fibroblasts or lymphoblasts, and two out of three patients showed impaired ubiquinone synthesis, strongly suggesting that ADCK3 is also involved in CoQ(10) biosynthesis. The deleterious nature of the three identified missense changes was confirmed by the introduction of them at the corresponding positions of the yeast COQ8 gene. Finally, a phylogenetic analysis shows that ADCK3 belongs to the family of atypical kinases, which includes phosphomositide and choline kinases, suggesting that ADCK3 plays an indirect regulatory role in ubiquinone biosynthesis possibly as part of a feedback loop that regulates ATP production.

Dilepton mass spectra in p plus p collisions at root s=200 GeV

PHENIX has measured the electron-positron pair mass spectrum from 0 to 8 GeV/c(2) in p + p collisions at root s = 200 GeV. The contributions from light meson decays to e(+)e(-) pairs have been determined based on measurements of hadron production cross sections by PHENIX. Within the systematic uncertainty of similar to 20% they account for all e(+)e(-) pairs in the mass region below similar to 1 GeV/c(2). The e(+)e(-) pair yield remaining after subtracting these contributions is dominated by semileptonic decays of charmed hadrons correlated through flavor conservation. Using the spectral shape predicted by PYTHIA, we estimate the charm production cross section to be 544 +/- 39(stat) +/- 142(syst) +/- 200(model) pb. which is consistent with QCD calculations and measurements of single leptons by PHENIX. (C) 2008 Elsevier BV. All rights reserved.

Deiro Collection Finding Aid

The Deiro Collection at the City University of New York Graduate Center's Mina Rees Library is an archival collection of materials related to the professional and personal lives of Guido Deiro (1886-1950), Pietro Deiro Sr. (1888-1954) and Pietro “Lee” Deiro Jr. (1913-1999). Immigrating from Italy to the United States in the early 1900's, the Deiro brothers Guido and Pietro, made enduring contributions to the popularization of the Piano Accordion in the 20th Century. As masters of the instrument, the Deiro's achieved headliner status on the vaudeville theatre circuit. Both composed, arranged and recorded an impressive repertoire of accordion music. Pietro Deiro Publications produced a catalogue of over 10,000 pieces of sheet music and instructional materials for the Piano Accordion. The Deiro Collection documents not only a singular segment of American musical history but also a unique aspect of the Italian-American experience in 20th Century America.

En retorisk analys av Fahrenheit 9/11 ur ett dokumentärhistoriskt perspektiv

The history of rhetorics in documentary film is a long one. The French brothers Louise and Auguste Lumiére were among the first persons who produced a documentary and their work has had a big impression in the history of documentary during the 1900 century. John Grierson has been called “the father” of documentary film. He produced films in the English speaking part of the world and he is the founder of the concept “documentary film”. Propaganda filming has a big part in the history of documentary film. Most frequently it appeard in Germany before and during the secound WW II, but propaganda films has been produced in Great Britain and USA as well as in other parts of the world. Rhetorics are of current interest today. It effects the news, papers, magazines, books and movies. The purpose with this essay is to investigate the rhetorics in Michael Moores controversial documentary of September 11th, Fahrenheit 9/11. The main question is: what rhetorical tools does Michael Moore use in order to communicate his politcal message in Fahrenheit 9/11? The rhetorical analysis includes exordium, narratio, propsitio, argumentatio and conclusio as well as ethos, logos and pathos. In order to conduct the analysis I have used Kurt Johannesson’s book Retorik eller konsten att övertyga, Maria Karlberg and Brigitte Mral’s book Heder och påverkan. Att analysera modern retorik. And at last a chapter by Brigitte Mral named Retorikanalys in the book Metoder i kommunikationeverenskap, by Mats Ekström and Larsåke Larsson. For the background about the history of documentary film I have used Barsam M. Richard, Nonfiction Film A Critical History and Bjørn Sørenssen’s book Å fange virkeligheten –Dokumentarfilmens århundre.The analysis shows that Moore uses different rhetorical tools in order to point out that George W Bush is the wrong man for the role as the president of United States of America. Some of the most effective tools that Moores uses are his own voice, pictures who awakes emotions, recordings and sharp arguments. His way of using ethos, logos and pathos is very clear. The disposal can also be distinct discern, however there are some exceptions towards the classical rhetoric. One example is that Moore doesn´t describe the arrangement of the movie in exordium.

Thick viewing : empirical examples of condensed moments of narrative impact in fiction film developing theoretical concepts for intensified engagements

Abstract  In a case study about viewing habits in a Swedish audience I sampled 309 questionnaires; interviews with five focus group were conducted together with ten in-depth individual interviews discussing altogether fifteen favorite films exploring specific scenes of idiosyncratic relevance.  The outcome supports claims about viewers as active and playful (cf. Höijer 1998, Frampton 2006, Hoover 2006, Plantinga 2009). In line with mediatization theory I also argue that spiritual meaning making takes place through mediated experiences and I support theories about fiction films as important sources for moral and spiritual reflection (Partridge 2004, Zillman 2005, Lynch 2007, Plantinga 2009). What Hjarvard calls the soft side of mediatization processes (2008) is illustrated showing adults experiencing enchantment through favorite films (Jerslev 2006, Partridge 2008, Klinger 2008, Oliver & Hartmann 2010).  Vernacular meaning making embedded in everyday life and spectators dealing with fiction narratives such as Gladiator, Amelie from Montmartre or Avatar highlights the need for a more nuanced understanding of elevated cinematic experiences. The reported impact of specific movies is analyzed through theories where cognition and affect are central aspects of spectators’ engagements with a film (Tan 1996, Caroll 1999, Grodal 2009). Crucially important are theories of meaning-making where viewers’ detailed interpretation of specific scenes are embedded in high-level meaning-making where world view issues and spectators’ moral frameworks are activated (Zillman 2005, Andersson & Andersson 2005, Frampton 2006, Lynch 2007, Avila 2007, Axelson 2008, Plantinga 2009).  Also results from a growing body of empirical oriented research in film studies are relevant with an interest in what happens with the flesh and blood spectator exposed to filmic narratives (Jerslev 2006, Klinger 2008, Barker 2009, Suckfüll 2010, Oliver & Hartmann 2010). Analyzing the qualitative results of my case study, I want to challenge the claim that the viewer has to suspend higher order reflective cognitive structures in order to experience suture (Butler & Palesh 2004). What I find in my empirical examples is responses related to spectators’ highest levels of mental activity, all anchored in the sensual-emotional apparatus (Grodal 2009). My outcome is in line with a growing number of empirical case studies which support conclusions that both thinking and behavior are affected by film watching (Marsh 2007, Sückfull 2010, Oliver & Hartmann 2010, Axelson forthcoming). The presentation contributes to a development of concepts which combines aesthetic, affective and cognitive components in an investigation of spectator’s moves from emotional evaluation of intra-text narration to extra-textual assessments, testing the narrative for larger significance in idiosyncratic ways (Bordwell & Thompson 1997, Marsh 2007, Johnston 2007, Bruun Vaage 2009, Axelson 2011). There are a several profitable concepts suggested to embrace the complex interplay between affects, cognition and emotions when individuals respond to fictional narratives. Robert K. Johnston label it “deepening gaze” (2007: 307) and “transformative viewing” (2007: 305). Philosopher Mitch Avila proposes “high cognition” (2007: 228) and Casper Thybjerg ”higher meaning” (2008: 60). Torben Grodal talks about “feelings of deep meaning” (Grodal 2009: 149). With a nod to Clifford Geertz, Craig Detweiler adopts “thick description” (2007: 47) as do Kutter Callaway altering it to ”thick interpretations” (Callaway 2013: 203).  Frampton states it in a paradox; ”affective intelligence” (Frampton 2006: 166). As a result of the empirical investigation, inspired by Geertz, Detweiler & Callaway, I advocate thick viewing for capturing the viewing process of these specific moments of film experience when profound and intensified emotional interpretations take place. The author As a sociologist of religion, Tomas Axelsons research deals with people’s use of mediated narratives to make sense of reality in a society characterized by individualization, mediatization and pluralized world views.  He explores uses of fiction film as a resource in every day life and he is currently finishing his three year project funded by the Swedish Research Council: Spectator engagement in film and utopian self-reflexivity. Moving Images and Moved Minds. http://www.du.se/sv/AVM/Personal/Tomas-Axelson Bibliography Axelson, T. (Forthcoming 2014). Den rörliga bildens förmåga att beröra.[1] Stockholm: Liber Axelson, T. (In peer review). Vernacular Meaning Making. Examples of narrative impact in fiction film questioning the ’banal’ notion in mediatization theory. Nordicom Review. Nordicom Göteborg. Axelson, T. (2011). Människans behov av fiktion. Den rörliga bildens förmåga att beröra människan på djupet.[2]Kulturella perspektiv. Volume 2. Article retrieved from www.kultmed.umu.se/digitalAssets/74/74304_axelson-22011.pdf Axelson, Tomas (2010) “Narration, Visualization and Mind. Movies in everyday life as a resource for utopian self-reflection.” Paper presentation at CMRC, 7th Conference of Media, Religion & Culture in Toronto, Canada 9 – 13th August 2010. Axelson, Tomas (2008) Movies and Meaning. Studying Audience, Favourite Films and Existential Matters. Particip@tions : Journal of Audience and Reception Studies. Volume 5, (1). Doctoral dissertation summary. ACTA UNIVERSITATIS UPSALIENSIS. Article retrieved from http://www.participations.org/Volume%205/Issue%201%20-%20special/5_01_axelson.htm  [1] English translation: Moving Images and Moved Minds. [2] English translation: Our need for fiction. Deeply Moved by Moving Images. Cultural Perspectives.

The Björling 'Opera' : A children's Nursery Academy and an Italian Conservatory in Miniature

The aim in this chapter is to develop a deeper understanding about the informal Björling 'School' in Sweden. Contextually the example is related to the micro history of opera education contributing to the macro perspective illuminating a provincial example of the concept of domestic opera schooling. The specific focus was on Karl David Björling (1873-1926), the teaching parent of the Swedish tenor Jussi Björling (1911-1960) and his brothers Gösta and Olle.   The Björling family model of opera schooling belongs to the classical canon of domestic home education which was common during the epoch. The phenomenon is also within the field of opera singing an important reference to the historical context of the Nordic opera history of vocal education.   The uniqueness concerning the Björling School seems to be the rigorous and exceptionally early training. David Björling’s pedagogy was rooted in earlier German theories of musical upbringing. It's clear from his results that he was familiar with the neo-humanistic ideal on which reformed music education was based. Of a specific interest is the term Gesang als Unterricht as a concept for developing childrens musical and memorising capacities.   Conceptually the roots of the Björling model are in the eighteenth-century Romantic view of prodigies and their abilities. The extensive touring is connected to the promotion of wonder-children, and David Björling’s educational style to the conservative Master-pupil tradition.   David Björling's vocal ideal was a part of the contemporary debate about “The decadence of the singing art”, and seems to have its roots in an older Italian tradition. There are recurring similarities between his educational methods and the didactic principles of the Lamperti School: Enjoying a revival around the late 1800s and early 1900s, it has been called the natural or the national school. Nevertheless, through authentic experiences and gramophone recordings the Italian tenor Enrico Caruso became David Björling’s pedagogical role model.

Hurricane Surge Inundation and Critical Resources in Maine

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Avoiding the Fog of Crisis: A Protocol for the Proper Domestic Use of the Military

Failure to contemplate and define an appropriate role for the armed forces of the national government in domestic crises of this sort is a serious problem. It is all the more serious now as these potential crises seem to multiply in character and scope. This thesis will explore the history of this problem and its recent implications. It will argue the need for a comprehensive, operational framework, codified in law, which defines the various alternative uses of all emergency services, both civilian and military, and is applicable to “all hazards.” I will attempt to provide a blue-print for what such a framework should look like.

INVASIVE AQUATIC INVASION: THE SPREAD OF VARIABLE LEAF MILFOIL IN MAINE

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Invasive Aquatic Invasion: The Spread of Variable Leaf Milfoil in Maine

Variable leaf milfoil, Myriophyllum heterophyllum, has been present in Maine since 1970. We created an analysis area including seventeen infestation sites and all bodies of water within a forty mile buffer. We also eliminated all water locations with a size less than 7,101 km2, the size of the smallest infestation site, Shagg Pond. Within those specifications we randomly selected seventeen un-infested bodies of water and used them as our uncontaminated sample. We looked for relationships between presence and number of boat launches, and proximity to a populated area. Using the Mann-Whitney test, we compared the sample size of non-infested lakes to the infested lakes. We found there was no significant difference in all three variables on the infestation of variable leaf milfoil.

O insumo modificado na sala de aula de língua estrangeira : a fala de um professor brasileiro

O presente trabalho objetiva investigar o insumo proporcionado por um professor em contexto formal de sala de aula. Nosso interesse se volta para os tipos de modificações lingüísticas propiciadas por um professor brasileiro para alunos de inglês de diferentes níveis de proficiência. São elencadas as modificações desse professor a partir da categorização de Lynch (1996). Focalizamos o conceito de ‘insumo compreensível’ como fundamental para a compreensão e aprendizagem. Para a coleta de dados, optamos pela produção de duas narrativas pelo professor a partir de gravuras. As produções do professor foram gravadas em áudio e vídeo e seguidas de entrevista com a pesquisadora. Os alunos demonstraram compreensão através de composição em sua língua materna, escrita imediatamente após as produções do professor e de respostas a um questionário especialmente elaborado. A partir da análise dos dados, concluímos que as modificações lingüísticas do professor assemelham-se aos tipos de modificações elencadas por Lynch. Implicações para a sala de aula e sugestões para estudos futuros são apresentadas.

A justiça distributiva como critério fundamental nas políticas públicas: o caso dos jogos panamericanos na cidade do Rio de Janeiro

This dissertation is a project of evaluation of the proposals of the Panamerican Games of 2007, that they will be carried through in the city of Rio De Janeiro. Great events present the possibility of improvement of the urban space and economic development, amongst other chances seen by politics and urban planners well. For this work we search to argue the city as field of specific study, understanding it in the current world-wide scene of globalization and computerization, the relations politics, economic and social existing interns. We search in material literature to study and to evaluate what it would be a ¿good city¿ and studies that had understood the development of the urban space. We also look for to understand the city of Rio de Janeiro as a singular urban space, since the origin of the city as a urban space, passing for the proposals gifts of modification of the carioca space. We dedicate part of the work, inside of the chapter where we understand the city of Rio de Janeiro, the presentation of the project of the Panamerican of 2007, comparing it with great previous events. The vision of technique and politics during the interviews was essential for the best understanding of the proposals of the Games. Finally we evaluate the proposals of modification of the Pan2007 for the city of Rio de Janeiro with the methodology of Kevin Lynch to evaluate the good form of the city" in communion with the proposal of distributive justice of Rawls. The result of this evaluation was that the current proposals of modification of the city for the Pan2007 will be able to generate resulted not deliberate for a considerable parcel of the population and to modify the economic relations harmfully, social and politics inside of the territory of the city. For such we consider measured compensatory satisfactory that they can reach the objectives of an equal citizenship and an equitable equality of chances, starting for the offering of the social minimums of just form. "

Para a construção do espaço geográfico na criança

o tema central da presente monografia é "Para a construção do espaço geográfico na criança". Na realização deste estudo , tentou-se através da análise do conceito do espaço-tempo , da aplicação da epistemologia genética e da pesquisa realizada sobre um espaço concreto-real, aclarar o processo de construção dos conceitos ao nível da ciência e do desenvolvimento da criança, para chegar a uma concepção de espaço, objeto de estudo à Geografia. Essa concepção de espaço, em que ressalta o "aspecto construtivo" do conhecimento poderá ser utilizada nas escolas, possibilitarão ao aluno atuar como sujeito deste processo. A pesquisa quarta séries do 19 foi realizada com alunos de terceira e Grau (quarenta alunos) entre a faixa etária de nove a quatorze anos de idade , de quatro escolas, três escolas públicas municipais e uma escola particular de orientação montessoriana , situadas nos bairros de Ipanema e Copacabana , do Rio de Janeiro, no período de março a dezembro de 1980 . Para análise dos "aspectos figurativos e operatório" relacionados à construção do espaço pelo criança foram construídos instrumentos ligados às atividades escolares e experimentos de J. Piaget adotados para o espaço m::-bano escolhido. Para interpretação dos dados coletados foram empregados estudos e pesquisas realizadas por Jean Piaget. e seus colaboradores, bem como as seguintes contribuições~ de Luquet Piaget sobre o espaço gráfico; de Kelvin Lynch sobre a imagem urbana, e a de Gilberto velho para a comparação dos aspectos ideológicos na representação do bairro de Copacabana.

Análise em tempo real de comportamento explosivo em preços no Brasil

O objetivo deste trabalho é determinar a existência de comportamento explosivo em preços no mercado brasileiro e identificar quando essas explosões ocorreram. Para tanto, foi utilizada uma nova metodologia recursiva de testes de raiz unitária, que identifica início e fim de explosões de preços em tempo real. Foram escolhidos os índices de mercado (Bovespa ajustado pelo dólar e pelo dividend yield do mercado) e a série de preços IGP-DI (taxa de inflação mensal e acumulada em 12 meses) por apresentarem evidências de comportamento explosivo ao longo do tempo. Os resultados obtidos apontaram a existência de comportamentos explosivos nas séries do Ibovespa ajustado pelo dólar nos anos de 1997, 2006 e 2008 (período anterior à quebra do Banco Lehman Brothers) e na taxa de inflação acumulada em 12 meses nas décadas de 80 e 90, previamente ao Plano Real.