La estética mas��nica en Lisboa: nuevas perspectivas para historiar la cuidad

Este trabajo parte de una perspectiva hol��stica y multidisciplinar, tiene como principal objetivo proponer l��neas de an��lisis de la estética mas��nica en la ciudad de Lisboa, principalmente a trav��s de su patrimonio arquitect��nico para dar respuesta a numerosas cuestiones art��sticas o socio-culturales presentes en ella y carentes de estudio. Desde el siglo xviii, la capital portuguesa presenta una serie de manifestaciones art��sticas en el espacio urbano vinculadas a la estética mas��nica. Arquitectos, comitentes y dirigentes pol��ticos adscritos a la Orden del Gran Arquitecto del Universo han intervenido de forma simb��lica en la ciudad. C��digos herm��ticos que deben ser teorizados desde la historia del arte con la m��xima cientificidad posible. El objetivo de este ensayo es dar a conocer las pautas metodol��gicas y aproximarnos al complejo campo simb��lico en la ciudad lisboeta a trav��s de algunos ejemplos paradigm��ticos.

Cuerpos abiertos, cineastas del corte. Una introducci��n al corte como dispositivo

La figuraci��n del cuerpo en el cine ha sido tambi��n la figuraci��n de su apertura, de la visualizaci��n de lo interno como un universo misterioso y siniestro. Pocos cineastas han vuelto su mirada hacia lo que esconde la piel de sus personajes. Se tratar�� de poner en escena una constelaci��n de cineastasque han hecho del corte el instrumento para hacer visible lo invisible de lo humano: David Lynch, Georges Franju, Alfred Hitchcok y Marina de Van. Asimismo, el corte es un elemento que define la imagen del cine, en tanto que es capaz de unir y sumar dos im��genes para formar un cuerpo. La idea delcorte y el intervalo como aquello ��nico en Jean-Luc Godard se estudiar�� a partir de ���Pasi��n���, una pel��cula considerada como filme intersticial en su corpus cinematogr��fico y filme nuclear en su voluntad de ver aquello que a��n no se ve.

Genetic and phenotypic characterization of complex hereditary spastic paraplegia

The hereditary spastic paraplegias are a heterogeneous group of degenerative disorders that are clinically classified as either pure with predominant lower limb spasticity, or complex where spastic paraplegia is complicated with additional neurological features, and are inherited in autosomal dominant, autosomal recessive or X-linked patterns. Genetic defects have been identified in over 40 different genes, with more than 70 loci in total. Complex recessive spastic paraplegias have in the past been frequently associated with mutations in SPG11 (spatacsin), ZFYVE26/SPG15, SPG7 (paraplegin) and a handful of other rare genes, but many cases remain genetically undefined. The overlap with other neurodegenerative disorders has been implied in a small number of reports, but not in larger disease series. This deficiency has been largely due to the lack of suitable high throughput techniques to investigate the genetic basis of disease, but the recent availability of next generation sequencing can facilitate the identification of disease- causing mutations even in extremely heterogeneous disorders. We investigated a series of 97 index cases with complex spastic paraplegia referred to a tertiary referral neurology centre in London for diagnosis or management. The mean age of onset was 16 years (range 3 to 39). The SPG11 gene was first analysed, revealing homozygous or compound heterozygous mutations in 30/97 (30.9%) of probands, the largest SPG11 series reported to date, and by far the most common cause of complex spastic paraplegia in the UK, with severe and progressive clinical features and other neurological manifestations, linked with magnetic resonance imaging defects. Given the high frequency of SPG11 mutations, we studied the autophagic response to starvation in eight affected SPG11 cases and control fibroblast cell lines, but in our restricted study we did not observe correlations between disease status and autophagic or lysosomal markers. In the remaining cases, next generation sequencing was carried out revealing variants in a number of other known complex spastic paraplegia genes, including five in SPG7 (5/97), four in FA2H (also known as SPG35) (4/97) and two in ZFYVE26/SPG15. Variants were identified in genes usually associated with pure spastic paraplegia and also in the Parkinson���s disease-associated gene ATP13A2, neuronal ceroid lipofuscinosis gene TPP1 and the hereditary motor and sensory neuropathy DNMT1 gene, highlighting the genetic heterogeneity of spastic paraplegia. No plausible genetic cause was identified in 51% of probands, likely indicating the existence of as yet unidentified genes.

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.

A Competitive assessment of the U.S. power tool industry /

"May 1992."

A competitive assessment of the U.S. paper machinery industry /

Mode of access: Internet.

O metacinema norte-americano e o storytelling : de Orson Welles a David Lynch

ABSTRACT: The American metacinema which, by tradition, is narrative but extremely formulaic favours the story above the telling. No man contributed more to alter this state of affairs than Orson Welles, whose cinematic practice exalted the filmic enunciation and linked it more explicitly to the narrative intentions of the creator, making it obvious that metanarrative is synonymous with metacinema. With Citizen Kane (1941), in particular, the cinema was made more disnarrative, as meant by French writer/director Alain Robbe���Grillet, well ahead of Modernism. The fragmented narration, the temporal convolutions, a tendency for paradox and the interpretative obstacles all come together to anticipate the serial practice of David Lynch in his last four features. Structuring the films in segments which constitute different but complementary versions of the same events, Lynch manages to express the director���s enunciation along with the narration of the characters, reinforcing the role of the telling in the midst of the story. In INLAND EMPIRE (2006) the use of mise-en-abyme as a way of duplicating stories and tellers further increases the objective and complicates what is clearly the reign of the puzzle or mind-game film. In the footsteps of Welles, Lynch contributed for an outbreak of metanarrative / metacinematic crossover indie films closer to the European aesthetic practice but still very much within the American narrative tradition.

O espectador (in)vis��vel: reflexividade na ��ptica do espectador em Inland empire de David Lynch

Com INLAND EMPIRE (2006) David Lynch realiza uma obra inteiramente metacinematogr��fica, provando que este g��nero transversal �� Hist��ria do cinema �� pass��vel de coexistir com uma po��tica arrojada, de pendor moderno, segundo a divis��o que Gilles Deleuze faz da S��tima Arte. A produ����o te��rica sobre esta vertente f��lmica de Lynch �� em termos internacionais muito escassa e em Portugal virtualmente inexistente, justificando que se proceda um trabalho de investiga����o nestes moldes. Ademais, INLAND EMPIRE n��o �� um exemplo isolado na obra do cineasta, mas sim o ponto culminante de uma carreira que tem vindo a prestar cada vez mais aten����o �� tem��tica meta-espectatorial. A abordagem de Lynch tem a particularidade de congregar num ��nico trabalho o desejo de manifestar o vidente espelhando igualmente o filme, o que permite abordar INLAND EMPIRE como reflexividade na ��ptica do espectador. Trata-se de encenar uma alegoria do vidente, mas equacionando produ����o com recep����o. Deste modo, o presente livro adopta o mecanismo da enuncia����o como base da reflexividade institucional e narrativa em duas sec����es te��ricas distintas versando, respectivamente, a (re)duplica����o especular e a mise en abyme. A cada um destes cap��tulos corresponde uma an��lise pr��tica directamente relacionada com os factores te��ricos apontados. A obra culmina com um areflex��o sobre as implica����es do cinema digital para a execu����o de alegorias cinematogr��ficas e a sua influ��ncia em INLAND EMPIRE, que j�� foi inteiramente produzido com esta tecnologia.

Letters from David Lynch to William Tudor,

Three letters regarding trade in Dutch Guyana.

Experi��ncia religiosa e experi��ncia estética em artistas pl��sticos : perspectivas da psicologia da religi��o

Pode a experi��ncia religiosa ser substitu��da pela experi��ncia estética? Os estudiosos se dividem na resposta. Situando a pergunta no quadro de refer��ncia conceitual e epistemol��gico que distingue entre religi��o substantiva e religi��o funcional, levantamos a hip��tese de que n��o h�� substitui����o substantiva, mas pode haver substitui����o funcional entre essas experi��ncias. Levantamos a hip��tese ulterior de que a experi��ncia estética pode ser uma experi��ncia do sagrado. As hip��teses foram examinadas com os dados obtidos em entrevistas com oito renomados artistas pl��sticos, por ocasi��o da 23�� Bienal de S��o Paulo, em 1996. Os dados revelaram diferen��as, continuidades e analogias entre arte e religi��o assim como entre arte e sagrado, que permitiram reconhecer em alguns casos substitui����o funcional da religi��o pela arte, principalmente se dotada da densidade do sagrado.

An��lise est��tica e din��mica de pain��is corrugados constru��dos em materiais comp��sitos

Os pain��is corrugados t��m cada vez maior aplicabilidade em engenharia. S��o frequentemente utilizados em estruturas sandwich, em telhados e t��m muitas outras aplica����es nas ind��strias civil, mec��nica, aeron��utica e naval. Esta crescente aplica����o deve-se ao facto de as suas corruga����es conferirem maior rigidez ao painel devido ao aumento do r��cio resist��ncia/peso, evitando-se assim o recurso a refor��os estruturais. Este facto, associado ��s caracter��sticas dos materiais comp��sitos laminados, que comparativamente com os materiais ���tradicionais���, evidenciam uma rela����o rigidez/peso, bem como um comportamento mec��nico; no mesmo sentido, contribui para uma potencial utiliza����o deste tipo de solu����o, em variad��ssimas situa����es na ��rea das engenharias. Este tipo de pain��is �� particularmente adequado em situa����es de carga em que se verificam esfor��os de compress��o e tor����o. Com o presente trabalho, pretende-se efectuar um estudo de an��lise do comportamento mec��nico, quer em termos de an��lise est��tica linear, quer de vibra����es livres de um painel corrugado em material comp��sito, sendo analisadas v��rias vari��veis, nomeadamente: o tipo de material, a geometria da corruga����o, a sequ��ncia de empilhamento das camadas de fibra, entre outras. Para este efeito, e ap��s o enquadramento do tipo de problema que se possui, �� utilizado o software comercial de an��lise por elementos finitos, Ansys��.

Transatlantic Influence in the Shaping of Business Education: The Origins of IMD, 1946-1990

The history of the International Institute for Management Development (IMD), one of the most prestigious business schools in the world, highlights the role of multinationals in establishing business education in Europe and the problem of legitimacy. The creation of IMD's predecessors CEI and IMEDE by Alcan and Nestl�� also illuminates the role of Harvard Business School in their development and the reciprocal influences of American and European management education after World War II.