3,191 research outputs found
Sjögren-Larsson syndrome: report of monozygote twins and a case with a novel mutation
No full textSjögren-Larsson syndrome is an autosomal recessive neurocutaneous disease caused by mutations in the ALDH3A2 gene for fatty aldehyde dehydrogenase, a microsomal enzyme that catalyzes the oxidation of medium- and long-chain aliphatic aldehydes fatty acids. We studied three Turkish Sjögren-Larsson syndrome patients with ichthyosis, developmental delay, spastic diplegia, and brain white matter disease. One patient was homozygous for a novel ALDH3A2 mutation in exon 5. The mutation involves the codon 228 (CGC) with the transversion G->A modifying the codon in CAC, leading to the substitution of the original arginine with a histidine (R228H), modifying the stereospecific properties of this region. These results add to the understanding of the genetic basis of Sjögren-Larsson syndrome and will be useful for DNA diagnosis of this disease
A imagem de Alessandro Baricco no Brasil
No full textDissertação (mestrado) - Universidade Federal de Santa Catarina, Centro de Comunicação e Expressão, Programa de Pós-Graduação em Estudos da Tradução, Florianópolis, 2013.Com a intenção de delinear o modo pelo qual o escritor italiano Alessandro Baricco se inseriu no sistema literário brasileiro e os caminhos percorridos pelos seus livros traduzidos, esta dissertação dá voz às experiências tradutórias de seus tradutores. A inserção de Bariccono Brasil tem seu início em 1997, através de uma proposição da Profa. Dra. Roberta Barni à editora Iluminuras da tradução de Oceano Mare. A partir daí, outras sete obras foram publicadas no Brasil, sendo três delas traduzidas por Roberta Barni e as outras quatro por quatro tradutores diferentes. De um lado, considera-se o tradutor como figura principal namediação entre culturas, e, de outro, se analisa a realidade desta figuradentro do sistema literário, sua invisibilidade, seus limites e o exercíciode sua profissão. A pesquisa conta, ainda, com críticas e resenhas referentes ao autor italiano publicadas em jornais consagrados no Brasil, considerando estas como parte constituinte da imagem de Baricco refletida em território nacional. Abstract : Intending to delineate the way the Italian writer Alessandro Baricco has been inserted in the Brazilian literary system and the paths his translated books have followed, this thesis gives voice to the translating experiences of his translators. Baricco's insertion in Brazil began in 1997, through a personal project of Dr. Roberta Barni, with her translation of Oceano Mare. Since then, seven other of his works have been published in Brazil, three of which were translated by Roberta Barni and the other four by four different translators. On the one hand,the translator is considered as the main figure in mediation betweencultures and, on the other, this figure's reality is analyzed within theliterary system: its invisibility, its limits and its professional practice. Criticisms and reviews of this Italian author published in well established Brazilian newspapers are also considered, with the understanding that they are part of Baricco's image reflected here
La maturità di Alessandro Fei del Barbiere, in bilico tra Maniera e Riforma
No full textThis article studies the mature career of the Florentine painter Alessandro Fei del Barbiere (1537-1592), beginning with the rediscovery of the 'Ascension' altarpiece formerly in the Albizi Chapel in the destroyed church of San Pier Maggiore, Florence. Studying this painting and others recorded in 1584 by the biographer Raffaello Borghini, such as the two altarpieces for Santa Maria delle Grazie and the Madonna dell'Umiltà in Pistoia, the author reconstructs a body of works showing how in the 1580s Fei gradually went beyond the archaic style of his apprenticeship - he had been trained by Ridolfo del Ghirlandaio and Pierfrancesco Foschi, but was also marked by the Maniera of Vasari - evolving towards naturalism in both mimesis and pictorial handling. In Florence, his development partly parallels that of Santi di Tito and his circle, but Fei was also influenced by a probable sojourn during the early part of that decade in Rome, where he could have been inspired by Girolamo Muziano and the painters working for Pope Gregory XIII. Among other proposals, the author suggests that the artist was responsible for decorating the chancel of Fiesole Cathedral (c. 1584-1589), which consisted of an altarpiece, only rarely discussed by scholars, and a cycle of frescoes hitherto attributed to Nicodemo Ferrucci
Tra socialdemocrazie e Perestrojka. Le relazioni internazionali del Pci attraverso le carte di Alessandro Natta
No full textThis essay reconstructs the foreign policy of the Italian Communist Party during the four
years of Alessandro Natta’s secretariat (1984-1988) through largely original archival documentation,
from the Alessandro Natta Fonds of the Historical Archive of the Chamber of
Deputies. Natta’s papers are also cross-referenced with those kept in the PCI Archive at the
Gramsci Foundation. The author analyses the relationship that the PCI establishes with
Gorbachev’s Perestroika, Deng’s China, and European social democracies during the last
years of the Cold War
Volta Alessandro
No full textThe New Dictionary of Scientific Biography (extension and updating of the Dictionary of Scientific Biography) is one of the most substantial reference works in the field of the history of science. An up-to-date overview on Alessandro Volta's science with new perspectives offered by the author. Information and critical analysis is also provided on the main secondary literature produced on this main protagonist of enlightenment science after the 1976 entry "Volta Alessandro" in the Dictionary of Scientific Biography
Impact of Different Illuminance Typical Years Models on a Climate Based Method for the Calculation of Artificial Lighting Energy Use in Office Buildings
No full textArtificial lighting has a relevant impact on the electricity uses in not residential buildings. The method to assess such uses is based on standards, which hardly take into account the daylighting contribution and the time evolution of the outdoor illuminance conditions. Different models were adopted to build diffuse illuminance reference years, starting from satellite images. These models lead to different daylight availability during the year. The paper explores the impact that these models have on the artificial lighting energy uses by hourly monthly mean calculations. The test was carried out with a climate based method, which takes into account: the outdoor luminous environment, the reference indoor visual task and the building daylight characteristics. A typical office building was used for the test in Rome, Italy. Results allowed to compare the impact of each model and to select the most suitable one to be implemented in the climate based method to predict artificial lighting use in buildings
"Muy señor nuestro Alessandro Varaldo". La ricognizione del mondo spagnolo e portoghese per riscoprire un autore italiano di successo ma dimenticato
No full textWho is Alessandro Varaldo (1876-1953)? This first analysis about what was said, adapted and translated of Varaldo’s work in the Iberian Peninsula and South America, maybe the only areas outside Italy where some traces of Italian author can be found, is a chance to go along his literary paths that overtook two temporal barriers (1900 and 1950), although it has not overtaken the oblivion’s threshold.Questa è una prima ricognizione di ciò che è stato detto, adattato e tradotto di Alessandro Varaldo (1876-1953) nella Penisola iberica e in Sud America, che si sospetta siano gli unici territori stranieri dove si riesca a ritracciare qualcosa dell’autore italiano, ed è un’occasione per ripercorrerne la traiettoria letteraria che ha superato due transenne temporali come il 1900 e il 1950 ma non quella della dimenticanza
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