163,694 research outputs found
A MOLECULAR EXPLANATION OF SLC25A1 DEFICIENCY RESULTING IN AGENESIS OF CORPUS CALLOSUM AND OPTIC NERVE HYPOPLASIA
No full textMitochondrial carriers (MCs) form a large family of nuclear-encoded transporters embedded in the inner mitochondrial membrane and in a few cases in other organelle membranes (Palmieri, 2013). The members of this superfamily are widespread in eukaryotes and involved in numerous metabolic pathways and cell functions. They can be easily recognized by their striking sequence features, i.e., a tripartite structure, six transmembrane α-helices and a 3-fold repeated signature motifs. Members of the family vary greatly in the nature and size of their transported substrates, modes of transport (i.e., uniport, symport or antiport) and driving forces, although the molecular mechanism of substrate translocation may be basically the same. In recent years mutations in the MC genes have been shown to be responsible for 11 diseases (Palmieri, 2013), highlighting the important role of MCs in metabolism. MC impairing mutations affect three main regions crucial for substrate translocation. A first group of mutations affects MC conformational changes and locates at PG levels or at the aromatic belts (Pierri et al., 2013). A second group of mutations affects substrate specificity and locates at the common substrate binding site (Robinson et al., 2008) and at the substrate binding area (Pierri et al., 2013). A further group of mutations locate at residues of the m-/c-gates (Palmieri et al., 2013; Robinson et al., 2008) and at residues of the m-gate area (Pierri et al. 2013). For this last group of mutations, it appears difficult to establish if the impaired function is due to the lack of substrate specificity (or substrate recognition) or to the wrong triggering of conformational changes. Two mutations, one at the PG level 1 and one at the common substrate binding site, impairing citrate translocation within SLC25A1_CTP protein are presented. The two mutations are found to be responsible of agenesis of corpus callosum and optic nerve hypoplasia (Edvardson et al., 2013).
References
1. Palmieri F. The mitochondrial transporter family SLC25: identification, properties and physiopathology. Mol Aspects Med. 2013;34:465.
2. Pierri CL, Palmieri F, De Grassi A. Single-nucleotide evolution quantifies the importance of each site along the structure of mitochondrial carriers. Cell Mol Life Sci. 2013.
3. Robinson AJ, Overy C, Kunji ER. The mechanism of transport by mitochondrial carriers based on analysis of symmetry. Proc Natl Acad Sci U S A. 2008;105:17766.
4. Edvardson S, Porcelli V, Jalas C, Soiferman D, Kellner Y, Shaag A, Korman SH, Pierri CL, Scarcia P, Fraenkel ND, Segel R, Schechter A, Frumkin A, Pines O, Saada A, Palmieri L, Elpeleg O. Agenesis of corpus callosum and optic nerve hypoplasia due to mutations in SLC25A1 encoding the mitochondrial citrate transporter. J Med Genet. 2013;50:240
Turbocharger speed monitoring based on vibration measurements for diesel engine management
No full text
The Latin jazz Eddie Palmieri : identity and dialogue
No full textOrientadores: Marcelo Gimenes, Antônio Rafael Carvalho dos SantosDissertação (mestrado) - Universidade Estadual de Campinas, Instituto de ArtesResumo: Esta pesquisa tem como objetivo investigar aspectos da construção do latin jazz de Eddie Palmieri. Para tal propõe descrever suas características como estilo musical: estruturação musical, relações com o jazz e com a música afro-cubana, elementos culturais e sociais. Um estudo mais detalhado enfocou a música "Palmas" procurando descrever o procedimento de Palmieri na manipulação de duas linguagens musicais, o jazz e o afro-cubano. Um estudo preliminar sobre a organização rítmica na tradição musical africana foi realizado para melhor compreensão da estrutura rítmica do objeto analisado. Eddie Palmieri é pianista, compositor e arranjador. Com mais de 50 anos atuando no cenário da música latina traz em sua obra uma característica peculiar, o uso do idioma jazzístico num diálogo intenso com elementos rítmicos da música afro-cubana. Este trabalho demonstra como Eddie Palmieri manipula essas linguagens descrevendo seu estilo através da análise de áudios e partiturasAbstract: This research aims to investigate aspects of the construction of latin jazz of Eddie Palmieri. For this proposes to describe his characteristics as a musical style: musical structure, relationships with jazz and the afro-cuban music, cultural and social elements. A more detailed study focused on the song "Palmas" trying to describe Palmieri¿s procedure in handling two musical languages, jazz and afro-cuban. A preliminary study on the rhythmic organization in African musical tradition was conducted to better understand the rhythmic structure of the analyzed object. Eddie Palmieri is a pianist, composer and arranger. With over 50 years working in the latin music scene brings to his work a peculiar feature, using the jazz language in an intense dialogue with rhythmic elements of afro-cuban music. This work demonstrates how Eddie Palmieri handles these languages describing his style by analyzing audio and sheet musicMestradoFundamentos TeoricosMestre em Músic
Aurelio Palmieri, O. S. Α., Dositeo patriarca greco di Gerusalemme (1641- 1707).
No full textJugie Martin. Aurelio Palmieri, O. S. Α., Dositeo patriarca greco di Gerusalemme (1641- 1707).. In: Échos d'Orient, tome 13, n°83, 1910. p. 250
A. Palmieri, O. S. Α., Il progresso dommatico nel concetto cattolico.
No full textJugie M. A. Palmieri, O. S. Α., Il progresso dommatico nel concetto cattolico. . In: Échos d'Orient, tome 13, n°84, 1910. p. 314
Le novelle maremmane
No full textLe novelle maremmane / di Antonio Palmieri. - Milano : Treves, 1907
Dedica manoscritta: All\u27a.e.Z. / a.e.Z. / 6 ott.bre 1907. Montalto
https://galileodiscovery.unipd.it/discovery/fulldisplay?context=L&vid=39UPD_INST:VU1&search_scope=MyInst_and_CI&tab=Everything&docid=alma99000228622020604
Il meraviglioso in età moderna. Dimensioni culturali, scientifiche e religiose
No full textIl volume propone un lavoro di indagine sul meraviglioso in età moderna in una prospettiva interdisciplinare, cercando di tenere insieme studi di storia religiosa, politica e culturale, storia della scienza e storia del diritto. Uno degli obiettivi cruciali è far luce sui meccanismi di gestione del “meraviglioso” e del “miracoloso” sviluppati dai poteri costituiti, attraverso organismi istituzionali destinati a governare la circolazione di idee, saperi, credenze. Tuttavia l’evoluzione delle ricerche ha fatto emergere l’importanza di diversi contesti fluidi, nei quali i dettami o gli orientamenti delle autorità si trovavano a interagire con forme creative di naturalismo, di “interesse” per la natura, o di
interazione con la natura. Sono di volta in volta i filosofi, i romanzieri, i
drammaturghi, i collezionisti, gli incantatori, gli impostori, i predicatori e i
devoti a far proprie le immagini di mostri, pietre preziose, tesori nascosti,
pozioni taumaturgiche per accendere le passioni del pubblico o per stimolare dibattiti. Con le loro iniziative, tutti questi soggetti si rivelano capaci
di offrire il loro contributo attivo nel modificare il disegno – inteso come
rappresentazione culturalmente condivisa – dell’ordine del mondo
Variations on the Author
No full text“Variations on the Author” discusses two of Eduardo Coutinho’s recent films (Um Dia na Vida, from 2010, and Últimas Conversas, posthumously released in 2015) and their contribution to the general question of documentary authorship. The director’s filmography is characterized by a consistent yet self-effacing form of authorial self-inscription: Coutinho often features as an interviewer that rather than express opinions propels discourses; an interviewer that is good at listening. This mode of self-inscription characterizes him as an author who is not expressive but who is nonetheless markedly present on the screen. In Um Dia na Vida, however, Coutinho is completely absent form the image, while Últimas Conversas, on the contrary, includes a confessional prologue that moves the director from the margins to the center of his films. This article examines the ways in which these works stand out in the filmography of a director who offers new insights into the notion of cinematic authorship
Performance Assessment of Hydraulic Micro Relief Valve
No full textDirectly operated relief valves often exhibit abnormal or unstable behavior resulting in noise, premature wear, degraded performance, and failure. In this work, an investigation is carried out to assess the role of the hydraulic damper on the poppet element. The article reports the details of the investigation approach, integrating specific experimental tests with lumped-parameter modelling.Starting from a reference layout, the hydraulic behavior of different damper configurations is characterized (ranging from undamped to strongly damped poppets), for pressure and flow-rate conditions typically encountered in micro-hydraulic power packs. Depending on the damper design, the poppet operation ranges from unacceptability (due to noise) to fine-tuned response. The investigation give the chance to highlight how the damper layout impacts on the valve behaviour, in terms of pressure overshoot and settling time and provides the necessary information for relief valve design, modelling and analysis
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