992 resultados para Niemann Pick C1
Resumo:
The aim of this contribution is to present a theoretical approach and two experimental campaigns (on wind tunnel and on the track) concerning the research work about the ballast train-induced-wind erosion (BTIWE) phenomenon. When a high speed train overpasses the critical speed, it produces a wind speed close to the track large enough to start the motion of the ballast elements, eventually leading to the rolling of the stones (Kwon and Park, 2006) and, if these stones get enough energy, they can jump and then initiate a saltation-like chain reaction, as found in the saltation processes of soil eolian erosion (Bagnold, 1941). The expelled stones can reach a height which is larger than the lowest parts of the train, striking them (and the track surroundings) producing considerable damage that should be avoided. There is not much published work about this phenomenon, in spite of the great interest that exists due to its relevant applications in increasing the maximum operative train speed. Particularly, the initiation of flight of ballast due to the pass of a high speed train has been studied by Kwon and Park (2006) by performing field and wind tunnel experiments.
Resumo:
After a short introduction the possibilities and limitations of polynomial simple elements with C1 continuity are discussed with reference to plate bending analysis. A family of this kind of elements is presented.. These elements are applied to simple cases in order to assess their computational efficiency. Finally some conclusions are shown, and future research is also proposed.
Resumo:
Es una aplicación del método de los elementos finitos (M.E.F) al cálculo de losas delgadas isotrópicas. Es pues un desarrollo de la función solución en suma de funciones a trozos. Dentro del M.E.F se utilizan como funciones interpolantes polinomios (integración numérica sencilla). La continuidad conseguida es C elevado a 1 (para el caso planteado representa convergencia monotónica ). Son elementos simples (de fácil extensión a láminas) y que forman una familia jerárquica (distintos grados de aproximación sin cambiar la malla). El primer elemento de la familia es el clough- felippa. Al final se dan resultados comparativos de algunas placas con otro tipo de elementos y la solución exacta.
Resumo:
This paper describes the experimental setup, procedure, and results obtained, concerning the dynamics of a body lying on a floor, attached to a hinge, and exposed to an unsteady flow, which is a model of the initiation of rotational motion of ballast stones due to the wind generated by the passing of a high-speed train. The idea is to obtain experimental data to support the theoretical model developed in Sanz-Andres and Navarro-Medina (J Wind Eng Ind Aerodyn 98, 772–783, (2010), aimed at analyzing the initial phase of the ballast train-induced-wind erosion (BATIWE) phenomenon. The experimental setup is based on an open circuit, closed test section, low-speed wind tunnel, with a new sinusoidal gust generator mechanism concept, designed and built at the IDR/UPM. The tunnel’s main characteristic is the ability to generate a flow with a uniform velocity profile and sinusoidal time fluctuation of the speed. Experimental results and theoretical model predictions are in good agreement.
Resumo:
Electrophysiological, morphological, and biochemical approaches were combined to study the effect of the presynaptic injection of the light chain of botulinum toxin C1 into the squid giant synapse. Presynaptic injection was accompanied by synaptic block that occurred progressively as the toxin filled the presynaptic terminal. Neither the presynaptic action potential nor the Ca2+ currents in the presynaptic terminal were affected by the toxin. Biochemical analysis of syntaxin moiety in squid indicates that the light chain of botulinum toxin C1 lyses syntaxin in vitro, suggesting that this was the mechanism responsible for synaptic block. Ultrastructure of the injected synapses demonstrates an enormous increase in the number of presynaptic vesicles, suggesting that the release rather than the docking of vesicles is affected by biochemical lysing of the syntaxin molecule.
Resumo:
Niemann–Pick type C1 (NPC1) disease results from a defect in the NPC1 protein and is characterized by a pathological accumulation of cholesterol and glycolipids in endocytic organelles. We followed the biosynthesis and trafficking of NPC1 with the use of a functional green fluorescent protein-fused NPC1. Newly synthesized NPC1 is exported from the endoplasmic reticulum and requires transit through the Golgi before it is targeted to late endosomes. NPC1-containing late endosomes then move by a dynamic process involving tubulation and fission, followed by rapid retrograde and anterograde migration along microtubules. Cell fusion studies with normal and mutant NPC1 cells show that exchange of contents between late endosomes and lysosomes depends upon ongoing tubulovesicular late endocytic trafficking. In turn, rapid endosomal tubular movement requires an intact NPC1 sterol-sensing domain and is retarded by an elevated endosomal cholesterol content. We conclude that the neuropathology and cellular lysosomal lipid accumulation in NPC1 disease results, at least in part, from striking defects in late endosomal tubulovesicular trafficking.
Resumo:
Since most of the examples of "exon shuffling" are between vertebrate genes, the view is often expressed that exon shuffling is limited to the evolutionarily recent lineage of vertebrates. Although exon shuffling in plants has been inferred from the analysis of intron phases of plant genes [Long, M., Rosenberg, C. & Gilbert, W. (1995) Proc. Natl. Acad. Sci. USA 92, 12495-12499] and from the comparison of two functionally unknown sunflower genes [Domon, C. & Steinmetz, A. (1994) Mol. Gen. Genet. 244, 312-317], clear cases of exon shuffling in plant genes remain to be uncovered. Here, we report an example of exon shuffling in two important nucleus-encoded plant genes: cytosolic glyceraldehyde-3-phosphate dehydrogenase (cytosolic GAPDH or GapC) and cytochrome c1 precursor. The intron-exon structures of the shuffled region indicate that the shuffling event took place at the DNA sequence level. In this case, we can establish a donor-recipient relationship for the exon shuffling. Three amino terminal exons of GapC have been donated to cytochrome c1, where, in a new protein environment, they serve as a source of the mitochondrial targeting function. This finding throws light upon an old important but unsolved question in gene evolution: the origin of presequences or transit peptides that generally exist in nucleus-encoded organelle genes.
Resumo:
Heuristics for stochastic and dynamic vehicle routing problems are often kept relatively simple, in part due to the high computational burden resulting from having to consider stochastic information in some form. In this work, three existing heuristics are extended by three different local search variations: a first improvement descent using stochastic information, a tabu search using stochastic information when updating the incumbent solution, and a tabu search using stochastic information when selecting moves based on a list of moves determined through a proxy evaluation. In particular, the three local search variations are designed to utilize stochastic information in the form of sampled scenarios. The results indicate that adding local search using stochastic information to the existing heuristics can further reduce operating costs for shipping companies by 0.5–2 %. While the existing heuristics could produce structurally different solutions even when using similar stochastic information in the search, the appended local search methods seem able to make the final solutions more similar in structure.
Resumo:
Objetivou-se avaliar a suplementação dietética de ácidos graxos saturados e insaturados sobre o metabolismo, e desempenhos produtivo e reprodutivo no período de transição e início de lactação de vacas leiteiras. Foram utilizadas 36 vacas da raça holandesa distribuídas aleatoriamente para receber uma das três dietas experimentais. No período pré-parto as dietas foram: Controle (CON), sem adição de gordura e 2,8% de extrato etéreo baseado na matéria seca; Gordura Saturada (SAT), com inclusão de 2,4% de MAGNAPAC® (Tectron Ltda.) com 4,7% de EE baseado na MS; Gordura Insaturada (INS), com inclusão de 11% de grão de Soja, com 4,7% de EE baseado na MS. No período pós-parto, Controle (CON), sem adição de gordura e com 2,8% de EE baseado na MS; Gordura Saturada (SAT), com inclusão de 2,6% de MAGNAPAC® (Tectron Ltda.) com 5,0% de EE baseado na MS; Gordura Insaturada (INS), com inclusão de 13% de Grão de Soja, com 5% de EE baseado na MS. As dietas foram fornecidas 35 dias da data prevista do parto até 90 dias de lactação. No período pré-parto foi utilizada silagem de milho como volumoso, em uma relação volumoso:concentrado de 70:30, enquanto que no período pós-parto foram utilizados 5% de feno de tifton e 45% de silagem de milho como fontes de volumoso, com uma relação entre volumoso:concentrado de 50:50. A produção de leite foi mensurada diariamente durante todo o período experimental. As amostras utilizadas para análise da composição e o perfil de ácidos graxos do leite foram coletadas semanalmente, sendo provenientes das duas ordenhas diárias. As amostras de sangue para análise dos metabólitos sanguíneos foram coletadas semanalmente. Do dia 14 ao dia 90 pós-parto foi realizado avaliação da dinâmica folicular por ultrassonografia. Nos dias 30, 60 e 90 foram realizadas aspirações foliculares, com posterior fertilização in vitro dos oócitos. Todas as variáveis mensuradas foram avaliadas pelo procedimento PROC MIXED do SAS (2004) utilizando-se os seguintes contrastes ortogonais: Controle vs Fontes de Lipídeo (C1); Fonte de ácidos graxos saturados x Fonte de ácidos graxos insaturados (C2). Foi utilizado nível de 5% de significância. No período pós-parto, a suplementação de lipídeos aumentou as concentrações de AGNE quando comparada a dieta CON. O tratamento INS reduziu as concentrações de proteínas totais e de BHB quando comparado ao SAT. Houve interação entre tempo e dieta paras as variáveis colesterol total, LDL e BHB. Houve redução da produção de leite corrigida para 3,5% de gordura, na produção total de gordura e de proteína, e no teor de gordura do leite quando comparado o tratamento INS com o SAT. A suplementação de lipídeo reduziu as concentrações do somatório dos ácidos graxos saturados (Σ A.G. Saturados), dos ácidos graxos com menos de 16 carbonos (>C16), e da relação entre ácidos graxos saturados com insaturados (Σ SFA/(MUFA+PUFA)); e aumentou as concentrações de ácidos graxos acima de 16 carbonos (>C16), de ácidos graxos insaturados com 18 carbonos, da somatória dos ácidos graxos insaturados e dos ácidos graxos poli-insaturados (Σ A.G. Poli-insaturados). O tratamento INS aumentou a concentração de ácidos graxos poli-insaturados totais (Σ A.G. poli-insaturados), e reduziu o total de ácidos graxos de 16 carbonos (C16) em relação ao tratamento SAT. Houve redução no número de folículos classe 1, e folículos totais (NC1 e NTFol) com suplementação de lipídeo. O tratamento SAT aumentou o número de folículos classe 5 (NC5), em relação ao INS. Não houve alteração na qualidade oocitária e embrionária com a suplementação de lipídeo e entre as duas fontes de lipídeo. A suplementação de lipídeos insaturados através da suplementação via grão de soja cru e integral, quando comparada à suplementação de lipídeos saturados, para vacas no período de transição e início de lactação, não interferiu na dinâmica folicular e qualidade oocitária e embrionária; e reduziu o desempenho produtivo, devido às reduções na produção de gordura do leite a na produção de leite corrigida para 3,5% dos animais suplementados
Resumo:
The 14C reservoir age of the surface ocean was determined for two Holocene periods (4908-4955 and 3008-3066 calendar (cal) B.P.) using U/Th-dated corals from Biscayne National Park, Florida, United States. We found that the average reservoir ages for these two time periods (294 ± 33 and 291 ± 27 years, respectively) were lower than the average value between A.D. 1600 and 1900 (390 ± 60 years) from corals. It appears that the surface ocean was closer to isotopic equilibrium with CO2 in the atmosphere during these two time periods than it was during recent times. Seasonal d18O measurements from the younger coral are similar to modern values, suggesting that mixing with open ocean waters was indeed occurring during this coral's lifetime. Likely explanations for the lower reservoir age include increased stratification of the surface ocean or increased D14C values of subsurface waters that mix into the surface. Our results imply that a more correct reservoir age correction for radiocarbon measurements of marine samples in this location from the time periods ~3040 and ~4930 cal years B.P. is ~292 ± 30 years, less than the canonical value of 404 ± 20 years.
Resumo:
The palaeoenvironmental development of the western Laptev Sea is understood primarily from investigations of exposed cliffs and surface sediment cores from the shelf. In 2005, a core transect was drilled between the Taymyr Peninsula and the Lena Delta, an area that was part of the westernmost region of the non-glaciated Beringian landmass during the late Quaternary. The transect of five cores, one terrestrial and four marine, taken near Cape Mamontov Klyk reached 12 km offshore and 77 m below sea level. A multiproxy approach combined cryolithological, sedimentological, geochronological (14C-AMS, OSL on quartz, IR-OSL on feldspars) and palaeoecological (pollen, diatoms) methods. Our interpretation of the proxies focuses on landscape history and the transition of terrestrial into subsea permafrost. Marine interglacial deposits overlain by relict terrestrial permafrost within the same offshore core were encountered in the western Laptev Sea. Moreover, the marine interglacial deposits lay unexpectedly deep at 64 m below modern sea level 12 km from the current coastline, while no marine deposits were encountered onshore. This implies that the position of the Eemian coastline presumably was similar to today's. The landscape reconstruction suggests Eemian coastal lagoons and thermokarst lakes, followed by Early to Middle Weichselian fluvially dominated terrestrial deposition. During the Late Weichselian, this fluvial landscape was transformed into a poorly drained accumulation plain, characterized by widespread and broad ice-wedge polygons. Finally, the shelf plain was flooded by the sea during the Holocene, resulting in the inundation and degradation of terrestrial permafrost and its transformation into subsea permafrost.