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91.
A variant phenotype of nonketotic hyperglycinemia has been described by our group associated with pulmonary hypertension. The aim of this study is to investigate the cerebrospinal fluid proteomes to get an insight into this neurodegenerative process producing leukoencephalopathy with white matter spongiform degeneration. DIGE and MALDI-TOF-TOF analyses were performed to carry out the proteomic study of four patients against three normal controls and one additional control of a classical nonketotic hyperglycinemia. The differential proteomic analysis showed a displacement of some series of spots toward the acidic side. The shifted proteins showed a high degree of carbonylation and increased methionine sulfoxidation was found in cystatin C and in vitamin-D-binding protein. These findings in addition to the increase of serum malondialdehyde concentration provide evidence of an oxidative stress in the patients under study, which is probably systemic rather than mainly confined to the CNS. The similarities of our findings with those found in other neurodegenerative diseases suggest that oxidative damage is commonly involved in these pathologies. DIGE technology improves the 2-D PAGE differential analysis and it is suitable in proteomic studies with a small number of cases.  相似文献   
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The pollen cone and the pollen grain of the two Argentinean species of Araucaria are described with LM, SEM and TEM. Primordia of pollen cones are formed in April and May and reach maturity by mid-October in A. angustifolia (Bert.) O. Kuntze and by mid-November in A. araucana. (Mol.) K. Koch. Characters of the mature pollen cones and microsporophylls between both taxa are clearly differentiated. Pollen grains are spheroidal-subspheroidal, inaperturate, and asaccate with granulate exine and a subequatorial annular area that corresponds to the sexine thickness. Sculpturing consists of irregularly dispersed granules that are sometimes fused to each other (A. angustifolia) or forming microrugulae (A. araucana). Microgranules and microspinules are also present. The pollen wall ultrastructure is formed by a granular ectexine and lamellated endexine. Granular elements in A. angustifolia are more loosely disposed, form more interstices, and are gradually smaller towards the endexine than in A. araucana. To asses the probable relationships within the family, we compared the pollen grains of the two Araucaria species with those of other extant genera (Agathis, Wollemia) and also with fossil pollen (Araucariacites, Balmeiopsis, Cyclusphaera, Dilwynites) attributed to Araucariaceae.  相似文献   
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The paper deals with the global stabilization of both the attitude and the angular velocities of an underactuated rigid body. First a stability theorem is proven for a class of systems; subsequently, the equations describing the physics of the rigid body are presented, showing that the rigid body belongs to the considered class of systems, and a sufficient condition for the application of the theorem to the stability of the rigid body equilibrium is pointed out. Finally, some simulation results are reported showing the effectiveness of the proposed methodology.  相似文献   
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Virtual Reality - With the high growth and prosperity of e-commerce, the retail industry needs to explore new technologies that improve digital shopping experiences. In the current technological...  相似文献   
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Applied Intelligence - Forecasting future heat load in smart district heating networks is a key problem for utility companies that need such predictions for optimizing their operational activities....  相似文献   
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This paper describes an inverse procedure to determine the constitutive constants and the friction conditions in the machining processes using Finite Elements (FE) simulations. In general, the FE modeling of machining processes is an effective tool to analyze the materials machinability under different cutting conditions. However, the use of reliable rheological and friction models represents the basis of a correct numerical investigation. The presented inverse procedure was based on the numerical results obtained using a commercial FE code and was developed considering a specific optimization problem, in which the objective functions that have to be minimized is the experimental/numerical error. This problem was performed by a routine developed in a commercial optimization software. In order to verify the goodness and the robustness of the methodology, it was applied on a Super Duplex Stainless Steel (SDSS) and on an Austenitic Stainless Steel (AUSS) orthogonal machining processes. This work, then, was focused on the identification of the Johnson-Cook (JC) coefficients (A,B,C, n and m) and on the calibration of a Coulomb friction model, in the specific cases of the SAF2507 SDSS and of an AISI 316 Based AUSS Alloy (AISI 316 ASBA). The identification phases were performed considering forces and temperatures experimental data, collected in two specific experimental tasks in which different orthogonal cutting tests were carried out under different cutting parameters conditions.  相似文献   
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We present three velocity‐based updated Lagrangian formulations for standard and quasi‐incompressible hypoelastic‐plastic solids. Three low‐order finite elements are derived and tested for non‐linear solid mechanics problems. The so‐called V‐element is based on a standard velocity approach, while a mixed velocity–pressure formulation is used for the VP and the VPS elements. The two‐field problem is solved via a two‐step Gauss–Seidel partitioned iterative scheme. First, the momentum equations are solved in terms of velocity increments, as for the V‐element. Then, the constitutive relation for the pressure is solved using the updated velocities obtained at the previous step. For the VPS‐element, the formulation is stabilized using the finite calculus method in order to solve problems involving quasi‐incompressible materials. All the solid elements are validated by solving two‐dimensional and three‐dimensional benchmark problems in statics as in dynamics. Copyright © 2016 John Wiley & Sons, Ltd.  相似文献   
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