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991.
Adenosine Deaminase 2 Deficiency (DADA2) (OMIM: 607575) is a monogenic, autoinflammatory disease caused by the loss of functional homozygous or heterozygous mutations in the ADA 2 gene (previously CECR1, Cat Eye Syndrome Chromosome Region 1). A timely diagnosis is crucial to start Anti-TNF therapies that are efficacious in controlling the disease. The confirmation of DADA2 is based on DNA sequencing and enzymatic assay. It is, thus, very important to have robust and reliable assays that can be rapidly utilized in specialized laboratories that can centralize samples from other centers. In this paper, we show a novel enzymatic assay based on liquid chromatography-tandem mass spectrometry that allows the accurate determination of the ADA2 enzyme activity starting from very small amounts of plasma spotted on filter paper (dried plasma spot). The method allows significantly distinguishing healthy controls from affected patients and carriers and could be of help in implementing the diagnostic workflow of DADA2.  相似文献   
992.
 Ce-V mixed oxide films have been deposited by RF sputtering with the aim of increasing the Li charge capacity of counter electrodes in smart windows. Such mixed oxides have shown high transmittance and optical passivity in the visible region. After electrode pre-conditioning by cyclic voltammetry, a good electrochemical reversibility in LiClO4– propylene carbonate electrolyte was observed, and large Li-charge capacity under galvanostatic charging (up to 50 mCċcm−2) has been measured. The electrode charge capacity decreased after prolonged insertion-deinsertion cycles, whereas the photoptic transmittance remained about constant. After 800 cycles the Li-charge capacity decreased to 40 mCċcm−2. The Li diffusion coefficient inside the films measured by electrochemical impedance and by galvanostatic titration ranged from 10−11 cm2ċs−1 to 10−13cm2ċs−1. We observed that the Li charge capacity of the film electrodes is a function of the film deposition conditions, because it increased with the vanadium oxide concentration in the target and with the oxygen content in the sputtering atmosphere.  相似文献   
993.
 Let N be an H-type group of homogeneous dimension Q. We study the space of biradial Schwartz functions on N by means of the Gelfand transform. This enables us to characterize the class of biradial homogeneous distributions on N of degree α, with 0 ? α< Q, which are away from the identity, via the Gelfand transform. (Received 26 April 2000; in revised form December 2000)  相似文献   
994.
995.

We study non reflexive Orlicz spaces \(L^\varPsi \) and their Morse subspace \(M^\varPsi \), i.e. the closure of \(L^\infty \) in \(M^\varPsi \) to determine when \((M^\varPsi ,L^\varPsi )\) can be described as having an oO type structure with respect to an equivalent norm on \(L^\varPsi \). Examples of classes of Young functions for which the answer is affirmative are provided, but also examples are given to show that this is not possible for all non-reflexive Orlicz spaces. An equivalent expression of the distance in \(L^\varPsi \) to \(M^\varPsi \), induced by the new norm, is also provided.

  相似文献   
996.
We provide fast and accurate adaptive algorithms for the spatial resolution of current densities in MEG. We assume that vector components of the current densities possess a sparse expansion with respect to preassigned wavelets. Additionally, different components may also exhibit common sparsity patterns. We model MEG as an inverse problem with joint sparsity constraints, promoting the coupling of non-vanishing components. We show how to compute solutions of the MEG linear inverse problem by iterative thresholded Landweber schemes. The resulting adaptive scheme is fast, robust, and significantly outperforms the classical Tikhonov regularization in resolving sparse current densities. Numerical examples are included.  相似文献   
997.
In the present paper we consider an orthotropic micropolar elastic material subject to a state of plane strain. In this context, we establish necessary and sufficient conditions for the strong ellipticity of constitutive coefficients. Furthermore, we study existence of progressive plane waves under the strong ellipticity conditions previously determined. Finally, we detail the results obtained for a specific class of materials related to tetragonal systems.  相似文献   
998.
A technique for fringe analysis using variance of directly fringe-shifted shearograms is demonstrated experimentally. Statistical variations in each of the fringe-shifted shearograms are calculated to obtain a contrast map which is then used to characterize sub-surface defects and mechanical loading points. The technique offers reduced computational load compared to Fourier transform-based fringe processing algorithms. The technique is implemented using both phase difference maps and interferograms. The latter allows the extension of the technique to physical (i.e., non-reconstructed) interferograms for the analysis of dynamic systems.  相似文献   
999.
1000.
In this paper, we investigate the problem of the strategic foundation of the Cournot–Walras equilibrium approach. To this end, we respecify à la Cournot–Walras the mixed version of a model of simultaneous, noncooperative exchange, originally proposed by Lloyd S. Shapley. We show, through an example, that the set of the Cournot–Walras equilibrium allocations of this respecification does not coincide with the set of the Cournot–Nash equilibrium allocations of the mixed version of the original Shapley’s model. As the nonequivalence, in a one-stage setting, can be explained by the intrinsic two-stage nature of the Cournot–Walras equilibrium concept, we are led to consider a further reformulation of the Shapley’s model as a two-stage game, where the atoms move in the first stage and the atomless sector moves in the second stage. Our main result shows that the set of the Cournot–Walras equilibrium allocations coincides with a specific set of subgame perfect equilibrium allocations of this two-stage game, which we call the set of the Pseudo–Markov perfect equilibrium allocations. We would like to thank Pierpaolo Battigalli, Marcellino Gaudenzi, and an anonymous referee for their comments and suggestions.  相似文献   
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