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排序方式: 共有3048条查询结果,搜索用时 125 毫秒
31.
Alon M. Douek Mitra Amiri Khabooshan Jason Henry Sebastian-Alexander Stamatis Florian Kreuder Georg Ramm Minna-Liisa nk Donald Wlodkowic Jan Kaslin 《International journal of molecular sciences》2021,22(11)
Mucopolysaccharidosis IIIA (MPS IIIA, Sanfilippo syndrome type A), a paediatric neurological lysosomal storage disease, is caused by impaired function of the enzyme N-sulfoglucosamine sulfohydrolase (SGSH) resulting in impaired catabolism of heparan sulfate glycosaminoglycan (HS GAG) and its accumulation in tissues. MPS IIIA represents a significant proportion of childhood dementias. This condition generally leads to patient death in the teenage years, yet no effective therapy exists for MPS IIIA and a complete understanding of the mechanisms of MPS IIIA pathogenesis is lacking. Here, we employ targeted CRISPR/Cas9 mutagenesis to generate a model of MPS IIIA in the zebrafish, a model organism with strong genetic tractability and amenity for high-throughput screening. The sgshΔex5−6 zebrafish mutant exhibits a complete absence of Sgsh enzymatic activity, leading to progressive accumulation of HS degradation products with age. sgshΔex5−6 zebrafish faithfully recapitulate diverse CNS-specific features of MPS IIIA, including neuronal lysosomal overabundance, complex behavioural phenotypes, and profound, lifelong neuroinflammation. We further demonstrate that neuroinflammation in sgshΔex5−6 zebrafish is largely dependent on interleukin-1β and can be attenuated via the pharmacological inhibition of Caspase-1, which partially rescues behavioural abnormalities in sgshΔex5−6 mutant larvae in a context-dependent manner. We expect the sgshΔex5−6 zebrafish mutant to be a valuable resource in gaining a better understanding of MPS IIIA pathobiology towards the development of timely and effective therapeutic interventions. 相似文献
32.
This paper presents a decentralized observer with a consensus filter for the state observation of discrete-time linear distributed systems. Each agent in the distributed system has an observer with a model of the plant that utilizes the set of locally available measurements, which may not make the full plant state detectable. This lack of detectability is overcome by utilizing a consensus filter that blends the state estimate of each agent with its neighbors’ estimates. It is proven that the state estimates of the proposed observer exponentially converge to the actual plant states under arbitrarily changing, but connected, communication and pseudo-connected sensing graph topologies. Except these connectivity properties, full knowledge of the sensing and communication graphs is not needed at the design time. As a byproduct, we obtained a result on the location of eigenvalues, i.e., the spectrum, of the Laplacian for a family of graphs with self-loops. 相似文献
33.
Jason Hochreiter Zhongkai HanSyed Zain Masood Spencer FonteMarshall Tappen 《Image and vision computing》2014
In this paper, we propose an album-oriented face-recognition model that exploits the album structure for face recognition in online social networks. Albums, usually associated with pictures of a small group of people at a certain event or occasion, provide vital information that can be used to effectively reduce the possible list of candidate labels. We show how this intuition can be formalized into a model that expresses a prior on how albums tend to have many pictures of a small number of people. We also show how it can be extended to include other information available in a social network. Using two real-world datasets independently drawn from Facebook, we show that this model is broadly applicable and can significantly improve recognition rates. 相似文献
34.
Aqueous photoresists that are capable of higher resolutions than are currently employed by the cathode ray tube (CRT) industry were developed by combining photoactivators, which activate at shorter UV wavelengths with polyvinylpyrrolidone (PVP). Two photoactivators were synthesized: 4,4′‐diazido‐2,2′‐biphenyl disodium disulfonate (DABP), which has a maximum absorbance at 264 nm, and 4,4′‐diazido‐2,2′‐biphenylethane disodium disulfonate (DABPE), which has a maximum absorbance at 258 nm. The PVP/DABP and PVP/DABPE photoresists successfully imaged a pattern with resolution as small as 4.4 μm with photoactivator concentrations greater than 20% of the PVP concentration. Addition of silane and emulgen greatly improved the performance of the photoresists with more uniform coatings of thicknesses of up to 1.4 μm. © 2006 Wiley Periodicals, Inc. J Appl Polym Sci 102: 1637–1644, 2006 相似文献
35.
A stochastic control scheme is developed for scalar, discrete-time, and linear-dynamic systems driven by Cauchy distributed process and measurement noises. When addressing the optimal control problem for such systems, the standard quadratic cost criteria cannot be used. In this study we introduce a new objective function that is functionally similar to the Cauchy probability density function. The performance index, defined as the expectation of this objective function with respect to the Cauchy densities, exists. The dynamic programming solution to the fixed and finite horizon optimal control problem that uses this performance index appears to be intractable. Therefore, a moving horizon optimal model predictive control problem is implemented, for which the conditional expected value of the objective function and its gradients can be computed in closed form and without assumptions such as certainty equivalence. Numerical results are shown for this m-step model predictive optimal controller and compared to a similar, Linear-Exponential-Gaussian model predictive controller. An essential difference between the Cauchy and Gaussian controllers when applied to a system with Cauchy noises is that, while the Gaussian controller is linear and reacts strongly to all noise pulses, the Cauchy controller can differentiate between measurement and process noise pulses by ignoring the former while responding to the latter. This property of the Cauchy controller occurs when an impulsive measurement noise is more likely than an impulsive process noise. The Cauchy and Gaussian controllers react similarly when applied to a system with Gaussian noises, demonstrating the robustness of the proposed control scheme. 相似文献
36.
Praying mantids are models for a wide variety of behavioral, physiological, and ecological studies, and sex pheromones have been assumed to be important components of their biology. However, no mantid pheromone has ever been identified. We collected volatiles emitted by females of the mantid, Sphodromantis lineola, via solid phase microextraction (SPME). Mass spectral analysis revealed the collected volatiles to be a mixture of pentadecanal and tetradecanal. We prepared a synthetic mixture of these compounds, and found that males were both attracted to this mixture and stimulated to exhibit typical precopulatory behavior. We then examined male antennae with scanning electron microscopy, and confirmed the presence of porous antennal sensilla typical of insect pheromone receptors, i.e., that male mantids are equipped with the appropriate morphological apparatus to receive volatile chemical signals. Pheromones, in conjunction with visual and tactile cues, are thus an important feature of the reproductive biology of this, and undoubtedly other species of mantids. In addition to adding a crucial aspect of behavioral biology to our knowledge of this group, identification and synthesis of mantid pheromones may be a first step in attracting and aggregating these generalist predators for use in pest control. 相似文献
37.
Michael J. Banach Stephen J. Clarson Gregory Beaucage Jason Benkoski Tom Mates Edward J. Kramer Richard A. Vaia 《应用聚合物科学杂志》2002,86(8):2021-2024
For many microelectronic and optoelectronic applications, polymer thin films require the addition of small molecules. However, the thin‐film geometry and associated processing techniques will influence the final morphology and compositional distribution of the constituents. It is therefore important that these be examined directly rather than inferred from bulk measurements. As an example system, the concentration and distribution of Disperse Red 1 (DR1) molecules in poly(methyl methacrylate) thin films were examined. Ultraviolet visibility spectroscopy and dynamic secondary ion mass spectrometry indicate that the composition of the molecules decreased dramatically with thermal treatment of the film. The sublimation of the chromophore was observed to occur at temperatures well below the melting point of the small molecule and the glass transition of the pure polymer; this solute loss manifested itself in changes in the glass transition temperature of the film. © 2002 Wiley Periodicals, Inc. J Appl Polym Sci 86: 2021–2024, 2002 相似文献
38.
Pt-V2O5-WO3/TiO2 catalysts supported on SiC filter for NO reduction at low temperature 总被引:1,自引:0,他引:1
Joo-Hong Choi Jin-Hyun Kim Young-Cheoul Bak Rose Amal Jason Scott 《Korean Journal of Chemical Engineering》2005,22(6):844-851
The catalytic filter, V2O5-WO3-TiO2 supported on a ceramic filter, is known as a promising material for treating particulates and NO
x
simultaneously at optimum temperatures around 320°C. In order to improve its catalytic activity at low temperatures, the
effect of Pt addition on the catalytic filter has been investigated. Catalytic filters, Pt-V2O5-WO3-TiO2/SiC, were prepared by co-impregnation of Pt, V, and W precursors on TiO2 coated-SiC filter by vacuum aided-dip coating. The Pt-added catalytic filter shifted the optimum working temperature from
280–330°C (for the non Pt-impregnated filter) to 180–230°C, providing N
x
slip concentration less than 20 ppm for the treatment of 700 ppm NO at a face velocity of 2 cm/s with the same value over
the non Pt-added catalytic filters. The promotional effect following the addition of Pt is believed to result from electrical
modification of the catalyst maintaining a high electron transfer state. Ammonia oxidation was also observed to be dominant
above the optimal temperature for SCR. 相似文献
39.
Jon Hills Jason Lines Edgaras Baranauskas James Mapp Anthony Bagnall 《Data mining and knowledge discovery》2014,28(4):851-881
Time-series classification (TSC) problems present a specific challenge for classification algorithms: how to measure similarity between series. A shapelet is a time-series subsequence that allows for TSC based on local, phase-independent similarity in shape. Shapelet-based classification uses the similarity between a shapelet and a series as a discriminatory feature. One benefit of the shapelet approach is that shapelets are comprehensible, and can offer insight into the problem domain. The original shapelet-based classifier embeds the shapelet-discovery algorithm in a decision tree, and uses information gain to assess the quality of candidates, finding a new shapelet at each node of the tree through an enumerative search. Subsequent research has focused mainly on techniques to speed up the search. We examine how best to use the shapelet primitive to construct classifiers. We propose a single-scan shapelet algorithm that finds the best $k$ shapelets, which are used to produce a transformed dataset, where each of the $k$ features represent the distance between a time series and a shapelet. The primary advantages over the embedded approach are that the transformed data can be used in conjunction with any classifier, and that there is no recursive search for shapelets. We demonstrate that the transformed data, in conjunction with more complex classifiers, gives greater accuracy than the embedded shapelet tree. We also evaluate three similarity measures that produce equivalent results to information gain in less time. Finally, we show that by conducting post-transform clustering of shapelets, we can enhance the interpretability of the transformed data. We conduct our experiments on 29 datasets: 17 from the UCR repository, and 12 we provide ourselves. 相似文献
40.
Jason Crampton Michael Huth Jim Huan-Pu Kuo 《International Journal on Software Tools for Technology Transfer (STTT)》2014,16(1):31-48
Many business processes are modeled as workflows, which often need to comply with business rules, legal requirements, and authorization policies. Workflow satisfiability is the problem of determining whether there exists a workflow instance that realizes the workflow specification while simultaneously complying with such constraints. This problem has already been studied by the computer security community, with the development of algorithms and the study of their worst-case complexity. These solutions are often tailored to a particular workflow model and are, therefore, of little or no use in analyzing different models; their worst-case complexities are likely to be an unreliable judge of their feasibility; and they lack support for other forms of analysis such as the determination of the smallest number of users required to satisfy a workflow specification. We propose model checking of an NP-complete fragment $\mathsf{LTL }(\mathsf{F })$ of propositional linear-time temporal logic as an alternative solution. We report encodings in LTL(F) that can compute a set of solutions (thus deciding satisfiability), compute minimal user bases and a safe bound on the resiliency of satisfiability under the removal of users. These theoretical contributions are validated through detailed experiments whose results attest to the viability of our proposed approach. 相似文献