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991.
Facioscapulohumeral muscular dystrophy (FSHD) is caused by a complex epigenetic mechanism finally leading to the misexpression of DUX4 in skeletal muscle. Detecting DUX4 and quantifying disease progression in FSHD is extremely challenging, thus increasing the need for surrogate biomarkers. We applied a shotgun proteomic approach with two different setups to analyze the protein repertoire of interstitial fluids obtained from 20 muscles in different disease stages classified by magnetic resonance imaging (MRI) and serum samples from 10 FSHD patients. A total of 1156 proteins were identified in the microdialysates by data independent acquisition, 130 of which only found in muscles in active disease stage. Proteomic profiles were able to distinguish FSHD patients from controls. Two innate immunity mediators (S100-A8 and A9) and Dermcidin were upregulated in muscles with active disease and selectively present in the sera of FSHD patients. Structural muscle and plasminogen pathway proteins were downregulated. Together with the upstream inhibition of myogenic factors, this suggests defective muscle regeneration and increased fibrosis in early/active FSHD. Our MRI targeted exploratory approach confirmed that inflammatory response has a prominent role, together with impaired muscle regeneration, before clear muscle wasting occurs. We also identified three proteins as tissue and possibly circulating biomarkers in FSHD.  相似文献   
992.
Liquid-phase-pressureless-sintered SiC-AlN-Y2O3 composites were coated by means of modified pack cementation process (OXPAC, OXidation-assisted-PAck Cementation) using rare-earth oxides, RE2O3 (RE = Sc, Er, Sm, Lu, Ho), as reactive powders. The coatings, composed by the oxidation products of SiC, AlN and rare-earth silicates, were adherent to the substrate, without porosity and with a thickness of 10 μm. The oxidation resistance of the coated SiC-AlN-Y2O3 ceramics was also investigated at 1500 °C for a period of 200 h. The coated samples showed specific weight gain lower than the uncoated and pre-oxidised samples. Furthermore, the specific weight gain linearly increased with the rare-earth cationic radius.  相似文献   
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995.
Siloxane/oxide hybrids have attracted growing attention thanks to their ability to modulate the surface energy, wettability, or self-lubricity of a material. Here, we compare two functionalisation procedures (chemical vapour deposition and wet impregnation) on substrate films composed by preformed oxide particles. Three kinds of particles, characterised by different natures (SiO2 and TiO2) and average particle size, were studied to highlight possible effects related to the chemical and morphological state of the substrate surface. Morphological characterisations were carried out using dynamic light scattering and scanning electron microscopy, while the structure of the hydrophobing layer was studied by combining Fourier transform infrared spectroscopy and solid-state nuclear magnetic resonance. The degree of functionalisation and the features of the siloxane layer vary significantly among the adopted oxides and functionalisation methods. The wettability features of the different hybrids closely mirror the results of the spectroscopic characterisations, giving rise to either Lotus leaf or patch-wise hydrophobic systems depending on the adopted conditions.  相似文献   
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997.
This paper describes the Remi‐Irpet macroeconometric multisectoral model. The Remi‐Irpet, based on an input‐output core structure, has its most distinctive feature in the modelling of vertical pecuniary externalities à la Fujita‐Krugman‐Venables which represents the core dynamization mechanism. Our contribution first presents the theoretical background of the model. Second, we present a simulation exercise performed through the model in which we evaluate the structural impact of incoming FDIs for Tuscany. The impact analysis is defined in terms of (i) change in the overall productive capacity and (ii) medium‐term changes in prices and productivity, driven by the changes in the accessibility to labour and intermediate inputs.  相似文献   
998.
This study concerns the behaviour of steel frames protected by different anti‐seismic devices (dampers). Typical hysteretic and viscous dampers are arranged in three steel moment‐resisting frames (MRFs) having different dynamical features but are designed to accomplish determined performance objectives. The proposed devices are selected following an iterative procedure based on the use of a suitable damage functional, which has been applied to control the behaviour of the protected structures under a specific seismic record. The outcomes obtained by implementing incremental dynamic analyses, carried out on the basis of seven historical records characterized by different features, allow to analyse the improvement of the structural performance due to the considered dampers and, therefore, to provide design information about their employment. The comparison of results is carried out taking into account the dampers capacity to protect the structures from damage, the inter‐storey drifts, the residual deformations and the possible amplification effects. In conclusion, the equivalent behaviour factors for each damper type are given, with the aim of providing useful design parameters for the implementation of simplified conventional linear analyses. Copyright © 2014 John Wiley & Sons, Ltd.  相似文献   
999.
One of the most interesting and promising challenges for Intelligent Transportation Systems (ITSs) relates to the traffic congestion problem. Congestion is a relevant issue for transportation because it reduces the efficiency of infrastructure and increases travel time, air pollution, and fuel consumption. Nowadays, the most promising technology in support of ITSs is found in the domain of Vehicular Ad Hoc Networks (VANETs). In this paper, we propose three protocols that are able to transmit traffic information for routes of interest on VANETs without any Road Side Unit (RSU) support. The proposed protocols adopt strategies to improve the performance of packet routing based on the density and location of vehicles; moreover, they enable an interesting comparison of the performance achievable with either reactive or proactive approaches. The extensive performance results reported show how it is possible to limit the congestion monitoring overhead along Routes of Interest (ROIs), while maintaining a sufficiently high performance in terms of traffic reporting. This may be done by employing context‐aware data delivery techniques that autonomously adapt to runtime conditions. Copyright © 2016 John Wiley & Sons, Ltd.  相似文献   
1000.
Viscoelastic properties of nanocomposite fibers of high density polyethylene (HDPE) and organically modified hydrotalcite were studied. Neat and nanofilled HDPE fibers (with nanofiller content between 0.5 and 3 wt%) were produced by melt spinning and hot‐drawing at different draw ratios up to 20. Effect of temperature on storage modulus, loss modulus, and creep compliance were compared. Rising nanofiller content and/or drawing ratio accounted for an increase in storage modulus in the glassy (i.e., below the γ transition at −100°C) as well as in the rubbery state of non‐crystalline regions. The α relaxation temperature read‐off for the maximum of the loss modulus peak ranged from 20 to 60°C being dependent on frequency, filler content and draw ratio. Sumita model was successfully applied to evaluate the effective volume fraction of the dispersed phase; maximum fraction of immobilized matrix was observed for the composite with 1 wt% of nanofiller. Creep behavior was evaluated by fitting experimental data with the Burgers model. The addition of a small amount of well‐dispersed hydrotalcite (0.5–1 wt%) had a beneficial effect on the creep resistance of drawn fibers at room temperature as well as at 70°C. TEM analysis evidenced a good dispersion of 0.5% nanofiller in as‐spun fibers and improved interfacial adhesion after drawing. The best mechanical properties were observed for the composition with 1 wt% of hydrotalcite, due to combined effects of nanofiller reinforcement and stiffening produced by hot drawing. POLYM. COMPOS., 288–298, 2016. © 2014 Society of Plastics Engineers  相似文献   
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