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41.
Anna Kutschenko Selma Staege Karen Grütz Hannes Glaß Norman Kalmbach Thomas Gschwendtberger Lisa M. Henkel Johanne Heine Anne Grünewald Andreas Hermann Philip Seibler Florian Wegner 《International journal of molecular sciences》2021,22(7)
Myoclonus-dystonia (DYT-SGCE, formerly DYT11) is characterized by alcohol-sensitive, myoclonic-like appearance of fast dystonic movements. It is caused by mutations in the SGCE gene encoding ε-sarcoglycan leading to a dysfunction of this transmembrane protein, alterations in the cerebello-thalamic pathway and impaired striatal plasticity. To elucidate underlying pathogenic mechanisms, we investigated induced pluripotent stem cell (iPSC)-derived striatal medium spiny neurons (MSNs) from two myoclonus-dystonia patients carrying a heterozygous mutation in the SGCE gene (c.298T>G and c.304C>T with protein changes W100G and R102X) in comparison to two matched healthy control lines. Calcium imaging showed significantly elevated basal intracellular Ca2+ content and lower frequency of spontaneous Ca2+ signals in SGCE MSNs. Blocking of voltage-gated Ca2+ channels by verapamil was less efficient in suppressing KCl-induced Ca2+ peaks of SGCE MSNs. Ca2+ amplitudes upon glycine and acetylcholine applications were increased in SGCE MSNs, but not after GABA or glutamate applications. Expression of voltage-gated Ca2+ channels and most ionotropic receptor subunits was not altered. SGCE MSNs showed significantly reduced GABAergic synaptic density. Whole-cell patch-clamp recordings displayed elevated amplitudes of miniature postsynaptic currents and action potentials in SGCE MSNs. Our data contribute to a better understanding of the pathophysiology and the development of novel therapeutic strategies for myoclonus-dystonia. 相似文献
42.
43.
Social medias have changed and challenge the way we interact with each other. Social medias, such as Facebook, open up new possibilities for presentation of self and of managing the self you present to others. Is this process different for those that have grown up with social medias (The Net Generation) [Tapscott (Growing up digital: the rise of the net generation. Mcgraw-Hill, NY, 1998)] from how an older group of social media users would do it? What is their primary use of Facebook and how does this differ between generations? Such questions are discussed through engaging a group of Facebook users, with clear ideas of why and how they use Facebook, in joint reflections. The participants represent two generations of internet users; Those who was introduced to internet and social medias, such as Facebook, as adults (i.e. 40 years and older) and those who have grown up with the technology (i.e. under 25 years old, also called “The Net Generation”). The discussion indicate that there are differences in how these two groups relate to social medias, such as Facebook and for what they use Facebook. Further research is necessary to pursue those differences. 相似文献
44.
Mette B. Let Charlotte Jacobsen Edwin N. Frankel Anne S. Meyer 《European Journal of Lipid Science and Technology》2003,105(9):518-528
The oxidative deterioration of milk emulsions supplemented with 1.5 wt‐% fish oil was investigated by sensory evaluation and by determining the peroxide value and volatile oxidation products after cold storage. Two types of milk emulsions were produced, one with a highly unsaturated tuna oil (38 wt‐% of n‐3 fatty acids) and one with cod liver oil (26 wt‐% of n‐3 fatty acids). The effect of added calcium disodium ethylenediaminetetraacetate (EDTA) on oxidation was also investigated. Emulsions based on cod liver oil with a slightly elevated peroxide value (1.5 meq/kg) oxidised significantly faster than the tuna oil emulsions, having a lower initial peroxide value (0.1 meq/kg). In the tuna oil emulsions the fishy off‐flavour could not be detected throughout the storage period. Addition of 5—50 ppm EDTA significantly reduced the development of volatile oxidation products in the cod liver oil emulsions, indicating that metal chelation with EDTA could inhibit the decomposition of lipid hydroperoxides in these emulsions. This study showed that an oxidatively stable milk emulsion containing highly polyunsaturated tuna fish oil could be prepared without significant fishy off‐flavour development upon storage, provided that the initial peroxide value was sufficiently low. 相似文献
45.
Lisbeth M Ottosen Ana T Lima Anne J Pedersen Alexandra B Ribeiro 《Journal of chemical technology and biotechnology (Oxford, Oxfordshire : 1986)》2006,81(4):553-559
The possibility of using fly ash from municipal solid waste incineration (MSWI) in, for example, concrete is considered. MSWI fly ash, however, has too high a concentration of heavy metals, which may cause leaching problems during use or problems with waste handling at the end of the lifetime of the concrete. The Cl content in MSWI fly ash is also too high and will cause corrosion problems in reinforced concrete. The possibility of removing some of the unwanted heavy metals (Cu and Pb) together with Cl from an MSWI fly ash suspended in water using an electrodialytic separation method was investigated. The removal of Pb and Cu was found to be highly pH dependent and the highest contents removed were 41 and 90%, respectively. The Cu concentration of the ash decreased from 2200 to 860 mg kg?1 but the Pb concentration increased from 8560 to 16 800 mg kg?1, showing that Pb is mainly found in the ash fraction that is least soluble. Hence electrodialytic treatment of the ash suspended in water is not a solution to improve the ash quality in terms of Pb. The water‐soluble Cl content per unit weight of the original ash was 12.4%. The removal of water‐soluble Cl was efficient and >98% of Cl was removed (calculated on the basis of mean initial and final concentrations). This result indicates that electrodialytic extraction may be a method that can be used for the removal of Cl from ash prior to its utilization in concrete. Copyright © 2006 Society of Chemical Industry 相似文献
46.
Dehydrogenation of Light Alkanes Over Rhenium Catalysts on Conventional and Mesoporous MFI Supports 总被引:1,自引:0,他引:1
Anne Krogh Rovik Anke Hagen Iver Schmidt Søren Dahl Ib Chorkendorff Claus Hviid Christensen 《Catalysis Letters》2006,109(3-4):153-156
Recently, Re/HZSM-5 (Si/Al = 15) was shown to be an efficient catalyst for ethane dehydrogenation and aromatization at 823 K
and atmospheric pressure. In this reaction, the major initial products were benzene, toluene and xylene (BTX), but increasing
amounts of ethene were produced with time on stream due to deactivation of the catalyst. We show that by use of rhenium impregnated
MFI supports with very few or no acidic sites (Si/Al > 500), highly selective ethane dehydrogenation catalysts are obtained
with ethene selectivities of 98%. By use of mesoporous MFI supports (Si/Al >500) the lifetime of the catalyst appears to be
slightly improved compared to conventional MFI crystals. The beneficial effect of a mesoporous MFI support is convincingly
demonstrated in propane dehydrogenation, where both conversion and selectivities on the mesoporous MFI (Si/Al > 500) impregnated
with Re are significantly higher than on Re supported on a comparable conventional MFI support. 相似文献
47.
Said Ghaout Alain Louveaux Anne Marie Mainguet Max Deschamps Youssef Rahal 《Journal of chemical ecology》1991,17(8):1499-1515
A field study of the food eaten by solitary desert locusts was carried out in a winter breeding area in Mauritania. The food eaten, determined by the plant epidermis found in fecal pellets, was compared to the plant's availability in the habitat.Schouwia purpurea, well represented in the diets, was dominant at the study site. Adults had a preference forTribulus terrester. Growth and feeding on these two plants were compared. The high water content ofS. purpurea leaves limited the dry matter eaten and slowed down growth. Glucosinolates were separated and quantified by gas chromatography. There are 132mol/g dry matter in green leaves. In multiple choice tests, with paper disks, glucosinolate extracts were phagostimulant at a low concentration (21mol/g dry matter) and repulsive at a higher one (214mol/ g dry matter). Biting behavior onS. purpurea was recorded and analyzed on video. The importance ofSchouwia purpurea in desert locust habitats and its defenses is discussed. 相似文献
48.
Antioxidant activity of grape extracts in a lecithin liposome system 总被引:15,自引:0,他引:15
Ock-Sook Yi Anne S. Meyer Edwin N. Frankel 《Journal of the American Oil Chemists' Society》1997,74(10):1301-1307
Extracts of 14 different grapes were tested for their antioxidant activities in a copper-catalyzed lecithin liposome oxidation
assay and analyzed for their phenolic components by high-performance liquid chromatography (HPLC). The total phenolic contents
of the grape extracts varied from 176 to 1236 mg gallic acid equivalents (GAE)/L. Extracts of red wine grape varieties contained
higher concentrations of phenolics than other varieties. When compared at the same 20 μM GAE basis, the grape extracts inhibited
formation of conjugated diene hydroperoxides by 25.1 to 67.9%, and hexanal formation by 49.3 to 97.8%. Extracts of red table
grape varieties Red Globe and Emperor and white wine grape varieties Chardonnay and Sauvignon Blanc gave the highest antioxidant
activities. The relative percentage inhibition of conjugated dienes and hexanal correlated with total phenols (r=0.86 and 0.89). HPLC analyses showed that anthocyanins were the most abundant phenolic compounds in extracts of red grapes,
and flavonols were most abundant in extracts of white grapes. 相似文献
49.
Tiina Reponen Anne Hyv rinen Juhani Ruuskanen Taisto Raunemaa Aino Nevalainen 《Journal of aerosol science》1994,25(8):1595-1603
Concentrations and types of airborne fungal spores and their aerodynamic sizes were studied in nine buildings with mould problems and in matched reference buildings in order to characterize the exposure in buildings with mould problems. Samples were taken with Andersen six-stage impactors in the autumn and winter. In winter, total concentrations of fungal spores in buildings with mould problems were twice those in reference buildings, even though all concentrations were quite small. In the autumn, the differences were less obvious due to the masking effect of outdoor air fungi. Also, the compositions of fungal genera differed in buildings with mould problems from that in reference buildings. The difference was most significant in the size range 2.1-4.7 μm. The size distribution of fungal spores in dwellings with mould problems skewed towards larger particles than in reference dwellings. The observed differences in size distribution indicate differences in airborne behaviour of fungal spores and, consequently, in respiratory exposure. 相似文献
50.
Kinetics of alkaline hydrolysis of mica-acrylonitrile graft copolymers was studied. The dependence of alkali concentration and the time of hydrolysis on the degree of saponification was determined. Chemical and spectroscopical techniques were used to determine the mechanism of hydrolysis. 相似文献