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排序方式: 共有163条查询结果,搜索用时 13 毫秒
1.
Autoimmune lymphoproliferative syndrome (ALPS) is a rare disease defined as a defect in the lymphocyte apoptotic pathway. Currently, the diagnosis of ALPS is based on clinical aspects, defective lymphocyte apoptosis and mutations in Fas, FasL and Casp 10 genes. Despite this, ALPS has been misdiagnosed. The aim of this work was to go one step further in the knowledge of the disease, through a molecular and proteomic analysis of peripheral blood mononuclear cells (PBMCs) from two children, a 13-year-old girl and a 6-year-old boy, called patient 1 and patient 2, respectively, with clinical data supporting the diagnosis of ALPS. Fas, FasL and Casp10 genes from both patients were sequenced, and a sample of the total proteins from patient 1 was analyzed by label-free proteomics. Pathway analysis of deregulated proteins from PBMCs was performed on the STRING and PANTHER bioinformatics databases. A mutation resulting in an in-frame premature stop codon and protein truncation was detected in the Fas gene from patient 2. From patient 1, the proteomic analysis showed differences in the level of expression of proteins involved in, among other processes, cell cycle, regulation of cell cycle arrest and immune response. Noticeably, the most down-regulated protein is an important regulator of the cell cycle process. This could be an explanation of the disease in patient 1.  相似文献   
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Ozone (O3) is an oxidizing agent that acts on phospholipids, proteins and sugars of cellular membranes producing free radicals, which cause oxidative damages. The O3 exposure has been used as a model to study oxidative stress, in which the respiratory airways represent the entrance to the organism. In this study, ultrastructural alterations were identified at the bronchiolar level during the intra-uterine lung development, using an O3 exposure model in pregnant rats during 18, 20 and 21 days of gestation. Twelve pregnant Wistar rats, six controls and six exposed to 1 ppm O3 inhalation during 12 h per day, were used. The rats were sacrificed at gestational days 18, 20 and 21; the fetuses were obtained and their lungs dissected. The ultrastructural analysis evidenced swollen mitochondria, cytoplasmic vacuolization of the epithelial cells and structural disorder caused by the oxidative stress. At gestation day 20, flake-off epithelial cells and laminar bodies in the bronchiolar lumen were observed. In the 21-gestation-day group, the mitochondria were edematous and their cristae were disrupted by the damage caused in mitochondrial membranes.  相似文献   
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Cascaded Stokes waves generation due to stimulated Brillouin scattering (SBS) of coherent optical pulses in a double-cladding Er/sup 3+/-Yb/sup 3+/ codoped fiber amplifier is reported. The highest attainable output power strongly depends on the amplifier pumping arrangement. A maximum of 40-W peak power has been obtained in counterpumping configuration. The highest energy extracted from the single-mode fiber amplifier in 1-/spl mu/s pulses is limited SBS to 15 /spl mu/J. Theses results have been theoretically confirmed using coupled-waves SBS model.  相似文献   
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High resolution 1H NMR has been applied to monitor the changes in the composition of natural mango juice subjected to spoilage and to microbial contamination with Penicillium expansum. A vast number of compounds undergoing changes upon these processes have been identified and their variations followed throughout time (132 h). Besides the formation of typical fermentation products (e.g. acetate, lactic acid, acetoin and isopropanol/2,3-butanediol) and the utilization of the major sugars (sucrose, glucose and fructose), there were changes in organic acids (e.g. decreases of quinic and shikimic acids with formation of 3,4,5-trihydroxycyclohexane acid in spoiled juice, and decreases of citric and malic acids in contaminated juice), amino acids (decreases of alanine, leucine, isoleucine and valine), and less abundant components such as oligosaccharides and aromatic compounds. The possibility of using these changes as early indicators of natural spoilage or P. expansum contamination is discussed.  相似文献   
5.
Inductive high-frequency impedance behaviour is often observed in metal electrodeposition systems. This behaviour is typically attributed to equipment limitations or non-idealities in the cell set-up and electrical connections. Such instrumental artefacts would nevertheless be relevant to a frequency range which is expected to be well above that in which inductive behaviour is in fact observed (down to a few tens of Hz). In this paper some results on an acidic Au—Sn electrodeposition system are reported. Electrochemical impedance and potentiostatic transients were measured. These results suggest that the high-frequency inductive behaviour may be related to metal nucleation processes. A correlation is proposed between the pseudo-inductive potentiostatic nucleation transients and the pseudo-inductive behaviour of the impedance spectra.  相似文献   
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Increasing use of petroleum, coupled with concern for global warming, demands the development and institution of CO2 reducing, non-fossil fuel-based alternative energy-generating strategies. Ethanol is a potential alternative, particularly when produced in a sustainable way as is envisioned for sugarcane in Brazil. We consider the expansion of sugarcane-derived ethanol to displace 5% of projected gasoline use worldwide in 2025. With existing technology, 21 million hectares of land will be required to produce the necessary ethanol. This is less than 7% of current Brazilian agricultural land and equivalent to current soybean land use. New production lands come from pasture made available through improving pasture management in the cattle industry. With the continued introduction of new cane varieties (annual yield increases of about 1.6%) and new ethanol production technologies, namely the hydrolysis of bagasse to sugars for ethanol production and sugarcane trash collection providing renewable process energy production, this could reduce these modest land requirements by 29–38%.  相似文献   
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The aim was to investigate mechanisms contributing to quercetin's previously described effects on cell-proliferation and -differentiation, which contradicted its proposed anticarcinogenic potency. In a 10-day experiment, 40 microM quercetin stabilized by 1 mM ascorbate reduced Caco-2 differentiation up to 50% (p < 0.001). Caco-2 RNA from days 5 and 10, hybridized on HG-U133A2.0 Affymetrix GeneChips(R), showed 1,743 affected genes on both days (p < 0.01). All 14 Caco-2 differentiation-associated genes showed decreased expression (p < 0.01), including intestinal alkaline phosphatase, that was confirmed technically (qRT-PCR) and functionally (enzyme-activity). The 1,743 genes contributed to 27 pathways (p < 0.05) categorized under six gene ontology (GO) processes, including apoptosis and cell-cycle. Genes within these GO-processes showed fold changes that suggest increased cell-survival and -proliferation. Furthermore, quercetin down-regulated expression of genes involved in tumor-suppression and phase II metabolism, and up-regulated oncogenes. Gene expression changes mediated by ascorbate-stabilized quercetin were concordant with those occurring in human colorectal carcinogenesis ( approximately 80-90%), but were opposite to those previously described for Caco-2 cells exposed to quercetin without ascorbate ( approximately 75-90%). In conclusion, gene expression among Caco-2 cells exposed to ascorbate-stabilized quercetin showed mechanisms contrary to what is expected for a cancer-preventive agent. Whether this unexpected in vitro effect is relevant in vivo, remains to be elucidated.  相似文献   
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