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51.
    
mitochondrial neuro-gastrointestinal encephalomyopathy (MNGIE) is a rare genetic disorder characterized by thymidine phosphorylase (TP) enzyme defect. The absence of TP activity induces the imbalance of mitochondrial nucleotide pool, leading to impaired mitochondrial DNA (mtDNA) replication and depletion. Since mtDNA is required to ensure oxidative phosphorylation, metabolically active tissues may not achieve sufficient energy production. The only effective life-saving approach in MNGIE has been the permanent replacement of TP via allogeneic hematopoietic stem cell or liver transplantation. However, the follow-up of transplanted patients showed that gut tissue changes do not revert and fatal complications, such as massive gastrointestinal bleeding, can occur. The purpose of this study was to clarify whether the reintroduction of TP after transplant can recover mtDNA copy number in a normal range. Using laser capture microdissection and droplet-digital-PCR, we assessed the mtDNA copy number in each layer of full-thickness ileal samples of a naive MNGIE cohort vs. controls and in a patient pre- and post-TP replacement. The treatment led to a significant recovery of gut tissue mtDNA amount, thus showing its efficacy. Our results indicate that a timely TP replacement is needed to maximize therapeutic success before irreversible degenerative tissue changes occur in MNGIE.  相似文献   
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International Journal on Software Tools for Technology Transfer -  相似文献   
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The C-terminal aminoacidic sequence from NPM1-mutated protein, absent in normal human tissues, may serve as a leukemia-specific antigen and can be considered an ideal target for NPM1-mutated acute myeloid leukemia (AML) immunotherapy. Different in silico instruments and in vitro/ex vivo immunological platforms have identified the most immunogenic epitopes from NPM1-mutated protein. Spontaneous development of endogenous NPM1-mutated-specific cytotoxic T cells has been observed in patients, potentially contributing to remission maintenance and prolonged survival. Genetically engineered T cells, namely CAR-T or TCR-transduced T cells, directed against NPM1-mutated peptides bound to HLA could prospectively represent a promising therapeutic approach. Although either adoptive or vaccine-based immunotherapies are unlikely to be highly effective in patients with full-blown leukemia, these strategies, potentially in combination with immune-checkpoint inhibitors, could be promising in maintaining remission or preemptively eradicating persistent measurable residual disease, mainly in patients ineligible for allogeneic hematopoietic stem cell transplant (HSCT). Alternatively, neoantigen-specific donor lymphocyte infusion derived from healthy donors and targeting NPM1-mutated protein to selectively elicit graft-versus-leukemia effect may represent an attractive option in subjects experiencing post-HSCT relapse. Future studies are warranted to further investigate dynamics of NPM1-mutated-specific immunity and explore whether novel individualized immunotherapies may have potential clinical utility in NPM1-mutated AML patients.  相似文献   
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A nickel catalyst (5.75 wt.%) supported on gamma-alumina was evaluated through autothermal reforming of methane (ATR). The reforming process was pointed to hydrogen production, following thermodynamic and stoichiometric predictions. The catalyst was characterised by several methods including atomic absorption spectroscopy (AAS), B.E.T.-N2, X-ray diffraction (XRD), scanning electron microscope (SEM) and thermal analyses (thermogravimetry, TG; derivate thermogravimetry, DTG; and differential thermal analysis, DTA). Experimental evaluations in a fixed-bed reactor (1023–1123 K, 1.00 bar, 150–400 cm3/min feed) presented methane conversions in the range of 40–65%. The effluent mixtures provided hydrogen yields in the range of 78–84%, carbon monoxide 3–14%, and carbon dioxide 5–18%. High molar H2/CO ratios, ranging from 8 to 90, were obtained. Operating autothermal conditions (excess of steam, 1023–1123 K, 1.00 bar) provided low coke formation and high hydrogen selectivity (81%) for methane reforming.  相似文献   
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Numerous studies have been focused to natural ingredients to maintain the quality and shell life of foods. Edibles coating, biodegradable ingredients, such as hydrocolloids and proteins can be used for coating fish with the purpose to suppress changes of quality during frozen storage. At the present time several developing of edibles packing, like glaze using hydrocolloids, (gelatin, carrageen, starch), that besides acts as protective film for food, they are economic, can be ingested by the consumer without toxicity risk, help to preserve organoleptics and nutritional characteristics of the product and, being biodegradable they contribute to the conservation of the atmosphere. The objective of the present study was to evaluate the incorporation ofhydrocolloids in the glazing tuna process as alternative of packing, to enhance its physical and chemical characteristics. Samples of tuna was glazed incorporating to its solutions of carrageen at 0.05, 0,1 and 0.15%, and gelatin at 0.5, 1%. The samples were shaped as square (15 cm3), frozen al -30 degrees C and glazed incorporating hydrocolloids submerging the sample its solutions at 0 degrees C by 30 seconds and storage at -10 degrees C by 0, 15 and 30 days. Proximal analysis results, soluble proteins, TBA, and color showed that all the samples glazed with hydrocolloids decrease deterioration of the tuna products respect to samples glazed without hydrocolloids; concluding that glazing adding hydrocolloids protects tuna for loss humidity and delays degradation of proteins. Moreover, carrageen shows to a better performance at the concentrations of 0.05% compared with the gelatin and the starch.  相似文献   
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A novel SBA-16-related synthesis of mesostructured silica containing framework aluminum is reported here. This material was successfully synthesized using aluminum sulphate and the pH-adjusting method. The structural and porosity properties of this novel material were studied by X-ray diffraction and N2 adsorption at 77 K. The Al insertion was determined by 27Al-MAS-NMR and the acidity was monitored by infrared spectroscopy of CO adsorption at 100 K.  相似文献   
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Engineered Water Injection (EWI) has been increasingly tested and applied to enhance fluid displacement in reservoirs. The modification of ionic concentration provides interactions with the pore wall,which facilitates the oil mobility. This mechanism in carbonates alters the natural rock wettability being quite an attractive recovery method. Currently,numerical simulation with this injection method remains limited to simplified models based on experimental data. Therefore,this study uses Artificial Neural Networks (ANN) learnability to incorporate the analytical correlation between the ionic combination and the relative permeability (Kr),which depicts the wettability alteration. The ionic composition in the injection system of a Brazilian Pre-Salt benchmark is optimized to maximize the Net Present Value (NPV) of the field. The optimization results indicate the EWI to be the most profitable method for the cases tested. EWI also increased oil recovery by about 8.7% with the same injected amount and reduced the accumulated water production around 52%,compared to the common water injection.  相似文献   
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Silver, gold, copper and platinum nanoparticles (NPs) were grown on surfaces in the form of patterns by the exposure of laser radiation onto droplets of metal ion solutions and the aid of a reducing agent. The generation of patterns from metallic NPs was achieved by combining induced growth of NPs and nanostructures by laser incidence directly on surfaces and optical image formation techniques for transferring the patterns. Near-UV (363.8 nm) and visible (532 nm) laser wavelengths were used for the laser-induced growth of NPs into microstructures on glass, quartz, stainless steel, silicon, and gold-on-silicon substrates. The sizes of the patterns formed were on the micrometer scale and the sizes of the transferred patterns were on the millimeter scale. The patterns formed were generated by optical transference of image and interference of laser beams. Ag and Au substrates were highly active in surface enhanced Raman spectroscopy (SERS). The enhanced Raman activity was measured for SERS probe molecules: 9H-purin-6-amine (adenine) and 1,2-bis (4-pyridyl)-ethane analytes on Ag and Au substrates, respectively. The enhancement factors obtained were 1.8×10(5) and 6.2×10(6), respectively.  相似文献   
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