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31.
Defects in whirlin,a PDZ domain molecule involved in stereocilia elongation,cause deafness in the whirler mouse and families with DFNB31 总被引:22,自引:0,他引:22
Mburu P Mustapha M Varela A Weil D El-Amraoui A Holme RH Rump A Hardisty RE Blanchard S Coimbra RS Perfettini I Parkinson N Mallon AM Glenister P Rogers MJ Paige AJ Moir L Clay J Rosenthal A Liu XZ Blanco G Steel KP Petit C Brown SD 《Nature genetics》2003,34(4):421-428
The whirler mouse mutant (wi) does not respond to sound stimuli, and detailed ultrastructural analysis of sensory hair cells in the organ of Corti of the inner ear indicates that the whirler gene encodes a protein involved in the elongation and maintenance of stereocilia in both inner hair cells (IHCs) and outer hair cells (OHCs). BAC-mediated transgene correction of the mouse phenotype and mutation analysis identified the causative gene as encoding a novel PDZ protein called whirlin. The gene encoding whirlin also underlies the human autosomal recessive deafness locus DFNB31. In the mouse cochlea, whirlin is expressed in the sensory IHC and OHC stereocilia. Our findings suggest that this novel PDZ domain-containing molecule acts as an organizer of submembranous molecular complexes that control the coordinated actin polymerization and membrane growth of stereocilia. 相似文献
32.
Demonstration of an all-optical quantum controlled-NOT gate 总被引:1,自引:0,他引:1
The promise of tremendous computational power, coupled with the development of robust error-correcting schemes, has fuelled extensive efforts to build a quantum computer. The requirements for realizing such a device are confounding: scalable quantum bits (two-level quantum systems, or qubits) that can be well isolated from the environment, but also initialized, measured and made to undergo controllable interactions to implement a universal set of quantum logic gates. The usual set consists of single qubit rotations and a controlled-NOT (CNOT) gate, which flips the state of a target qubit conditional on the control qubit being in the state 1. Here we report an unambiguous experimental demonstration and comprehensive characterization of quantum CNOT operation in an optical system. We produce all four entangled Bell states as a function of only the input qubits' logical values, for a single operating condition of the gate. The gate is probabilistic (the qubits are destroyed upon failure), but with the addition of linear optical quantum non-demolition measurements, it is equivalent to the CNOT gate required for scalable all-optical quantum computation. 相似文献
33.
The c-fms proto-oncogene encodes a transmembrane glycoprotein that is probably identical to the receptor for the macrophage colony stimulating factor, CSF-1. Forty C-terminal amino acids of the normal receptor are replaced by 11 unrelated residues in the feline v-fms oncogene product, deleting a C-terminal tyrosine residue (Tyr969) whose phosphorylation might negatively regulate the receptor kinase activity. We show that the human c-fms gene stimulates growth of mouse NIH 3T3 cells in agar in response to human recombinant CSF-1, indicating that receptor transduction is sufficient to induce a CSF-1 responsive phenotype. Although cells transfected with c-fms genes containing either Tyr969 or Phe969 were not transformed, cotransfection of these genes with CSF-1 complementary DNA induced transformation, with c-fms(Phe969) showing significantly more activity than c-fms(Tyr969). In the absence of CSF-1, chimaeric v-fms/c-fms genes encoding the wild-type c-fms C terminus were poorly transforming, whereas chimaeras bearing Phe969 were as transforming as v-fms. Thus, the Phe969 mutation, although not in itself sufficient to induce transformation, activates the oncogenic potential of c-fms in association with an endogenous ligand or in conjunction with mutations elsewhere in the c-fms gene that confer ligand-independent signals for growth. 相似文献
34.
Video Structure Analysis 总被引:2,自引:0,他引:2
Video structure analysis is a basic requirement for most content-based video editing and processing systems. This paper presents a fast video structure analysis method based on image segmentation in each frame, with region matching between frames. The structure analysis decomposes the video into several moving objects, including information about their colors, positions, shapes, movements, and lifetimes. The method also supports user interactions to improve the results. The result shows that this method is fast and stable and can complete video analyzinq interactivelv. 相似文献
35.
多环芳烃(PAHs)是近年来在大气污染问题中逐渐受到关注的一类污染物,不仅其自身严重威胁着人体健康,还可作为低挥发性物质促进二次颗粒物的生长.世界多国开始不断通过各种技术手段对废气中PAHs的排放进行控制,PHAs已成为大气环境领域共同关注的热点问题.吸附法是最具潜力且已被工业应用认可的一类PAHs控制净化关键技术,吸附剂对PAHs的吸、脱附性能是其中的关键.目前国内外学者无论是基于传统碳类吸附剂,还是新型的介孔吸附剂,都针对此类特殊低挥发性气体的吸附相平衡、动力学以及脱附特性做了相关研究,探悉了获取PAHs吸脱附最优平衡的关键因素以及最适吸附剂.本文针对这些结果及相关应用进行了综述,对比分析了介孔吸附剂较传统吸附剂在PAHs吸脱附特性上呈现的优势,旨在为PAHs及其他低挥发性气体吸附净化的相关工作提供有效参考. 相似文献
36.
Ralph Adolphs 《自然科学进展》2007,17(13):99-105
Cognitive neuroscience has provided powerful tools that now permit the investigation of human social cognition and behavior with unprecedented accuracy. This review summarizes some of the features of the human brain that differentiate it from the brains of other animals, some of the methods used in cognitive neuroscience, and concludes with an example of research from the author’s own lab that implicates the amygdala in emotion recognition, social judgment, and autism. 相似文献
37.
Ten years ago, we reported that SM, a patient with rare bilateral amygdala damage, showed an intriguing impairment in her ability to recognize fear from facial expressions. Since then, the importance of the amygdala in processing information about facial emotions has been borne out by a number of lesion and functional imaging studies. Yet the mechanism by which amygdala damage compromises fear recognition has not been identified. Returning to patient SM, we now show that her impairment stems from an inability to make normal use of information from the eye region of faces when judging emotions, a defect we trace to a lack of spontaneous fixations on the eyes during free viewing of faces. Although SM fails to look normally at the eye region in all facial expressions, her selective impairment in recognizing fear is explained by the fact that the eyes are the most important feature for identifying this emotion. Notably, SM's recognition of fearful faces became entirely normal when she was instructed explicitly to look at the eyes. This finding provides a mechanism to explain the amygdala's role in fear recognition, and points to new approaches for the possible rehabilitation of patients with defective emotion perception. 相似文献
38.
39.
Morphological aberrations of setae 1-C (preclypeal seta), 2-C (inner clypeal seta) and 3-C (outer clypeal seta) observed in third- and fourth-instar larvae of Anopheles dthali, Anopheles maculipennis, Anopheles marteri, Culex hortensis, Culex theileri and Culex tritaeniorhynchus collected in Iran are discussed and illustrated. Variations in the shape, relative thickness, length and pigmentation of seta 1-C of Culex species that occur in southwestern Asia are reviewed. 相似文献
40.