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51.
The economics models of reputation and quality in markets can be classified in three categories. (i) Pure hidden action, where only one type of seller is present who can provide goods of different quality. (ii) Pure hidden information, where sellers of different types have no control over product quality. (iii) Mixed frameworks, which include both hidden action and hidden information. In this paper we develop a pure hidden action model of reputation and Bertrand competition, where consumers and firms interact repeatedly in a market with free entry. The price of the good produced by the firms is contractible, whilst the quality is noncontractible, hence it is promised by the firms when a contract is signed. Consumers infer future quality from all available information, i.e., both from what they know about past quality and from current prices. According to early contributions, competition should make reputation unable to induce the production of high-quality goods. We provide a simple solution to this problem by showing that high quality levels are sustained as an outcome of a stationary symmetric equilibrium.  相似文献   
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The function of lysosomes relies on the ability of the lysosomal membrane to fuse with several target membranes in the cell. It is known that in lysosomal storage disorders (LSDs), lysosomal accumulation of several types of substrates is associated with lysosomal dysfunction and impairment of endocytic membrane traffic. By analysing cells from two severe neurodegenerative LSDs, we observed that cholesterol abnormally accumulates in the endolysosomal membrane of LSD cells, thereby reducing the ability of lysosomes to efficiently fuse with endocytic and autophagic vesicles. Furthermore, we discovered that soluble N‐ethylmaleimide‐sensitive factor attachment protein (SNAP) receptors (SNAREs), which are key components of the cellular membrane fusion machinery are aberrantly sequestered in cholesterol‐enriched regions of LSD endolysosomal membranes. This abnormal spatial organization locks SNAREs in complexes and impairs their sorting and recycling. Importantly, reducing membrane cholesterol levels in LSD cells restores normal SNARE function and efficient lysosomal fusion. Our results support a model by which cholesterol abnormalities determine lysosomal dysfunction and endocytic traffic jam in LSDs by impairing the membrane fusion machinery, thus suggesting new therapeutic targets for the treatment of these disorders.  相似文献   
54.
Summary The results obtained by different mass spectrometric approaches in the field of advanced glycation of proteins are reported and discussed in detail in comparison with those obtained by other analytical methodologies (fluorescence and absorbance spectroscopies, radioimmunoassay, enzyme-linked immunosorbent assay). They have been subdivided in three main groups: analysis on degraded glycated proteins, direct analysis of glycated proteins and studies on the reaction between protected lysine and glucose. The general overview so achieved indicate mass spectrometry as a particularly valid analytical method in this field of research.  相似文献   
55.
The compounds resulting from the reaction of glucose with proteins (advanced glycation products) can be important markers of chronic diabetic complications. To test the possible diagnostic value of advanced glycation products containing the furoyl moiety, collagen samples from diabetic and healthy rats were analyzed by parent ion spectroscopy. In our study, we compared normal collagen, diabetic collagen and normal collagen incubated with different glucose concentrations and we employed different hydrolysis procedures (HCl and proteinase). Mass spectroscopic measurements performed on hydrolyzed samples showed that either different samples or different hydrolysis procedures produce a similar set of furoyl-containing compounds. 2-(2-Furoyl)-4(5)-(2-furanyl)-1H-imidazole (FFI) which has been reported to be one of the advanced glycation products, was never found in any of the samples examined. Hence neither FFI nor furoyl-containing molecules can be considered markers of advanced glycation processes.  相似文献   
56.
To evaluate the influence of good metabolic equilibrium on Diabetic Autonomic Neuropathy (DAN), cardiovascular autonomic reflexes were monitored in 9 male insulin-dependent diabetic patients with DAN, treated with Continuous Subcutaneous Insulin Infusion (CSII) by pump: 9 for 10 days, 4 for 1 year and 2 for 20 months. Autonomic neuropathy was assessed evaluating 5 cardiovascular autonomic tests: Valsalva Manoeuvre (VR), Deep Breathing (DB), Lying-to-Standing (L-S), Sustained Handgrip (SHG), and Postural Hypotension (PH). Metabolic control was assessed evaluating the mean daily plasma glucose, glucosuria and glycosylated hemoglobin. Ten days of CSII treatment induced a normalization of glucose balance and a slight but significant improvement in some parasympathetic cardiovascular tests (VR: from 1.09 +/- 0.01 to 1.13 +/- 0.02; P less than 0.05). After 4-8 months of CSII treatment a significant improvement in VR (P less than 0.05); DB (P less than 0.01) and L-S (P less than 0.05) was recorded. The long-term treatment with CSII did not seem to induce a further amelioration in cardiovascular autonomic reflexes. These results show that the slight improvement induced by good metabolic balance in the cardiovascular autonomic response could be related to functional-metabolic rather than structural changes in the nerves.  相似文献   
57.

Background

Erdheim Chester disease (ECD) is a rare, non-Langerhans cell histiocytosis characterized by widespread tissue infiltration by CD68-positive, CD1a-negative foamy histiocytes. ECD can be difficult to identify, and diagnosis relies on the presence of histiocytes with certain histologic and immunophenotypic features in an appropriate clinical and radiologic setting. Clinical signs and symptoms are variable depending on which organ systems are involved. Most patients have at least skeletal involvement with bone pain as well as fatigue. Other common manifestations include diabetes insipidus, cardiac, periaortic, or retro-orbital infiltration/fibrosis, kidney impairment, xanthelasmas, among others.

Case presentation

Herein, we describe a case of BRAF-mutation positive ECD in a patient with Burkitt lymphoma, and we review recent literature.

Conclusion

Underlying BRAF and other MAPK pathway mutations are identified in approximately 50% of cases of ECD, which aids in diagnosis as well as enables novel targeted treatments. ECD patients have an increased risk of myeloid neoplasms; however, unlike other histiocytoses, an association with lymphoproliferative disorders has not been recognized.
  相似文献   
58.
In order to investigate the secondary, tertiary, and dynamic structure of the iron-free (apo) and iron-saturated (holo) forms of human serum transferrin and its amino (N)-terminal lobe at the physiologically relevant pHs 7.4 and 5.0, we have combined ultraviolet circular dichroism (CD) spectroscopy with transient-electric birefringence (TEB) measurements. No significant changes are found in the protein's secondary structure under the different conditions studied. The tertiary structure as monitored by near-UV CD is affected by iron binding, but does not change upon decrease in pH. In contrast, TEB results indicate dramatic changes in the dynamic structure of transferrin both upon binding of iron and decrease of pH. In apotransferrin freedom of movement is found for the lobes with respect to each other, and for the domains within the lobes. The interlobal flexibility is considerably enhanced at the lower pH. Holotransferrin is found to behave as a rigid molecule. © 1995 Wiley-Liss, Inc.  相似文献   
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We compared the average body size (wing span) of Finnish geometrid moth species in relation to their degree of polyphagy and quality of food. The first hypothesis, originally constructed for mammals and birds, states that smaller species should more often be specialists than large species, because of the different relationships between body size and home range size, and body size and daily energy requirements. According to the second hypothesis, smaller species should feed more often on herbs than do larger species, because of the different defence mechanisms of herbs and woody plants. The results support both of these hypotheses. Specialist species are smaller than oligophagous or polyphagous species, and small species concentrate on herbs. We conclude that quality and quantity of food resources may explain the pattern.  相似文献   
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