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71.
Baala L Romano S Khaddour R Saunier S Smith UM Audollent S Ozilou C Faivre L Laurent N Foliguet B Munnich A Lyonnet S Salomon R Encha-Razavi F Gubler MC Boddaert N de Lonlay P Johnson CA Vekemans M Antignac C Attie-Bitach T 《American journal of human genetics》2007,80(1):186-194
Joubert syndrome (JS) is an autosomal recessive disorder characterized by cerebellar vermis hypoplasia associated with hypotonia, developmental delay, abnormal respiratory patterns, and abnormal eye movements. The association of retinal dystrophy and renal anomalies defines JS type B. JS is a genetically heterogeneous condition with mutations in two genes, AHI1 and CEP290, identified to date. In addition, NPHP1 deletions identical to those that cause juvenile nephronophthisis have been identified in a subset of patients with a mild form of cerebellar and brainstem anomaly. Occipital encephalocele and/or polydactyly have occasionally been reported in some patients with JS, and these phenotypic features can also be observed in Meckel-Gruber syndrome (MKS). MKS is a rare, autosomal recessive lethal condition characterized by central nervous system malformations (typically, occipital meningoencephalocele), postaxial polydactyly, multicystic kidney dysplasia, and ductal proliferation in the portal area of the liver. Since there is obvious phenotypic overlap between JS and MKS, we hypothesized that mutations in the recently identified MKS genes, MKS1 on chromosome 17q and MKS3 on 8q, may be a cause of JS. After mutation analysis of MKS1 and MKS3 in a series of patients with JS (n=22), we identified MKS3 mutations in four patients with JS, thus defining MKS3 as the sixth JS locus (JBTS6). No MKS1 mutations were identified in this series, suggesting that the allelism is restricted to MKS3. 相似文献
72.
Remi L. Gratacap Audrey C. Bergeron Robert T. Wheeler 《Journal of visualized experiments : JoVE》2014,(93)
Early defense against mucosal pathogens consists of both an epithelial barrier and innate immune cells. The immunocompetency of both, and their intercommunication, are paramount for the protection against infections. The interactions of epithelial and innate immune cells with a pathogen are best investigated in vivo, where complex behavior unfolds over time and space. However, existing models do not allow for easy spatio-temporal imaging of the battle with pathogens at the mucosal level.The model developed here creates a mucosal infection by direct injection of the fungal pathogen, Candida albicans, into the swimbladder of juvenile zebrafish. The resulting infection enables high-resolution imaging of epithelial and innate immune cell behavior throughout the development of mucosal disease. The versatility of this method allows for interrogation of the host to probe the detailed sequence of immune events leading to phagocyte recruitment and to examine the roles of particular cell types and molecular pathways in protection. In addition, the behavior of the pathogen as a function of immune attack can be imaged simultaneously by using fluorescent protein-expressing C. albicans. Increased spatial resolution of the host-pathogen interaction is also possible using the described rapid swimbladder dissection technique.The mucosal infection model described here is straightforward and highly reproducible, making it a valuable tool for the study of mucosal candidiasis. This system may also be broadly translatable to other mucosal pathogens such as mycobacterial, bacterial or viral microbes that normally infect through epithelial surfaces. 相似文献
73.
Diana J. Cole Byron J. T. Morgan Rachel S. McCrea Roger Pradel Olivier Gimenez Remi Choquet 《Ecology and evolution》2014,4(11):2124-2133
We examine memory models for multisite capture–recapture data. This is an important topic, as animals may exhibit behavior that is more complex than simple first‐order Markov movement between sites, when it is necessary to devise and fit appropriate models to data. We consider the Arnason–Schwarz model for multisite capture–recapture data, which incorporates just first‐order Markov movement, and also two alternative models that allow for memory, the Brownie model and the Pradel model. We use simulation to compare two alternative tests which may be undertaken to determine whether models for multisite capture–recapture data need to incorporate memory. Increasing the complexity of models runs the risk of introducing parameters that cannot be estimated, irrespective of how much data are collected, a feature which is known as parameter redundancy. Rouan et al. (JABES, 2009, pp 338–355) suggest a constraint that may be applied to overcome parameter redundancy when it is present in multisite memory models. For this case, we apply symbolic methods to derive a simpler constraint, which allows more parameters to be estimated, and give general results not limited to a particular configuration. We also consider the effect sparse data can have on parameter redundancy and recommend minimum sample sizes. Memory models for multisite capture–recapture data can be highly complex and difficult to fit to data. We emphasize the importance of a structured approach to modeling such data, by considering a priori which parameters can be estimated, which constraints are needed in order for estimation to take place, and how much data need to be collected. We also give guidance on the amount of data needed to use two alternative families of tests for whether models for multisite capture–recapture data need to incorporate memory. 相似文献
74.
75.
Hino S Kito A Yokoshima R Sugino R Oshima K Morita T Okajima T Nadano D Uchida K Matsuda T 《Biochemical and biophysical research communications》2012,421(2):329-334
Phagocytes engulf pathogenic microbes, kill them and degrade their cellular macromolecules by hydrolytic enzymes in phagolysosomes. However, such enzymes are unable to degrade some microbial polysaccharides, and fate of such indigestible polysaccharides in phagocytes remains uncertain. Using the extracellular domain of Dectin-1 as β-glucan-specific probes, we succeeded in detection of soluble and Dectin-1-reactive β-glucan discharged from mouse RAW 264.7 and human THP-1 macrophage cell lines as well as mouse peritoneal macrophages, which had phagocytized insoluble β-glucan particles. The RAW 264.7 cell culture-supernatant containing the discharged β-glucan stimulated naïve RAW 264.7 cells, resulting in the induction of cytokine expression. Such discharge of Dectin-1-reactive β-glucan from macrophage cells was inhibited by either NADPH oxidase inhibitors (apocynin and diphenylene iodonium) or radical scavengers (N-acetyl cysteine and MCI-186). Moreover, reactive oxygen species (ROS) produced by a Cu2+/ascorbic acid system solubilized insoluble β-glucan particles in vitro, and a part of the solubilized β-glucan was Dectin-1 reactive and biologically active in macrophage activation. The soluble and biologically active β-glucan was degraded further during prolonged exposure to ROS. These results suggest that degraded but Dectin-1-reactive β-glucan is discharged from macrophage cells phagocytizing insoluble β-glucan particles and stimulates not only themselves again but also the other naïve phagocytes, leading to the effective elimination of infecting microbes and the ultimate breakdown and inactivation of metabolically resistant β-glucan. 相似文献
76.
Takeuchi T Sennari R Sugiura K Tateno H Hirabayashi J Kasai K 《Biochemical and biophysical research communications》2008,377(1):303-306
C-type lectins are a family of proteins with an affinity to carbohydrates in the presence of Ca2+. In the genome of Caenorhabditis elegans, almost 300 genes encoding proteins containing C-type lectin-like domains (CTLDs) have been assigned. However, none of their products has ever been shown to have carbohydrate-binding activity. In the present study, we selected 6 potential C-type lectin genes and prepared corresponding recombinant proteins. One of them encoded by clec-79 was found to have sugar-binding activity by using a newly developed glycoconjugate microarray based on evanescent-field excited fluorescence. CLEC-79 exhibited affinity to sugars containing galactose at the non-reducing terminal, especially to the Galβ1-3GalNAc structure, in the presence of Ca2+. Combined with structural information of the glycans of C. elegans, these results suggest that CLEC-79 preferentially binds to O-glycans in vivo. 相似文献
77.
Naima Taqarort Abdelouahed Echairi Remi Chaussod Rachida Nouaim Hassan Boubaker Abdellah A. Benaoumar Elhassan Boudyach 《World journal of microbiology & biotechnology》2008,24(12):3031-3038
Epiphytic yeasts isolated from the surface of citrus fruits, harvested in several orchards in the Souss-Massa-Draa Valley,
Agadir, Morocco, were in vivo screened for antagonistic activity against Penicillium digitatum, the causal agent of green mold of citrus. From a total of 245 yeast strains assessed for their biocontrol activity against
P. digitatum, fifteen reduced the incidence of disease to less than 50%. The effectiveness of the best selected yeast strains showed that
Pichia anomala (YT73), Debaryomyces hansenii (YT22) and Hanseniaspora guilliermondii (YT13) were the most effective, with a reduction of green mold incidence from 65 to ~80%, compared to the control. The identification
of the fifteen selected yeast strains was carried out through an integrated approach including phenotypic and genotypic (sequencing
of D1/D2 domain of 26S rDNA encoding gene) methods. These 15 selected were identified as: H. guilliermondii, D. hansenii, H. uvarum and P. anomala. The study of the dynamics of two of the best strains, H. guilliermondii and D. hansenii, showed that these strains can grow rapidly, by approximately 2 log units, in citrus fruit wounds. Such rapid growth in wounds
indicates that these antagonist yeasts are excellent colonizers of citrus wounds and can thrive on citrus fruits as a substrate. 相似文献
78.
79.
“Metabolically Healthy” Obesity and Hyperuricemia Increase Risk for Hypertension and Diabetes: 5‐year Japanese Cohort Study
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80.
13CNMR and 1HNMR studies revealed that aluminum citrate (Al-citrate) was metabolized intracellularly and that oxalic acid was an important
product in the Al-stressed cells. This dicarboxylic acid was produced via the oxidation of glyoxylate, a precursor generated
through the cleavage of isocitrate. In the control cells, citrate was biotransformed essentially with the aid of regular tricarboxylic
cycle (TCA) enzymes. However, these control cells were able neither to uptake nor to metabolize Al-citrate. Al-stressed cells
obtained at 38–40 h of growth showed maximal Al-citrate uptake and biotransforming activities. At least a fourfold increase
in the activity of the enzyme isocitrate lyase (ICL, E. C. 4.1.3.1) has been observed in the Al-stressed cells compared with
the control cells. The transport of Al-citrate was sensitive to p-dinitrophenol and sodium azide, but not to dicyclohexylcarbodiimide. Experiments with the dye 9-aminoacridine revealed that
the translocation of Al-citrate led to an increase in intracellular pH. Thus, it appears that after the uptake of Al-citrate,
this complex is metabolized intracellularly.
Received: 13 August 2002 / Accepted: 4 September 2002 相似文献