全文获取类型
收费全文 | 76193篇 |
免费 | 19263篇 |
国内免费 | 14篇 |
出版年
2023年 | 258篇 |
2021年 | 816篇 |
2020年 | 2530篇 |
2019年 | 4061篇 |
2018年 | 4660篇 |
2017年 | 4907篇 |
2016年 | 5067篇 |
2015年 | 4958篇 |
2014年 | 4788篇 |
2013年 | 6064篇 |
2012年 | 5033篇 |
2011年 | 5067篇 |
2010年 | 4881篇 |
2009年 | 3046篇 |
2008年 | 3670篇 |
2007年 | 3363篇 |
2006年 | 3099篇 |
2005年 | 2957篇 |
2004年 | 2818篇 |
2003年 | 2643篇 |
2002年 | 2618篇 |
2001年 | 1880篇 |
2000年 | 2009篇 |
1999年 | 1198篇 |
1998年 | 546篇 |
1997年 | 442篇 |
1996年 | 509篇 |
1995年 | 446篇 |
1994年 | 477篇 |
1993年 | 420篇 |
1992年 | 559篇 |
1991年 | 469篇 |
1990年 | 445篇 |
1989年 | 418篇 |
1988年 | 398篇 |
1987年 | 409篇 |
1986年 | 354篇 |
1985年 | 435篇 |
1984年 | 480篇 |
1983年 | 423篇 |
1982年 | 393篇 |
1981年 | 361篇 |
1980年 | 329篇 |
1979年 | 334篇 |
1978年 | 319篇 |
1977年 | 300篇 |
1976年 | 288篇 |
1975年 | 351篇 |
1974年 | 282篇 |
1973年 | 265篇 |
排序方式: 共有10000条查询结果,搜索用时 15 毫秒
991.
992.
The 3-end of the cDNA encoding the smg GDP dissociation stimulator (smg GDS) protein shares 100% homology with the previously published expressed sequence tag 00038 site. This site extends the 3-end of the smg GDS gene by 212 bp. It has been localized to human chromosome 4. Here, we have refined the localization of smg GDP to human chromosome 4q21-q25 using a mapping panel of rodent/human somatic cell hybrids containing different parts of chromosome 4. This chromosomal localization of smg GDP to 4q21-25 overlaps with a region of allele loss in primary hepatocellular carcinoma (4q13-q26).HGM symbol: RAP1GDS1 相似文献
993.
Prosaposin deficiency: further characterization of the sphingolipid activator protein-deficient sibs
V. Bradová F. Šmíd B. Ulrich-Bott W. Roggendorf B. C. Paton K. Harzer 《Human genetics》1993,92(2):143-152
Sphingolipid activator protein (SAP) deficiency, previously described in two sibs and shown to be caused by the absence of the common saposin precursor (prosaposin), was further characterized by biochemical lipid and enzyme studies and by ultrastructural analysis. The 20 week old fetal sib had increased concentrations of neutral glycolipids, including mono-, di-, tri- and tetrahexosylceramide, in liver, kidney and cultured skin fibroblasts compared with the controls. Glucosylceramide and lactosylceramide were particularly elevated. The kidney of the affected fetus showed additional increases in the concentration of sulphatide, galactosylceramide and digalactosylceramide. Free ceramide was stored in the liver and kidney, and GM3 and GM2 gangliosides were elevated in the liver, but not the brain, of the fetus. Phospholipids, however, were normal in the affected fetus. In the liver biopsy of the propositus, who later died at 16 weeks of age, only a few lipids could be studied. Glucosylceramide, dihexosylceramide and ceramide were elevated in agreement with our previous study. Enzyme studies were undertaken using detergent free liposomal substrate preparations and fibroblast extracts. The sibs' -glucocerebrosidase and -galactocerebrosidase activities were clearly reduced, but their sphingomyelinase activities were normal. The normal activity of the latter enzyme and the almost normal tissue concentration of sphingomyelin in prosaposin deficiency suggest that the prosaposin derived SAPs are not required for sphingomyelinase activity in vivo. In keeping with the biochemical findings, skin biopsies from the sibs showed massive lysosomal storage with a vesicular and membranous ultrastructure. The function of SAPs in sphingolipid degradation and the role of SAPs for enzyme activity in vitro are discussed. In addition, the similarity in neutral glycolipid accumulations in Niemann Pick disease type C and in prosaposin deficiency are noted. The phenotype of the prosaposin deficient sibs resembled acute neuronopathic (type 2) Gaucher disease more than Farber disease in several aspects, but their genotype was unique.This paper is dedicated to Prof. Jürgen Pfeiffer on the occasion of his 70th birthday 相似文献
994.
Nadja Zimmer Elisabeth Göttert Joachim Kraus Klaus D. Zang Wolfram Henn 《Human genetics》1993,91(4):317-320
Chromosomal mosaicism in chorionic villus samples (CVS) may arise from different sources, such as clonal diversity within the chorionic tissue or contamination with maternal cells. To determine the origin of karyotyped cells, we compared the immunocytochemical features of mitotic cells in CVS long-term cultures with histological sections of their tissue of origin, i.e. chorionic villi. Immunolabelling of intermediate filaments specific for epithelial cells (cytokeratin) and mesenchymal cells (vimentin) established that mitoses yielded from CVS long-term cultures indeed stem from independently growing clones derived from both the epithelial and mesenchymal parts of the chorionic villi. Thus, mosaicism in CVS cultures may reflect true genetic heterogeneity within the biopsy. However, epithelial chorionic cells undergo in vitro metaplasia leading to co-expression of cytokeratins and vimentin. Fetal-specific immune markers (-HCG and SP1-glycoprotein) are not reliably expressed in CVS cell culture. 相似文献
995.
Markus M. Nöthen Thomas Eggermann Jeanette Erdmann Bernd Eiben Dieter Hofmann Peter Propping Gesa Schwanitz 《Human genetics》1993,92(4):347-349
The parental origin of the extra chromosome in trisomy 18 was traced in 30 informative families using highly polymorphic (CA) repeats mapped on the long arm of chromosome 18. Proband DNA was recovered from slides of chromosome preparations in 28 cases and from paraffin-embedded tissues in two cases. The extra chromosome was found to be of maternal origin in 26 cases (86.7%), and paternal origin in 4 cases (13.3%). 相似文献
996.
Agneta Nordenskjöld Fredrik Hedborg Holger Luthman Magnus Nordenskjöld 《Human genetics》1993,92(3):296-298
The Beckwith-Wiedemann syndrome (BWS) is characterized by somatic overgrowth, developmental anomalies, and proneness to embryonic tumor development. The majority of cases are sporadic, but several families with an autosomal dominant mode of inheritance with variable expression and reduced penetrance have been described. In three such families, BWS has been linked to DNA markers for the insulin gene (INS) and H-ras on chromosome band 11p15. Two additional families with inherited BWS are described here. Linkage analysis has been performed with a highly informative marker for the tyrosine hydroxylase (TH) locus within the INS-IGF2 (insulin-like growth factor II)-TH gene cluser and confirms the previous observed linkage to this region (lod score 2.16 at = 0). Linkage analysis to TH provides a basis for informed genetic counselling and carrier detection in the hereditary form of the syndrome. Based on the hypothesis that IGF2 may be a candidate gene for BWS, we screened for mutations in the coding exons 7 and 9, but found no abnormalities in 5 unrelated BWS cases. 相似文献
997.
998.
Marjolijn J. A. Christianen Fee O. H. Smulders Jan Arie Vonk Leontine E. Becking Tjeerd J. Bouma Sabine M. Engel Rebecca K. James Mabel I. Nava Jaco C. de Smit Jurjan P. van der Zee Per J. Palsbøll Elisabeth S. Bakker 《Global Change Biology》2023,29(1):215-230
Large grazers (megaherbivores) have a profound impact on ecosystem functioning. However, how ecosystem multifunctionality is affected by changes in megaherbivore populations remains poorly understood. Understanding the total impact on ecosystem multifunctionality requires an integrative ecosystem approach, which is especially challenging to obtain in marine systems. We assessed the effects of experimentally simulated grazing intensity scenarios on ecosystem functions and multifunctionality in a tropical Caribbean seagrass ecosystem. As a model, we selected a key marine megaherbivore, the green turtle, whose ecological role is rapidly unfolding in numerous foraging areas where populations are recovering through conservation after centuries of decline, with an increase in recorded overgrazing episodes. To quantify the effects, we employed a novel integrated index of seagrass ecosystem multifunctionality based upon multiple, well-recognized measures of seagrass ecosystem functions that reflect ecosystem services. Experiments revealed that intermediate turtle grazing resulted in the highest rates of nutrient cycling and carbon storage, while sediment stabilization, decomposition rates, epifauna richness, and fish biomass are highest in the absence of turtle grazing. In contrast, intense grazing resulted in disproportionally large effects on ecosystem functions and a collapse of multifunctionality. These results imply that (i) the return of a megaherbivore can exert strong effects on coastal ecosystem functions and multifunctionality, (ii) conservation efforts that are skewed toward megaherbivores, but ignore their key drivers like predators or habitat, will likely result in overgrazing-induced loss of multifunctionality, and (iii) the multifunctionality index shows great potential as a quantitative tool to assess ecosystem performance. Considerable and rapid alterations in megaherbivore abundance (both through extinction and conservation) cause an imbalance in ecosystem functioning and substantially alter or even compromise ecosystem services that help to negate global change effects. An integrative ecosystem approach in environmental management is urgently required to protect and enhance ecosystem multifunctionality. 相似文献
999.
Donatella Zona Peter M. Lafleur Koen Hufkens Beniamino Gioli Barbara Bailey George Burba Eugénie S. Euskirchen Jennifer D. Watts Kyle A. Arndt Mary Farina John S. Kimball Martin Heimann Mathias Göckede Martijn Pallandt Torben R. Christensen Mikhail Mastepanov Efrén López-Blanco Albertus J. Dolman Roisin Commane Charles E. Miller Josh Hashemi Lars Kutzbach David Holl Julia Boike Christian Wille Torsten Sachs Aram Kalhori Elyn R. Humphreys Oliver Sonnentag Gesa Meyer Gabriel H. Gosselin Philip Marsh Walter C. Oechel 《Global Change Biology》2023,29(5):1267-1281
Long-term atmospheric CO2 concentration records have suggested a reduction in the positive effect of warming on high-latitude carbon uptake since the 1990s. A variety of mechanisms have been proposed to explain the reduced net carbon sink of northern ecosystems with increased air temperature, including water stress on vegetation and increased respiration over recent decades. However, the lack of consistent long-term carbon flux and in situ soil moisture data has severely limited our ability to identify the mechanisms responsible for the recent reduced carbon sink strength. In this study, we used a record of nearly 100 site-years of eddy covariance data from 11 continuous permafrost tundra sites distributed across the circumpolar Arctic to test the temperature (expressed as growing degree days, GDD) responses of gross primary production (GPP), net ecosystem exchange (NEE), and ecosystem respiration (ER) at different periods of the summer (early, peak, and late summer) including dominant tundra vegetation classes (graminoids and mosses, and shrubs). We further tested GPP, NEE, and ER relationships with soil moisture and vapor pressure deficit to identify potential moisture limitations on plant productivity and net carbon exchange. Our results show a decrease in GPP with rising GDD during the peak summer (July) for both vegetation classes, and a significant relationship between the peak summer GPP and soil moisture after statistically controlling for GDD in a partial correlation analysis. These results suggest that tundra ecosystems might not benefit from increased temperature as much as suggested by several terrestrial biosphere models, if decreased soil moisture limits the peak summer plant productivity, reducing the ability of these ecosystems to sequester carbon during the summer. 相似文献
1000.
Jörg Müller Oliver Mitesser Marc W. Cadotte Fons van der Plas Akira S. Mori Christian Ammer Anne Chao Michael Scherer-Lorenzen Petr Baldrian Claus Bässler Peter Biedermann Simone Cesarz Alice Claßen Benjamin M. Delory Heike Feldhaar Andreas Fichtner Torsten Hothorn Claudia Kuenzer Marcell K. Peters Kerstin Pierick Thomas Schmitt Bernhard Schuldt Dominik Seidel Diana Six Ingolf Steffan-Dewenter Simon Thorn Goddert von Oheimb Martin Wegmann Wolfgang W. Weisser Nico Eisenhauer 《Global Change Biology》2023,29(6):1437-1450
Intensification of land use by humans has led to a homogenization of landscapes and decreasing resilience of ecosystems globally due to a loss of biodiversity, including the majority of forests. Biodiversity–ecosystem functioning (BEF) research has provided compelling evidence for a positive effect of biodiversity on ecosystem functions and services at the local (α-diversity) scale, but we largely lack empirical evidence on how the loss of between-patch β-diversity affects biodiversity and multifunctionality at the landscape scale (γ-diversity). Here, we present a novel concept and experimental framework for elucidating BEF patterns at α-, β-, and γ-scales in real landscapes at a forest management-relevant scale. We examine this framework using 22 temperate broadleaf production forests, dominated by Fagus sylvatica. In 11 of these forests, we manipulated the structure between forest patches by increasing variation in canopy cover and deadwood. We hypothesized that an increase in landscape heterogeneity would enhance the β-diversity of different trophic levels, as well as the β-functionality of various ecosystem functions. We will develop a new statistical framework for BEF studies extending across scales and incorporating biodiversity measures from taxonomic to functional to phylogenetic diversity using Hill numbers. We will further expand the Hill number concept to multifunctionality allowing the decomposition of γ-multifunctionality into α- and β-components. Combining this analytic framework with our experimental data will allow us to test how an increase in between patch heterogeneity affects biodiversity and multifunctionality across spatial scales and trophic levels to help inform and improve forest resilience under climate change. Such an integrative concept for biodiversity and functionality, including spatial scales and multiple aspects of diversity and multifunctionality as well as physical and environmental structure in forests, will go far beyond the current widely applied approach in forestry to increase resilience of future forests through the manipulation of tree species composition. 相似文献