全文获取类型
收费全文 | 1338篇 |
免费 | 6篇 |
国内免费 | 12篇 |
专业分类
系统科学 | 31篇 |
教育与普及 | 4篇 |
理论与方法论 | 29篇 |
现状及发展 | 178篇 |
研究方法 | 265篇 |
综合类 | 792篇 |
自然研究 | 57篇 |
出版年
2021年 | 3篇 |
2020年 | 6篇 |
2019年 | 3篇 |
2018年 | 10篇 |
2017年 | 9篇 |
2016年 | 22篇 |
2015年 | 13篇 |
2014年 | 17篇 |
2013年 | 24篇 |
2012年 | 103篇 |
2011年 | 207篇 |
2010年 | 38篇 |
2009年 | 6篇 |
2008年 | 111篇 |
2007年 | 110篇 |
2006年 | 119篇 |
2005年 | 115篇 |
2004年 | 108篇 |
2003年 | 99篇 |
2002年 | 121篇 |
2001年 | 8篇 |
2000年 | 9篇 |
1999年 | 3篇 |
1998年 | 4篇 |
1996年 | 3篇 |
1994年 | 2篇 |
1993年 | 9篇 |
1992年 | 2篇 |
1991年 | 2篇 |
1990年 | 5篇 |
1989年 | 7篇 |
1988年 | 10篇 |
1987年 | 4篇 |
1986年 | 3篇 |
1985年 | 3篇 |
1984年 | 4篇 |
1983年 | 3篇 |
1982年 | 2篇 |
1980年 | 2篇 |
1975年 | 5篇 |
1973年 | 6篇 |
1972年 | 1篇 |
1971年 | 2篇 |
1970年 | 1篇 |
1968年 | 2篇 |
1967年 | 1篇 |
1965年 | 1篇 |
1963年 | 2篇 |
1962年 | 1篇 |
1959年 | 1篇 |
排序方式: 共有1356条查询结果,搜索用时 31 毫秒
71.
Peter J. Perkins Frederick G. Lindzey Jay A. Roberson Gregory McDaniel Randy Berger 《西北部美国博物学家》2011,49(2)
Winter habitat use and food habits of Blue Grouse ( Dendragapus obscurus ) were studied in an isolated Utah desert mountain range that contained little typical Douglas-fir ( Pseudotsuga menziesii ) winter habitat. Habitat use was concentrated in the Douglas-fir and pinyon ( Pinus edulis )-juniper ( Juniperus spp. ) habitat. Douglas-fir and pinyon pine were the most consumed foods. Other foods that represented >15% of the composition of an individual fecal sample were limber pine ( Pinus flexilis ), mahogany ( Cercocarpus ledifoliu ), juniper, and an Anteunaria-Cirsium type. The breadth in winter diet indicates that Blue Grouse may successfully occupy other habitats when typical winter habitat is scarce. 相似文献
72.
The population structure of 2 Great Basin odonate species was assessed using protein electrophoresis. Analyses included 7 populations of Sympetrum corruptum (suborder Anisoptera), a migratory and highly mobile dragonfly, and 8 populations of Enallagma carunculatum (suborder Zygoptera), a weak flier that is not known to migrate far from natal water sources. Though we expected the damselfly ( E. carunculatum ) to show greater genetic isolation than the dragonfly ( S. corruptum ), both species apparently had high levels of gene flow (theta = 0.0604 for S. corruptum , theta = 0.0485 for E. carunculatum ) and showed no evidence for isolation by distance. These results suggest that both species are highly vagile and that the most important factors affecting population structure of these odonates may be ecological conditions such as habitat patchiness and the ephemerality of water sources. 相似文献
73.
Jean-Pierre Vilardaga Guillermo Romero Peter A. Friedman Thomas J. Gardella 《Cellular and molecular life sciences : CMLS》2011,68(1):1-13
The parathyroid hormone (PTH) receptor type 1 (PTHR), a G protein-coupled receptor (GPCR), transmits signals to two hormone
systems—PTH, endocrine and homeostatic, and PTH-related peptide (PTHrP), paracrine—to regulate different biological processes.
PTHR responds to these hormonal stimuli by activating heterotrimeric G proteins, such as GS that stimulates cAMP production. It was thought that the PTHR, as for all other GPCRs, is only active and signals through
G proteins on the cell membrane, and internalizes into a cell to be desensitized and eventually degraded or recycled. Recent
studies with cultured cell and animal models reveal a new pathway that involves sustained cAMP signaling from intracellular
domains. Not only do these studies challenge the paradigm that cAMP production triggered by activated GPCRs originates exclusively
at the cell membrane but they also advance a comprehensive model to account for the functional differences between PTH and
PTHrP acting through the same receptor. 相似文献
74.
Dobbins SE Broderick P Melin B Feychting M Johansen C Andersson U Brännström T Schramm J Olver B Lloyd A Ma YP Hosking FJ Lönn S Ahlbom A Henriksson R Schoemaker MJ Hepworth SJ Hoffmann P Mühleisen TW Nöthen MM Moebus S Eisele L Kosteljanetz M Muir K Swerdlow A Simon M Houlston RS 《Nature genetics》2011,43(9):825-827
To identify susceptibility loci for meningioma, we conducted a genome-wide association study of 859 affected individuals (cases) and 704 controls with validation in two independent sample sets totaling 774 cases and 1,764 controls. We identified a new susceptibility locus for meningioma at 10p12.31 (MLLT10, rs11012732, odds ratio = 1.46, P(combined) = 1.88 × 10(-14)). This finding advances our understanding of the genetic basis of meningioma development. 相似文献
75.
Anderson BH Kasher PR Mayer J Szynkiewicz M Jenkinson EM Bhaskar SS Urquhart JE Daly SB Dickerson JE O'Sullivan J Leibundgut EO Muter J Abdel-Salem GM Babul-Hirji R Baxter P Berger A Bonafé L Brunstom-Hernandez JE Buckard JA Chitayat D Chong WK Cordelli DM Ferreira P Fluss J Forrest EH Franzoni E Garone C Hammans SR Houge G Hughes I Jacquemont S Jeannet PY Jefferson RJ Kumar R Kutschke G Lundberg S Lourenço CM Mehta R Naidu S Nischal KK Nunes L Ounap K Philippart M Prabhakar P Risen SR 《Nature genetics》2012,44(3):338-342
Coats plus is a highly pleiotropic disorder particularly affecting the eye, brain, bone and gastrointestinal tract. Here, we show that Coats plus results from mutations in CTC1, encoding conserved telomere maintenance component 1, a member of the mammalian homolog of the yeast heterotrimeric CST telomeric capping complex. Consistent with the observation of shortened telomeres in an Arabidopsis CTC1 mutant and the phenotypic overlap of Coats plus with the telomeric maintenance disorders comprising dyskeratosis congenita, we observed shortened telomeres in three individuals with Coats plus and an increase in spontaneous γH2AX-positive cells in cell lines derived from two affected individuals. CTC1 is also a subunit of the α-accessory factor (AAF) complex, stimulating the activity of DNA polymerase-α primase, the only enzyme known to initiate DNA replication in eukaryotic cells. Thus, CTC1 may have a function in DNA metabolism that is necessary for but not specific to telomeric integrity. 相似文献
76.
van de Laar IM Oldenburg RA Pals G Roos-Hesselink JW de Graaf BM Verhagen JM Hoedemaekers YM Willemsen R Severijnen LA Venselaar H Vriend G Pattynama PM Collée M Majoor-Krakauer D Poldermans D Frohn-Mulder IM Micha D Timmermans J Hilhorst-Hofstee Y Bierma-Zeinstra SM Willems PJ Kros JM Oei EH Oostra BA Wessels MW Bertoli-Avella AM 《Nature genetics》2011,43(2):121-126
Thoracic aortic aneurysms and dissections are a main feature of connective tissue disorders, such as Marfan syndrome and Loeys-Dietz syndrome. We delineated a new syndrome presenting with aneurysms, dissections and tortuosity throughout the arterial tree in association with mild craniofacial features and skeletal and cutaneous anomalies. In contrast with other aneurysm syndromes, most of these affected individuals presented with early-onset osteoarthritis. We mapped the genetic locus to chromosome 15q22.2-24.2 and show that the disease is caused by mutations in SMAD3. This gene encodes a member of the TGF-β pathway that is essential for TGF-β signal transmission. SMAD3 mutations lead to increased aortic expression of several key players in the TGF-β pathway, including SMAD3. Molecular diagnosis will allow early and reliable identification of cases and relatives at risk for major cardiovascular complications. Our findings endorse the TGF-β pathway as the primary pharmacological target for the development of new treatments for aortic aneurysms and osteoarthritis. 相似文献
77.
78.
Peter Kosso 《Foundations of Science》2010,15(3):213-225
A central point of controversy in the time of the Copernican Revolution was the motion, or not, of the earth. We now take it for granted that Copernicus and Galileo were right; the earth really does move. But to what extent is this conclusion based on observation? This paper explores the meaning and observability of the rotation of the earth and shows that the phenomenon was not observable at the time of Galileo, and it is not observable now. 相似文献
79.
Estimating the proportion of variation in susceptibility to schizophrenia captured by common SNPs 总被引:1,自引:0,他引:1
Lee SH DeCandia TR Ripke S Yang J;Schizophrenia Psychiatric Genome-Wide Association Study Consortium 《Nature genetics》2012,44(3):247-250
Schizophrenia is a complex disorder caused by both genetic and environmental factors. Using 9,087 affected individuals, 12,171 controls and 915,354 imputed SNPs from the Schizophrenia Psychiatric Genome-Wide Association Study (GWAS) Consortium (PGC-SCZ), we estimate that 23% (s.e. = 1%) of variation in liability to schizophrenia is captured by SNPs. We show that a substantial proportion of this variation must be the result of common causal variants, that the variance explained by each chromosome is linearly related to its length (r = 0.89, P = 2.6 × 10(-8)), that the genetic basis of schizophrenia is the same in males and females, and that a disproportionate proportion of variation is attributable to a set of 2,725 genes expressed in the central nervous system (CNS; P = 7.6 × 10(-8)). These results are consistent with a polygenic genetic architecture and imply more individual SNP associations will be detected for this disease as sample size increases. 相似文献
80.
Loss-of-function mutations in TGFB2 cause a syndromic presentation of thoracic aortic aneurysm 总被引:2,自引:0,他引:2
ME Lindsay D Schepers NA Bolar JJ Doyle E Gallo J Fert-Bober MJ Kempers EK Fishman Y Chen L Myers D Bjeda G Oswald AF Elias HP Levy BM Anderlid MH Yang EM Bongers J Timmermans AC Braverman N Canham GR Mortier HG Brunner PH Byers J Van Eyk L Van Laer HC Dietz BL Loeys 《Nature genetics》2012,44(8):922-927
Loeys-Dietz syndrome (LDS) associates with a tissue signature for high transforming growth factor (TGF)-β signaling but is often caused by heterozygous mutations in genes encoding positive effectors of TGF-β signaling, including either subunit of the TGF-β receptor or SMAD3, thereby engendering controversy regarding the mechanism of disease. Here, we report heterozygous mutations or deletions in the gene encoding the TGF-β2 ligand for a phenotype within the LDS spectrum and show upregulation of TGF-β signaling in aortic tissue from affected individuals. Furthermore, haploinsufficient Tgfb2(+/-) mice have aortic root aneurysm and biochemical evidence of increased canonical and noncanonical TGF-β signaling. Mice that harbor both a mutant Marfan syndrome (MFS) allele (Fbn1(C1039G/+)) and Tgfb2 haploinsufficiency show increased TGF-β signaling and phenotypic worsening in association with normalization of TGF-β2 expression and high expression of TGF-β1. Taken together, these data support the hypothesis that compensatory autocrine and/or paracrine events contribute to the pathogenesis of TGF-β-mediated vasculopathies. 相似文献