全文获取类型
收费全文 | 11628篇 |
免费 | 44篇 |
国内免费 | 72篇 |
专业分类
系统科学 | 61篇 |
丛书文集 | 29篇 |
教育与普及 | 21篇 |
理论与方法论 | 32篇 |
现状及发展 | 5433篇 |
研究方法 | 586篇 |
综合类 | 5454篇 |
自然研究 | 128篇 |
出版年
2012年 | 205篇 |
2011年 | 300篇 |
2010年 | 76篇 |
2009年 | 74篇 |
2008年 | 201篇 |
2007年 | 242篇 |
2006年 | 246篇 |
2005年 | 233篇 |
2004年 | 202篇 |
2003年 | 195篇 |
2002年 | 247篇 |
2001年 | 435篇 |
2000年 | 427篇 |
1999年 | 288篇 |
1992年 | 236篇 |
1991年 | 194篇 |
1990年 | 215篇 |
1989年 | 191篇 |
1988年 | 201篇 |
1987年 | 219篇 |
1986年 | 190篇 |
1985年 | 252篇 |
1984年 | 226篇 |
1983年 | 171篇 |
1982年 | 176篇 |
1981年 | 180篇 |
1980年 | 170篇 |
1979年 | 403篇 |
1978年 | 340篇 |
1977年 | 250篇 |
1976年 | 290篇 |
1975年 | 260篇 |
1974年 | 272篇 |
1973年 | 224篇 |
1972年 | 244篇 |
1971年 | 308篇 |
1970年 | 374篇 |
1969年 | 254篇 |
1968年 | 307篇 |
1967年 | 292篇 |
1966年 | 249篇 |
1965年 | 178篇 |
1964年 | 99篇 |
1959年 | 88篇 |
1958年 | 162篇 |
1957年 | 100篇 |
1956年 | 91篇 |
1955年 | 85篇 |
1954年 | 77篇 |
1948年 | 64篇 |
排序方式: 共有10000条查询结果,搜索用时 15 毫秒
901.
902.
903.
904.
905.
906.
Gene polymorphism in Netherton and common atopic disease. 总被引:13,自引:0,他引:13
A J Walley S Chavanas M F Moffatt R M Esnouf B Ubhi R Lawrence K Wong G R Abecasis E Y Jones J I Harper A Hovnanian W O Cookson 《Nature genetics》2001,29(2):175-178
Atopic dermatitis (AD) and asthma are characterized by IgE-mediated atopic (allergic) responses to common proteins (allergens), many of which are proteinases. Loci influencing atopy have been localized to a number of chromosomal regions, including the chromosome 5q31 cytokine cluster. Netherton disease is a rare recessive skin disorder in which atopy is a universal accompaniment. The gene underlying Netherton disease (SPINK5) encodes a 15-domain serine proteinase inhibitor (LEKTI) which is expressed in epithelial and mucosal surfaces and in the thymus. We have identified six coding polymorphisms in SPINK5 (Table 1) and found that a Glu420-->Lys variant shows significant association with atopy and AD in two independent panels of families. Our results implicate a previously unrecognized pathway for the development of common allergic illnesses. 相似文献
907.
K. J. Clemetson 《Cellular and molecular life sciences : CMLS》1998,54(6):499-501
Integrins are a family of adhesive receptors consisting of α- and β-subunits which attach cells together via adhesive protein ligands or bind cells to extracellular matrix. They are found on virtually all cell types and link the external ligand to the cytoskeleton of the cell. Integrins also act as signal transducers both from the outside of the cell to the interior and also inside-out. Their main functions are in recognition and in tight but regulated binding. The series of reviews presented here cover both basic aspects of integrin function, including signal transduction, snake disintegrins and structure and function of I-domains in some integrin α-subunits, as well as the role of integrins in diseases, cancer, inflammation and cardiovascular diseases. The search for suitable inhibitors of integrins for treatment of these diseases and future prospects for their use are also discussed. 相似文献
908.
K. C. Wilhelmsen 《Cellular and molecular life sciences : CMLS》1998,54(9):920-924
Chromosome 17-linked dementias have been defined by linkage analysis. The most common of these syndromes has been estimated to be the cause of between 2 and 20% of all dementia and has alternately been called frontotemporal dementia, Pick's disease (without Pick bodies) and dementia lacking distinctive features [1 – 3]. The identification of the mutation responsible for these conditions in a group of clinically and pathologically heterogeneous disorders may allow us to gain broad insight into the processes of neurodegeneration. 相似文献
909.
Integrins and cardiovascular disease 总被引:2,自引:0,他引:2
Cardiovascular diseases involve abnormal cell-cell interactions leading to the development of atherosclerotic plaque, which when ruptured causes massive platelet activation and thrombus formation. Parts of a loose thrombus may detach to form an embolus, blocking circulation at a more distant point. The integrins are a family of adhesive cell receptors interacting with adhesive proteins or with counterreceptors on other cells. There is now solid evidence that the major integrin on platelets, the fibrinogen receptor α IIb β 3 , has an important role in several aspects of cardiovascular diseases and that its regulated inhibition leads to a reduction in incidence and mortality due to these disorders. The development of α IIb β 3 inhibitors is an important strategy of many pharmaceutical companies which foresee a large market for the treatment of acute conditions in surgery, the symptoms of chronic conditions and, it is hoped, maybe even the successful prophylaxis of these conditions. Although all the associated problems have not been solved, the undoubted improvements in patient care resulting from the first of these treatments in the clinic have stimulated further research on the role of integrins on other vascular cells in these processes and in the search for new inhibitors. Both the development of specific inhibitors and of mice with specific integrin subunit genes ablated have contributed to a better understanding of the function of integrins in development of the cardiovascular system. 相似文献
910.
Somatic histone H3 alterations in pediatric diffuse intrinsic pontine gliomas and non-brainstem glioblastomas 总被引:1,自引:0,他引:1
Wu G Broniscer A McEachron TA Lu C Paugh BS Becksfort J Qu C Ding L Huether R Parker M Zhang J Gajjar A Dyer MA Mullighan CG Gilbertson RJ Mardis ER Wilson RK Downing JR Ellison DW Zhang J Baker SJ;St. Jude Children's Research Hospital–Washington University Pediatric Cancer Genome Project 《Nature genetics》2012,44(3):251-253
To identify somatic mutations in pediatric diffuse intrinsic pontine glioma (DIPG), we performed whole-genome sequencing of DNA from seven DIPGs and matched germline tissue and targeted sequencing of an additional 43 DIPGs and 36 non-brainstem pediatric glioblastomas (non-BS-PGs). We found that 78% of DIPGs and 22% of non-BS-PGs contained a mutation in H3F3A, encoding histone H3.3, or in the related HIST1H3B, encoding histone H3.1, that caused a p.Lys27Met amino acid substitution in each protein. An additional 14% of non-BS-PGs had somatic mutations in H3F3A causing a p.Gly34Arg alteration. 相似文献