全文获取类型
收费全文 | 1077篇 |
免费 | 4篇 |
专业分类
系统科学 | 3篇 |
理论与方法论 | 4篇 |
现状及发展 | 435篇 |
研究方法 | 70篇 |
综合类 | 564篇 |
自然研究 | 5篇 |
出版年
2018年 | 9篇 |
2017年 | 11篇 |
2016年 | 9篇 |
2014年 | 10篇 |
2013年 | 10篇 |
2012年 | 47篇 |
2011年 | 61篇 |
2010年 | 17篇 |
2009年 | 12篇 |
2008年 | 61篇 |
2007年 | 68篇 |
2006年 | 61篇 |
2005年 | 78篇 |
2004年 | 79篇 |
2003年 | 68篇 |
2002年 | 86篇 |
2001年 | 16篇 |
2000年 | 19篇 |
1999年 | 7篇 |
1988年 | 5篇 |
1986年 | 6篇 |
1985年 | 7篇 |
1984年 | 8篇 |
1980年 | 10篇 |
1979年 | 15篇 |
1978年 | 11篇 |
1977年 | 9篇 |
1976年 | 12篇 |
1975年 | 11篇 |
1974年 | 8篇 |
1973年 | 19篇 |
1972年 | 10篇 |
1971年 | 19篇 |
1970年 | 10篇 |
1969年 | 12篇 |
1968年 | 8篇 |
1967年 | 13篇 |
1966年 | 15篇 |
1965年 | 8篇 |
1962年 | 6篇 |
1961年 | 7篇 |
1960年 | 8篇 |
1959年 | 5篇 |
1958年 | 6篇 |
1957年 | 7篇 |
1956年 | 6篇 |
1955年 | 10篇 |
1954年 | 6篇 |
1946年 | 8篇 |
1945年 | 5篇 |
排序方式: 共有1081条查询结果,搜索用时 46 毫秒
81.
Rosenberg MJ Agarwala R Bouffard G Davis J Fiermonte G Hilliard MS Koch T Kalikin LM Makalowska I Morton DH Petty EM Weber JL Palmieri F Kelley RI Schäffer AA Biesecker LG 《Nature genetics》2002,32(1):175-179
The disorder Amish microcephaly (MCPHA) is characterized by severe congenital microcephaly, elevated levels of alpha-ketoglutarate in the urine and premature death. The disorder is inherited in an autosomal recessive pattern and has been observed only in Old Order Amish families whose ancestors lived in Lancaster County, Pennsylvania. Here we show, by using a genealogy database and automated pedigree software, that 23 nuclear families affected with MCPHA are connected to a single ancestral couple. Through a whole-genome scan, fine mapping and haplotype analysis, we localized the gene affected in MCPHA to a region of 3 cM, or 2 Mb, on chromosome 17q25. We constructed a map of contiguous genomic clones spanning this region. One of the genes in this region, SLC25A19, which encodes a nuclear mitochondrial deoxynucleotide carrier (DNC), contains a substitution that segregates with the disease in affected individuals and alters an amino acid that is highly conserved in similar proteins. Functional analysis shows that the mutant DNC protein lacks the normal transport activity, implying that failed deoxynucleotide transport across the inner mitochondrial membrane causes MCPHA. Our data indicate that mitochondrial deoxynucleotide transport may be essential for prenatal brain growth. 相似文献
82.
Entangled polymer solutions and melts exhibit elastic, solid-like resistance to quick deformations and a viscous, fluid-like response to slow deformations. This viscoelastic behaviour reflects the dynamics of individual polymer chains driven by brownian motion: since individual chains can only move in a snake-like fashion through the mesh of surrounding polymer molecules, their diffusive transport, described by reptation, is so slow that the relaxation of suddenly imposed stress is delayed. Entangled polymer solutions and melts therefore elastically resist deforming motions that occur faster than the stress relaxation time. Here we show that the protein myosin II permits active control over the viscoelastic behaviour of actin filament solutions. We find that when each actin filament in a polymerized actin solution interacts with at least one myosin minifilament, the stress relaxation time of the polymer solution is significantly shortened. We attribute this effect to myosin's action as a 'molecular motor', which allows it to interact with randomly oriented actin filaments and push them through the solution, thus enhancing longitudinal filament motion. By superseding reptation with sliding motion, the molecular motors thus overcome a fundamental principle of complex fluids: that only depolymerization makes an entangled, isotropic polymer solution fluid for quick deformations. 相似文献
83.
84.
85.
The scale of the human immunodeficiency virus (HIV)/AIDS epidemic has exceeded all expectations since its identification 20 years ago. Globally, an estimated 36 million people are currently living with HIV, and some 20 million people have already died, with the worst of the epidemic centred on sub-Saharan Africa. But just as the spread of HIV has been greater than predicted, so too has been its impact on social capital, population structure and economic growth. Responding to AIDS on a scale commensurate with the epidemic is a global imperative, and the tools for an effective response are known. Nothing less than a sustained social mobilization is necessary to combat one of the most serious crises facing human development. 相似文献
86.
87.
Zaugg CE Spaniol M Kaufmann P Bellahcene M Barbosa V Tolnay M Buser PT Krähenbühl S 《Cellular and molecular life sciences : CMLS》2003,60(4):767-775
Carnitine is essential for mitochondrial metabolism of long-chain fatty acids and thus for myocardial energy production. Accordingly, carnitine deficiency can be associated with cardiomyopathy. To better understand this disease, we determined myocardial function and energy metabolism in a rat model of carnitine deficiency. Carnitine deficiency was induced by a 3- or 6-week diet containing N-trimethyl-hydrazine-3-propionate, reducing cardiac and plasma carnitine by 70-85%. Myocardial function was investigated in isolated isovolumic heart preparations. Carnitine-deficient hearts showed left ventricular systolic dysfunction, reduced contractile reserve, and a blunted frequency-force relationship independently of the substrate used (glucose or palmitate). After glycogen depletion, palmitate could not sustain myocardial function. Histology and activities of carnitine palmitoyl transferase, citrate synthase, and cytochrome c oxidase were unaltered. Thus, as little as 3-6 weeks of systemic carnitine deficiency can lead to abnormalities in myocardial function. These abnormalities are masked by endogenous glycogen and are not accompanied by structural alterations of the myocardium or by altered activities of important mitochondrial enzymes. 相似文献
88.
NIH 'roadmap' charts course to tackle big research issues 总被引:1,自引:0,他引:1
89.
90.