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101.
Rui M Costa Nikolai B Federov Jeff H Kogan Geoffrey G Murphy Joel Stern Masuo Ohno Raju Kucherlapati Tyler Jacks Alcino J Silva 《Nature》2002,415(6871):526-530
Neurofibromatosis type I (NF1) is one of the most common single-gene disorders that causes learning deficits in humans. Mice carrying a heterozygous null mutation of the Nfl gene (Nfl(+/-) show important features of the learning deficits associated with NF1 (ref. 2). Although neurofibromin has several known properties and functions, including Ras GTPase-activating protein activity, adenylyl cyclase modulation and microtubule binding, it is unclear which of these are essential for learning in mice and humans. Here we show that the learning deficits of Nf1(+/-) mice can be rescued by genetic and pharmacological manipulations that decrease Ras function. We also show that the Nf1(+/-) mice have increased GABA (gamma-amino butyric acid)-mediated inhibition and specific deficits in long-term potentiation, both of which can be reversed by decreasing Ras function. Our results indicate that the learning deficits associated with NF1 may be caused by excessive Ras activity, which leads to impairments in long-term potentiation caused by increased GABA-mediated inhibition. Our findings have implications for the development of treatments for learning deficits associated with NF1. 相似文献
102.
The failing heart. 总被引:19,自引:0,他引:19
Cardiomyopathies are disorders affecting heart muscle that usually result in inadequate pumping of the heart. They are the most common cause of heart failure and each year kill more than 10,000 people in the United States. In recent years, there have been breakthroughs in understanding the molecular mechanisms involved in this group of conditions, with knowledge of the genetic basis for cardiomyopathies perhaps seeing the largest advance, enabling clinicians to devise improved diagnostic strategies and preparing the stage for new therapies. 相似文献
103.
采用数值方法分析心肌动作电位对心脏节律的影响。心肌动作电位用Noble模型来描述,心脏节律用Bernardo-Signorint模型来模拟。数值分析结果表明,心肌动作电位形成的靶环波和螺旋波对心脏节律影响较大。当心肌动作电位表现为噪声时,心脏节律可完全破坏。 相似文献
104.
本文研究APPLE─Ⅱ微机利用编程控制获得自动调节脉冲信号的重复频率和宽度的方法。实验证明,这种方法可以使APPLE─Ⅱ微机作为可调脉冲信号源使用。 相似文献
105.
用血管铸型和透明方法观察了家兔膀胱的血供情况。膀胱动脉主要起自脐动脉,沿膀胱侧缘行向膀胱顶,沿途发出1~4支基本对称的分支分布于膀胱前面,两侧膀胱动脉的分支在其表面有吻合现象。膀胱动脉及其分支均呈不同程度的弯曲或弹簧状,可能是适应膀胱生理性增大的结构基础。 相似文献
106.
本文叙述了用于定量分析电镀添加剂中丙烯磺酸钠和糖精钠的快速、准确、精密的NMR分析方法.该方法是依据每个组分的NMR特征信号和用作内标的四甲基澳化按NMR信号的积分比值建立的,各组分不需要预先分离. 相似文献
107.
对δ(G)≥8的连续三次图G是边可重构的定理给出了一个简单证明。此外还证明了,当δ(G)=7,γ(G)≤82以及δ(G)=6,γ(G)≤17时,连续三次图G是边可重构的 相似文献
108.
Heat-shock proteins are normal constituents of cells whose synthesis is increased on exposure to various forms of stress. They are interesting because of their ubiquity and high conservation during evolution. Two families of heat-shock proteins, hsp60s and hsp70s, have been implicated in accelerating protein folding and oligomerization and also in maintaining proteins in an unfolded state, thus facilitating membrane transport. The Escherichia coli hsp70 analogue, DnaK, and two other heat-shock proteins, DnaJ and GrpE, are required for cell viability at high temperatures and are involved in DNA replication of phage lambda and plasmids P1 and F. These three proteins are involved in replication in vitro of P1 DNA along with many host replication proteins and the P1 RepA initiator protein. RepA exists in a stable protein complex with DnaJ containing a dimer each of RepA and DnaJ. We report here that DnaK and DnaJ mediate an alteration in the P1 initiator protein, rendering it much more active for oriP1 DNA binding. 相似文献
109.
Abnormal pattern detected in fragile-X patients by pulsed-field gel electrophoresis. 总被引:26,自引:0,他引:26
The fragile-X syndrome is the most frequent inherited form of mental retardation, with an incidence of 1 in 1,500 males. It is characterized by the presence of a fragile site at Xq27.3 induced in vitro by folate deprivation or by inhibitors of deoxynucleotide synthesis. Its mode of inheritance is unusual for an X-linked trait, with incomplete penetrance in both males and females. Some phenotypically normal males transmit the mutation to all their daughters who rarely express any symptoms, but penetrance is high in sons and daughters of these carrier women. Genetic and physical mapping of the Xq27-q28 region has confirmed that the disease locus is located at or very near the fragile site. Hypotheses proposed to account for the abnormalities in the inheritance of the disease include sequence rearrangements by meiotic recombination or a mutation that affects reactivation of an inactive X chromosome during differentiation of female germ cells. To detect such rearrangements, or methylation changes that may reflect a locally inactive X chromosome, we used pulsed-field gel analysis of DNA from fragile-X patients with probes close to the fragile-X locus. The probe Do33 (DXS465) detected abnormal patterns in fragile-X patients, but not in normal controls or in non-expressing male transmitters. 相似文献
110.
利用有机气体化学裂解技术 ,用二甲苯作碳源 ,二茂铁作催化剂 ,噻吩作助长剂 ,氢气作载气 ,对碳纳米管的制备进行了研究 .研究结果表明 ,二甲苯流量、氢气流量及有机气体裂解温度等工艺参数对碳纳米管的产量及形态有很大的影响 ;在反应温度为 10 0 0~ 110 0℃ ,氢气流量为 15 0mL·min- 1,二甲苯的流量为 0 .12 1mL·min- 1时 ,能获得直径为 4 0~ 10 0nm的碳纳米管 ,碳纳米管的纯度可达 95 %以上 . 相似文献