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991.
Many small proteins seem to fold by a simple process explicable by conventional chemical kinetics and transition-state theory. This assumes an instant equilibrium between reactants and a high-energy activated state. In reality, equilibration occurs on timescales dependent on the molecules involved, below which such analyses break down. The molecular timescale, normally too short to be seen in experiments, can be of a significant length for proteins. To probe it directly, we studied very rapidly folding mutants of the five-helix bundle protein lambda(6-85), whose activated state is significantly populated during folding. A time-dependent rate coefficient below 2 micro s signals the onset of the molecular timescale, and hence the ultimate speed limit for folding. A simple model shows that the molecular timescale represents the natural pre-factor for transition state models of folding. 相似文献
992.
ZOU Benteng 《系统科学与复杂性》2001,(3)
1 IntroductionThis paper is concerned with the a priori estimates and the ekistence of solutions of theinitiaLoblique derivative boundary value problem for quasilinear non-divergence parabolic equa-tions as follows:where O is a bounded domain in R"(N > 2) with smooth boundary 0a e C' ', QT =[o,T) x a be the parabolic domain in H" ,, ST = [o,T) x aa is the lateral boundary, andarQT is the parabolic boundary of QT' The repeated indices means summatiOns from 1 to N.In [11, the first bounda… 相似文献
993.
Xiuli Chao 《系统科学与复杂性》2001,(3)
1 IntroductionQueueing networks have been one of the most importallt techniques in the modeling andanalysis of computer, communication, transportation, and Iogistics systems. The first queueingnetwork model was introduced by J..k,..[i'2], in which Jackson considers a network with Nnodes, and transitions of the typex -- x -- ej ek5 i,k = 0,1,'',N, (1)where x = (x1, x2)'' 5 XN) denotes the number of customers at the N nodes, ej is the unitvector with a 1 at the j-th position and 0 elsewh… 相似文献
994.
Family change, when adults depart or arrive around children, raises policy issues. Its measurement depends upon the evidence collected and from whom. This paper compares British children's histories obtained from fathers and mothers. The evidence, on one birth cohort of parents, comes from two sources: the National Child Development Study and the ONS Longitudinal Study. The resulting account of family change is not substantially different between parents. There is some under-reporting of children not living with their fathers. This is due to under-reporting by those included in the studies and to under-representation in them of absent fathers and lone parents. 相似文献
995.
Nitric oxide can inhibit apoptosis or switch it into necrosis 总被引:4,自引:0,他引:4
Melino G Catani MV Corazzari M Guerrieri P Bernassola F 《Cellular and molecular life sciences : CMLS》2000,57(4):612-622
Nitric oxide (NO) and its related molecules are important messengers that play central roles in pathophysiology. Redox modulation of thiol groups on protein cysteine residues by S-nitrosylation can modulate protein function. NO has emerged as a potent regulator of apoptosis in many cell types, either preventing cell death or driving an apoptotic response into a necrotic one. NO protects neuroblastoma cells from retinoid- and cisplatin-induced apoptosis, without significantly increasing necrotic cell damage. Nitrosylation of thiol groups of several critical factors may be important for cell survival. Indeed, S-nitrosylation of the active-site cysteine residue of apoptotic molecules, such as caspases and tissue transglutaminase, results in the inhibition of their catalytic activities and has important implications for the regulation of apoptosis by NO. On the other hand, NO is able to shift the anti-CD95- and ceramide-triggered apoptotic response of Jurkat T cells into necrotic cell death. In these apoptotic models, NO is therefore unable to solely inhibit cell death, indicating that it may act below the point of no return elicited by CD95-ligation and ceramide stimulation. 相似文献
996.
997.
Limb-girdle muscular dystrophy type 2G is caused by mutations in the gene encoding the sarcomeric protein telethonin 总被引:16,自引:0,他引:16
Moreira ES Wiltshire TJ Faulkner G Nilforoushan A Vainzof M Suzuki OT Valle G Reeves R Zatz M Passos-Bueno MR Jenne DE 《Nature genetics》2000,24(2):163-166
Autosomal recessive limb-girdle muscular dystrophies (AR LGMDs) are a genetically heterogeneous group of disorders that affect mainly the proximal musculature. There are eight genetically distinct forms of AR LGMD, LGMD 2A-H (refs 2-10), and the genetic lesions underlying these forms, except for LGMD 2G and 2H, have been identified. LGMD 2A and LGMD 2B are caused by mutations in the genes encoding calpain 3 (ref. 11) and dysferlin, respectively, and are usually associated with a mild phenotype. Mutations in the genes encoding gamma-(ref. 14), alpha-(ref. 5), beta-(refs 6,7) and delta (ref. 15)-sarcoglycans are responsible for LGMD 2C to 2F, respectively. Sarcoglycans, together with sarcospan, dystroglycans, syntrophins and dystrobrevin, constitute the dystrophin-glycoprotein complex (DGC). Patients with LGMD 2C-F predominantly have a severe clinical course. The LGMD 2G locus maps to a 3-cM interval in 17q11-12 in two Brazilian families with a relatively mild form of AR LGMD (ref. 9). To positionally clone the LGMD 2G gene, we constructed a physical map of the 17q11-12 region and refined its localization to an interval of 1.2 Mb. The gene encoding telethonin, a sarcomeric protein, lies within this candidate region. We have found that mutations in the telethonin gene cause LGMD 2G, identifying a new molecular mechanism for AR LGMD. 相似文献
998.
Simmler MC Cohen-Salmon M El-Amraoui A Guillaud L Benichou JC Petit C Panthier JJ 《Nature genetics》2000,24(2):139-143
Genes specifically expressed in the inner ear are candidates to underlie hereditary nonsyndromic deafness. The gene Otog has been isolated from a mouse subtractive cDNA cochlear library. It encodes otogelin, an N-glycosylated protein that is present in the acellular membranes covering the six sensory epithelial patches of the inner ear: in the cochlea (the auditory sensory organ), the tectorial membrane (TM) over the organ of Corti; and in the vestibule (the balance sensory organ), the otoconial membranes over the utricular and saccular maculae as well as the cupulae over the cristae ampullares of the three semi-circular canals. These membranes are involved in the mechanotransduction process. Their movement, which is induced by sound in the cochlea or acceleration in the vestibule, results in the deflection of the stereocilia bundle at the apex of the sensory hair cells, which in turn opens the mechanotransduction channels located at the tip of the stereo-cilia. We sought to elucidate the role of otogelin in the auditory and vestibular functions by generating mice with a targeted disruption of Otog. In Otog-/- mice, both the vestibular and the auditory functions were impaired. Histological analysis of these mutants demonstrated that in the vestibule, otogelin is required for the anchoring of the otoconial membranes and cupulae to the neuroepithelia. In the cochlea, ultrastructural analysis of the TM indicated that otogelin is involved in the organization of its fibrillar network. Otogelin is likely to have a role in the resistance of this membrane to sound stimulation. These results support OTOG as a possible candidate gene for a human nonsyndromic form of deafness. 相似文献
999.
Engert JC Bérubé P Mercier J Doré C Lepage P Ge B Bouchard JP Mathieu J Melançon SB Schalling M Lander ES Morgan K Hudson TJ Richter A 《Nature genetics》2000,24(2):120-125
Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS or SACS) is an early onset neurodegenerative disease with high prevalence (carrier frequency 1/22) in the Charlevoix-Saguenay-Lac-Saint-Jean (CSLSJ) region of Quebec. We previously mapped the gene responsible for ARSACS to chromosome 13q11 and identified two ancestral haplotypes. Here we report the cloning of this gene, SACS, which encodes the protein sacsin. The ORF of SACS is 11,487 bp and is encoded by a single gigantic exon spanning 12,794 bp. This exon is the largest to be identified in any vertebrate organism. The ORF is conserved in human and mouse. The putative protein contains three large segments with sequence similarity to each other and to the predicted protein of an Arabidopsis thaliana ORF. The presence of heat-shock domains suggests a function for sacsin in chaperone-mediated protein folding. SACS is expressed in a variety of tissues, including the central nervous system. We identified two SACSmutations in ARSACS families that lead to protein truncation, consistent with haplotype analysis. 相似文献
1000.
Rideout WM Wakayama T Wutz A Eggan K Jackson-Grusby L Dausman J Yanagimachi R Jaenisch R 《Nature genetics》2000,24(2):109-110