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61.
Molecular analysis of Gaucher disease: distribution of eight mutations and the complete gene deletion in 27 patients from Germany 总被引:2,自引:0,他引:2
P. le Coutre A. Demina Ernest Beutler Michael Beck P. E. Petrides 《Human genetics》1997,99(6):816-821
Gaucher disease is the most common lysosomal storage disease with a high prevalence in the Ashkenazi Jewish population but
it is also present in other populations. The presence of eight mutations (1226G, 1448C, IVS2+1, 84GG, 1504T, 1604T, 1342C
and 1297T) and the complete deletion of the β-glucocerebrosidase gene was investigated in 25 unrelated non-Jewish patients
with Gaucher’s disease in Germany. In the Jewish population, three of these mutations account for more than 90% of all mutated
alleles. In addition, relatives of two patients were included in our study. Restriction fragment length polymorphism analysis
and sequencing of PCR products obtained from DNA of peripheral blood leukocytes was performed for mutation analysis. Gene
deletion was detected by comparison of radioactively labelled PCR fragments of both the functional β-glucocerebrosidase gene
and the pseudogene. Among the unrelated patients, 50 alleles were investigated and the mutations identified in 35 alleles
(70%), whereas 15 alleles (30%) remained unidentified. The most prevalent mutation in our group of patients was the 1226G
(370Asn→Ser) mutation, accounting for 18 alleles (36%), followed by the 1448C (444Leu→Pro) mutation, that was found in 12 alleles (24%). A complete gene deletion was present in two alleles (4%). The IVS1+2 (splicing
mutation), the 1504T (463Arg→Cys) as well as the 1342C (409Asp→His) mutations were each present in one allele (2%). None of the alleles carried the 84GG (frameshift), 1604A (496Arg→His) or the 1297T (394Val→Leu) mutation. This distribution is different from the Ashkenazi Jewish population but is similar to other Caucasian groups like
the Spanish and Portuguese populations. Our results confirm the variability of mutation patterns in Gaucher patients of different
ethnic origin. All patients were divided into nine groups according to their genotype and their clinical status was related
to the individual genotype. Genotype/phenotype characteristics of the 1226G, 1448C, and 1342C mutations of previous studies
were confirmed by our results.
Received: 19 November 1996 / Revised: 29 January 1997 相似文献
62.
Abarca Héctor Morán-Ordoñez Alejandra Villero Dani Guinart Daniel Brotons Lluís Hermoso Virgilio 《Biodiversity and Conservation》2022,31(4):1197-1215
Biodiversity and Conservation - Biodiversity keeps declining in the European Union despite the large conservation effort done over the last decades. The Biodiversity Strategy for 2030 aims to... 相似文献
63.
Antibody responses of mice exposed to low-power microwaves under combined, pulse-and-amplitude modulation 总被引:3,自引:0,他引:3
B Veyret C Bouthet P Deschaux R de Seze M Geffard J Joussot-Dubien M le Diraison J M Moreau A Caristan 《Bioelectromagnetics》1991,12(1):47-56
Irradiation by pulsed microwaves (9.4 GHz, 1 microsecond pulses at 1,000/s), both with and without concurrent amplitude modulation (AM) by a sinusoid at discrete frequencies between 14 and 41 MHz, was assessed for effects on the immune system of Balb/C mice. The mice were immunized either by sheep red blood cells (SRBC) or by glutaric-anhydride conjugated bovine serum albumin (GA-BSA), then exposed to the microwaves at a low rms power density (30 microW/cm2; whole-body-averaged SAR approximately 0.015 W/kg). Sham exposure or microwave irradiation took place during each of five contiguous days, 10 h/day. The antibody response was evaluated by the plaque-forming cell assay (SRBC experiment) or by the titration of IgM and IgG antibodies (GA-BSA experiment). In the absence of AM, the pulsed field did not greatly alter immune responsiveness. In contrast, exposure to the field under the combined-modulation condition resulted in significant, AM-frequency-dependent augmentation or weakening of immune responses. 相似文献
64.
65.
Analysis of genetic diversity in common loon Gavia immer using RAPD and mitochondrial RFLP techniques 总被引:2,自引:0,他引:2
A. K. Dhar M. A. Pokras D. K. Garcia D. C. Evers Z. J. Gordon & A. Alcivar-Warren 《Molecular ecology》1997,6(6):581-586
We used random amplified polymorphic DNA (RAPD) and restriction fragment length polymorphisms (RFLP) of mitochondrial cytochrome b (cyt b ) gene to evaluate the genetic diversity in common loon Gavia immer populations from two regions in the United States: New England (NE) and Michigan (MI). RAPD analysis with 18 primers showed 74% polymorphism in NE and 50% in MI loons (similarity coefficient F = 0.92). Although no population-specific markers were found, the frequencies of some RAPD bands varied between the two populations suggesting geographical differences. RFLP analyses with Bam HI enzyme and a 307-bp mitochondrial cyt b gene showed four haplotypes in the NE loon samples and two in the MI samples. The mtDNA haplotype diversity was 0.74 for NE and 0.51 for MI loons, supporting the RAPD data that NE loons have greater genetic diversity than MI loons. 相似文献
66.
Sternfeld L Krause E Schmid A Anderie I Latas A Al-Shaldi H Köhl A Evers K Hofer HW Schulz I 《Cellular signalling》2005,17(8):951-960
This study investigates the role of tyrosine phosphorylation and dephosphorylation in the regulation of the Ca(2+) permeant TRPV6 channel. HEK293 cells co-transfected with TRPV6 and the tyrosine phosphatase PTP1B show a constitutive Ca(2+) entry which was independent of tyrosine phosphorylation under resting conditions. Following depletion of intracellular Ca(2+) stores, TRPV6-mediated Ca(2+) entry could be increased in the presence of a tyrosine phosphatase inhibitor (bis-(N,N-dimethyl-hydroxamido) hydroxo-vanadate; DMHV). Inhibition of Src-kinases completely abolished DMHV-induced increase in TRPV6-mediated Ca(2+) influx. Co-transfection with Src led to tyrosine phosphorylation of TRPV6 which could be dephosphorylated by PTP1B. In vivo interaction of TRPV6 with PTP1B was visualized using the bimolecular fluorescence complementation (BiFC) method and proved by co-immunoprecipitation of both proteins. These data indicate that tyrosine phosphorylation is involved in the regulation of the TRPV6 channel protein. 相似文献
67.
Yanase N Hata K Shimo K Hayashida M Evers BM Mizuguchi J 《Experimental cell research》2005,310(1):10-21
Interferon alpha (IFN-alpha) inhibits growth, at least in part, through induction of apoptosis. However, the molecular mechanisms underlying IFN-alpha-induced apoptosis are not completely understood. In the present study, we found that IFN-alpha induced a sustained activation of c-Jun N-terminal kinase 1 (JNK1), but not extracellular kinases (ERKs), in Daudi B lymphoma cells, as assessed by Western blotting using phospho-specific antibodies. Several lines of evidence support the notion that the IFN-alpha-induced activation of JNK is responsible for IFN-alpha-induced apoptosis, at least in part, through upregulation of TNF-related apoptosis-inducing ligand (TRAIL). First, pretreatment of Daudi cells with a JNK inhibitor reduced IFN-alpha-induced upregulation of TRAIL and loss of mitochondrial membrane potential (DeltaPsim) and annexin-positive cells, which was assessed by flow cytometry. Second, a dominant-negative form of JNK1 (dnJNK1) also reduced these apoptotic events, while a constitutively active form of JNK1, MKK7-JNK1beta, enhanced them. Finally, treatment with IFN-alpha enhanced the promoter activity of the TRAIL gene, which was partially abrogated by either JNK inhibitor or dnJNK1, while it was moderately enhanced by MKK7-JNK1beta. These findings are useful for understanding molecular mechanisms of IFN-alpha-induced apoptosis and also for development of treatment modalities of some tumors with IFN-alpha. 相似文献
68.
Teresa Ribeiro Jo?o Loureiro Concei??o Santos Leonor Morais-Cec��lio 《Tree Genetics & Genomes》2011,7(6):1113-1122
The Fagaceae is one of the most important plant families in European forest ecosystems, and it includes several genera distributed
in the Northern hemisphere. In this work we studied the genome organization and evolution within the family, by karyotyping
and physically mapping rDNA in ten European and Asian species of the genera Fagus, Quercus, and Castanea. All of the species studied had a chromosome number of 2n=2x=24, except for the first report of a single individual of Quercus suber which proved to be triploid (2n=3x=36). The rDNA physical mapping revealed several patterns: the dominant one is present in European and Asian Quercus subgenus Quercus, and in Castanea sativa and Castanea crenata, consisting of two 18S–25S rDNA loci (one subterminal major and one pericentromeric minor) and one 5S rDNA pericentromeric
locus. In Fagus sylvatica and in Quercus sessilifolia, different patterns were observed: four terminal 18S–25S rDNA loci and two 5S rDNA pericentromeric loci in the former, and
five 18S–25S rDNA loci (three terminal and two intercalary) and one 5S rDNA pericentromeric locus in the latter. In Castanea mollissima a distinct rDNA distribution pattern with two intercalary 18S–25S rDNA loci and two 5S rDNA was found. These findings suggest
rDNA loci restructuring during Castanea evolution, and variability of 18S–25S loci between Quercus and Cyclobalanopsis subgenera. 相似文献
69.
Rastislav?Varha? Marián?AntalíkEmail author Mikulá??Bánó 《Journal of biological inorganic chemistry》2004,9(1):12-22
Thermally denatured horse heart ferrocytochrome c (ferrocyt c) has been characterized using absorption spectroscopy, differential scanning calorimetry (DSC) and viscometry at pH 7.0. DSC experiments have yielded the transition temperature of denaturant-free ferrocyt c unfolding as 100.6±0.3 °C, indicating an extremely high stability of the protein. The presence of guanidine hydrochloride (GdnHCl) facilitated estimation of the structural features of thermally unfolded ferrocyt c. The stability of the protein, expressed by G
D at 25 °C, is 59±5 kJ mol–1 (DSC) and 65±6 kJ mol–1 (absorption spectroscopy). An absorption spectrum of ferrocyt c demonstrates that the heme occurs in the high-spin state at extreme denaturing conditions (94 °C, 6.6 M GdnHCl). Absorption spectroscopy, using heme as a probe, shows that thermal denaturation of ferrocyt c occurs as a transition from a native low-spin (Met80/His18) to a high-spin disordered state with involvement of non-native, low-spin (bis-His) species.Abbreviations CD
circular dichroism
- cyt c
cytochrome c
- DSC
differential scanning calorimetry
- ferricyt c
ferricytochrome c
- ferrocyt c
ferrocytochrome c
- GdnHCl
guanidine hydrochloride
- NHE
normal hydrogen electrode 相似文献
70.