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111.
112.
Clinico‐molecular analysis of eleven patients with Hermansky–Pudlak type 5 syndrome,a mild form of HPS 下载免费PDF全文
Vincent Michaud Eulalie Lasseaux Claudio Plaisant Alain Verloes Yaumara Perdomo‐Trujillo Christian Hamel Nursel H. Elcioglu Bart Leroy Josseline Kaplan Pierre‐Simon Jouk Didier Lacombe Patricia Fergelot Fanny Morice‐Picard Benoit Arveiler 《Pigment cell & melanoma research》2017,30(6):563-570
Hermansky–Pudlak syndrome (HPS), first described in 1959, is a rare form of syndromic oculocutaneous albinism associated with bleeding diathesis and in some cases pulmonary fibrosis and granulomatous colitis. All 10 HPS types are caused by defects in vesicle trafficking of lysosome‐related organelles (LRO) proteins. The HPS5 protein associates with HPS3 and HPS6 to form the biogenesis of lysosome‐related organelles complex‐2 (BLOC‐2). Here, we report the clinical and genetic data of 11 patients with HPS‐5 analyzed in our laboratory. We report 11 new pathogenic variants. The 11 patients present with ocular features that are typical for albinism, with mild hypopigmentation, and with no other major complication, apart from a tendency to bleed. HPS‐5 therefore appears as a mild form of HPS, which is often clinically undistinguishable from mild oculocutaneous or ocular forms of albinism. Molecular analysis is therefore required to establish the diagnosis of this mild HPS form, which has consequences in terms of prognosis and of clinical management of the patients. 相似文献
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115.
Nathalie Sibille Xavier Hanoulle Fanny Bonachera Dries Verdegem Isabelle Landrieu Jean-Michel Wieruszeski Guy Lippens 《Journal of biomolecular NMR》2009,43(4):219-227
Adding the 13C labelled 2-keto-isovalerate and 2-oxobutanoate precursors to a minimal medium composed of 12C labelled glucose instead of the commonly used (2D, 13C) glucose leads not only to the 13C labelling of (I, L, V) methyls but also to the selective 13C labelling of the backbone Cα and CO carbons of the Ile and Val residues. As a result, the backbone (1H, 15N) correlations of the Ile and Val residues and their next neighbours in the (i + 1) position can be selectively identified in HN(CA) and HN(CO) planes. The availability of a selective HSQC spectrum corresponding
to the sole amide resonances of the Ile and Val residues allows connecting them to their corresponding methyls by the intra-residue
NOE effect, and should therefore be applicable to larger systems. 相似文献
116.
Frédéric Schmitt Padavattan Govindaswamy Olivier Zava Georg Süss-Fink Lucienne Juillerat-Jeanneret Bruno Therrien 《Journal of biological inorganic chemistry》2009,14(1):101-109
Mononuclear 5-(4-pyridyl)-10,15,20-triphenylporphyrin and 5-(3-pyridyl)-10,15,20-triphenylporphyrin as well as tetranuclear
5,10,15,20-tetra(4-pyridyl)porphyrin (tetra-4-pp) and 5,10,15,20-tetra(3-pyridyl)porphyrin) (tetra-3-pp) arene ruthenium(II)
derivatives (arene is C6H5Me or p-Pr
i
C6H4Me) were prepared and evaluated as potential dual photosensitizers and chemotherapeutics in human Me300 melanoma cells. In
the absence of light, all tetranuclear complexes were cytotoxic (IC50 ≤ 20 μM), while the mononuclear derivatives were not (IC50 ≥ 100 μM). Kinetic studies of tritiated thymidine and tritiated leucine incorporations in cells exposed to a low concentration
(5 μM) of tetranuclear p-cymene derivatives demonstrated a rapid inhibition of DNA synthesis, while protein synthesis was inhibited only later, suggesting
arene ruthenium–DNA interactions as the initial cytotoxic process. All complexes exhibited phototoxicities toward melanoma
cells when exposed to laser light of 652 nm. At low concentration (5 μM), LD50 of the mononuclear derivatives was between 5 and 10 J/cm2, while for the tetranuclear derivatives LD50 was approximately 2.5 J/cm2 for the [Ru4(η6-arene)4(tetra-4-pp)Cl8] complexes and less than 0.5 J/cm2 for the [Ru4(η6-arene)4(tetra-3-pp)Cl8] complexes. Examination of cells under a fluorescence microscope revealed the [Ru4(η6-arene)4(tetra-4-pp)Cl8] complexes as cytoplasmic aggregates, whereas the [Ru4(η6-arene)4(tetra-3-pp)Cl8] complexes were homogenously dispersed in the cytoplasm. Thus, these complexes present a dual synergistic effect with good
properties of both the arene ruthenium chemotherapeutics and the porphyrin photosensitizer. 相似文献
117.
Gregor Kozlowski Sandra Bürcher Matthieu Fleury Fanny Huber 《Biodiversity and Conservation》2009,18(3):649-662
The purpose of this study was to examine the composition, distribution, ecology, and conservation status of the Atlantic elements
of the Swiss flora. About 195 Atlantic and 80 Mediterranean–Atlantic vascular plant species of the European flora have been
used as the basis for our analysis. The complete list of 3,143 taxa has been used as the reference for the Swiss flora. The
distributions of the species are illustrated in coincidence maps based on the computer database of the Data Centre of the
Swiss Flora in Geneva, Switzerland. Our study demonstrates clearly that the Atlantic flora of Europe requires a new biogeographical
appraisal. The Swiss flora comprises 66 Atlantic and Mediterranean–Atlantic taxa, which are taxonomically and ecologically
highly diverse. Switzerland contains 44% of all European Sub-Atlantic plants. This confirms the Sub-Atlantic geographical
position of Switzerland. Only one Eu-Atlantic species growing in Switzerland, Vicia orobus, can be classified as native with certainty. This species is critically endangered and merits the highest conservation priority.
Although a very alpine country, Switzerland has a relatively large number of Mediterranean–Atlantic species. The Atlantic
and Mediterranean–Atlantic plants are a very threatened group in Switzerland, with wetland plants the most imperilled ecological
group.
Electronic supplementary material The online version of this article (doi:) contains supplementary material, which is available to authorized users. 相似文献
118.
Jochen Heinrichs Fanny Klugmann Jrn Hentschel Harald Schneider 《Molecular phylogenetics and evolution》2009,53(1):113-121
The Neotropical-African liverwort Marchesinia brachiata has long been regarded as a polymorphic species. This hypothesis is examined using a dataset including sequences of the nuclear internal transcribed spacer region and the plastidic trnL–trnF region of 39 Marchesinia accessions. Maximum parsimony, maximum likelihood and Bayesian analyses indicate that Marchesinia robusta is nested within M. brachiata s.l. The molecular topologies support at least three partly sympatric biological species within M. brachiata s.l., the Neotropical M. bongardiana and M. languida, and the Neotropical-African M. brachiata s.s. These species are incompletely separated by subtle differences in underleaf shape and leaf dentation. Long branches within M. brachiata s.s. suggest ongoing speciation processes that are not yet reflected in distinguishable morphological variation. Divergence time estimates based on nrITS sequence variation and the liverwort fossil record indicate an establishment of the species M. bongardiana, M. brachiata, M. languida, M. madagassa, and M. robusta in the Late Oligocene and Miocene. The intraspecific diversity shows distinctive patterns with evidence for constant accumulation of genetic diversity in M. robusta and M. brachiata whereas M. bongardiana and M. languida likely went through a recent extinction or expansion process as indicated by the bottleneck pattern of genetic diversity. The tropical American-African disjunction of M. brachiata is the result of dispersal rather than Western Gondwanan vicariance. 相似文献
119.
Vanessa Machault Libasse Gadiaga Cécile Vignolles Fanny Jarjaval Samia Bouzid Cheikh Sokhna Jean-Pierre Lacaux Jean-François Trape Christophe Rogier Frédéric Pagès 《Malaria journal》2009,8(1):1-21
Background
Urbanization has a great impact on the composition of the vector system and malaria transmission dynamics. In Dakar, some malaria cases are autochthonous but parasite rates and incidences of clinical malaria attacks have been recorded at low levels. Ecological heterogeneity of malaria transmission was investigated in Dakar, in order to characterize the Anopheles breeding sites in the city and to study the dynamics of larval density and adult aggressiveness in ten characteristically different urban areas.Methods
Ten study areas were sampled in Dakar and Pikine. Mosquitoes were collected by human landing collection during four nights in each area (120 person-nights). The Plasmodium falciparum circumsporozoite (CSP) index was measured by ELISA and the entomological inoculation rates (EIR) were calculated. Open water collections in the study areas were monitored weekly for physico-chemical characterization and the presence of anopheline larvae. Adult mosquitoes and hatched larvae were identified morphologically and by molecular methods.Results
In September-October 2007, 19,451 adult mosquitoes were caught among which, 1,101 were Anopheles gambiae s.l. The Human Biting Rate ranged from 0.1 bites per person per night in Yoff Village to 43.7 in Almadies. Seven out of 1,101 An. gambiae s.l. were found to be positive for P. falciparum (CSP index = 0.64%). EIR ranged from 0 infected bites per person per year in Yoff Village to 16.8 in Almadies. The An. gambiae complex population was composed of Anopheles arabiensis (94.8%) and Anopheles melas (5.2%). None of the An. melas were infected with P. falciparum. Of the 54 water collection sites monitored, 33 (61.1%) served as anopheline breeding sites on at least one observation. No An. melas was identified among the larval samples. Some physico-chemical characteristics of water bodies were associated with the presence/absence of anopheline larvae and with larval density. A very close parallel between larval and adult densities was found in six of the ten study areas.Conclusion
The results provide evidence of malaria transmission in downtown Dakar and its surrounding suburbs. Spatial heterogeneity of human biting rates was very marked and malaria transmission was highly focal. In Dakar, mean figures for transmission would not provide a comprehensive picture of the entomological situation; risk evaluation should therefore be undertaken on a small scale. 相似文献120.