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901.
B S Mankoo N S Collins P Ashby E Grigorieva L H Pevny A Candia C V Wright P W Rigby V Pachnis 《Nature》1999,400(6739):69-73
The skeletal muscles of the limbs develop from myogenic progenitors that originate in the paraxial mesoderm and migrate into the limb-bud mesenchyme. Among the genes known to be important for muscle development in mammalian embryos are those encoding the basic helix-loop-helix (bHLH) myogenic regulatory factors (MRFs; MyoD, Myf5, myogenin and MRF4) and Pax3, a paired-type homeobox gene that is critical for the development of limb musculature. Mox1 and Mox2 are closely related homeobox genes that are expressed in overlapping patterns in the paraxial mesoderm and its derivatives. Here we show that mice homozygous for a null mutation of Mox2 have a developmental defect of the limb musculature, characterized by an overall reduction in muscle mass and elimination of specific muscles. Mox2 is not needed for the migration of myogenic precursors into the limb bud, but it is essential for normal appendicular muscle formation and for the normal regulation of myogenic genes, as demonstrated by the downregulation of Pax3 and Myf5 but not MyoD in Mox2-deficient limb buds. Our findings show that the MOX2 homeoprotein is an important regulator of vertebrate limb myogenesis. 相似文献
902.
Structure and assembly of the 20S proteasome 总被引:3,自引:0,他引:3
W. L. H. Gerards W. W. de Jong W. Boelens H. Bloemendal 《Cellular and molecular life sciences : CMLS》1998,54(3):253-262
The barrel-shaped 20S proteasome is one of the two components of a larger 26S particle, the multicatalytic 2000-kDa protease
complex. The proteolytic sites are located in the inner chamber of the 20S particle and are only accessible via narrow entrances.
This paper reviews the current knowledge concerning proteasome formation, proteolytic activities, structural aspects and assembly.
Eukaryotic proteasomes are made up by four rings each of which contains seven different subunits occurring at fixed positions.
While the outer rings contain α-type subunits, the inner ones comprise β-type subunits. The current assembly model for eukaryotic 20S proteasomes is based upon the detection of 13S and 16S intermediates,
respectively, in addition to previous findings with archaebacterial and eubacterial proteasome assembly. The available data
suggest a cooperative assembly of the α-type and β-type subunits into half proteasome-like complexes followed by dimerization into proteasomes. During or after dimerization
of half proteasomes, the β-type subunits are processed. The prosequence of the β-type subunits is essential for the assembly process and prevents protease activity of immature proteasomes. 相似文献
903.
Henk W. de Regt 《Foundations of Science》1999,4(4):405-426
This article analyses an episode in the earlyhistory of quantum theory: the controversy betweenPauli and Heisenberg about the anomalous Zeemaneffect, which was a main stumbling block for the oldquantum theory of Bohr. It is argued that theindividual philosophical views of both Pauli andHeisenberg directed their attempts to solve theanomaly and decisively influenced the solutions theyproposed. The results of this case study arecompared with the assertions of four theories ofscientific change, namely those of Kuhn, Lakatos,Laudan and Giere. 相似文献
904.
A polycystic kidney-disease gene homologue required for male mating behaviour in C. elegans. 总被引:6,自引:0,他引:6
The stereotyped mating behaviour of the Caenorhabditis elegans male is made up of several substeps: response, backing, turning, vulva location, spicule insertion and sperm transfer. The complexity of this behaviour is reflected in the sexually dimorphic anatomy and nervous system. Behavioural functions have been assigned to most of the male-specific sensory neurons by means of cell ablations; for example, the hook sensory neurons HOA and HOB are specifically required for vulva location. We have investigated how sensory perception of the hermaphrodite by the C. elegans male controls mating behaviours. Here we identify a gene, lov-1 (for location of vulva), that is required for two male sensory behaviours: response and vulva location. lov-1 encodes a putative membrane protein with a mucin-like, serine-threonine-rich amino terminus followed by two blocks of homology to human polycystins, products of the autosomal dominant polycystic kidney-disease loci PKD1 and PKD2. LOV-1 is the closest C. elegans homologue of PKD1. lov-1 is expressed in adult males in sensory neurons of the rays, hook and head, which mediate response, vulva location, and potentially chemotaxis to hermaphrodites, respectively. PKD-2, the C. elegans homologue of PKD2, is localized to the same neurons as LOV-1, suggesting that they function in the same pathway. 相似文献
905.
根据岩性、沉积构造、古生物化石和地球化学标志,系统分析了博格达山南缘广泛发育的二叠系芦草沟组的沉积环境。研究表明:博格达山南缘芦草沟组深湖相沉积十分发育,并在此背景上沉积了近岸浊积扇和远岸浊积扇。其中,深湖相暗色泥、页岩和油页岩构成了良好的烃源岩。同时,认为吐哈盆地前侏罗系具有良好的勘探前景。 相似文献
906.
907.
908.
Mutations in the human homologue of mouse dl cause autosomal recessive and dominant hypohidrotic ectodermal dysplasia. 总被引:23,自引:0,他引:23
A W Monreal B M Ferguson D J Headon S L Street P A Overbeek J Zonana 《Nature genetics》1999,22(4):366-369
X-linked hypohidrotic ectodermal dysplasia results in abnormal morphogenesis of teeth, hair and eccrine sweat glands. The gene (ED1) responsible for the disorder has been identified, as well as the analogous X-linked gene (Ta) in the mouse. Autosomal recessive disorders, phenotypically indistinguishable from the X-linked forms, exist in humans and at two separate loci (crinkled, cr, and downless, dl) in mice. Dominant disorders, possibly allelic to the recessive loci, are seen in both species (ED3, Dlslk). A candidate gene has recently been identified at the dl locus that is mutated in both dl and Dlslk mutant alleles. We isolated and characterized its human DL homologue, and identified mutations in three families displaying recessive inheritance and two with dominant inheritance. The disorder does not map to the candidate gene locus in all autosomal recessive families, implying the existence of at least one additional human locus. The putative protein is predicted to have a single transmembrane domain, and shows similarity to two separate domains of the tumour necrosis factor receptor (TNFR) family. 相似文献
909.
D E Humphries G W Wong D S Friend M F Gurish W T Qiu C Huang A H Sharpe R L Stevens 《Nature》1999,400(6746):769-772
910.
C. Kohlhauser W. Mosgoeller H. Hoeger G. Lubec B. Lubec 《Cellular and molecular life sciences : CMLS》1999,55(11):1491-1501
Perinatal asphyxia (PA) is considered to lead to a variety of brain disorders including spasticity, epilepsy, mental retardation,
and minimal brain disorder syndromes and may form the basis for psychiatric and neurodegenerative diseases later in life.
We examined markers for neuronal transmission involved in the pathomechanisms of PA and candidates as mediators for long-term
sequelae. We tested tyrosine hydroxylase (TH) and the vesicular monoamine transporter (VMAT) representing the monoaminergic
system, the vesicular acetylcholine transporter (VAChT), and the excitatory amino acid carrier 1 (EAAC1), a neuronal subtype
of the glutamate transporter, using immunohistochemistry on brain sections of rats subjected to graded PA. Three months following
the asphyxiant insult immunoreactive (IR)-TH was decreased in striatum, hippocampus, thalamus, frontal cortex, and cerebellum;
IR-VMAT was increased, and IR-VAChT was decreased in striatum. IR-EAAC1 glutamate transporter was increased in frontal cortex.
The cholinergic, monoaminergic, and glutamatergic changes, still observed 3 months after the asphyxiant insult, may reflect
their involvement in the pathomechanisms of PA and indicate mechanisms leading to long-term complications of PA. The variable
consequences on the individual markers in several brain regions may be explained by specific susceptibility of cholinergic,
monoaminergic, and glutamatergic neurons to the asphyxiant insult.
Received 16 March 1999; received after revision 20 May 1999; accepted 8 July 1999 相似文献