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91.
Summary The administration of progesterone to ovariectomized rats induces an increase in the volume density (Vv) of the mitochondria and the appearance of giant mitochondria in the uterine glandular cells. This experimental model, including a stereological analysis, allowed us to investigate and quantify a direct effect of progesterone on a well-defined cellular structure without the intervention of estrogen in a priming phase. Synthetic compounds, promegestone, gestrinone and RU 38486, were tested in this model either in place of progesterone or simultaneously with progesterone. The potent progestomimetic activity of promegestone was confirmed by the proliferation of giant mitochondria and a high Vv value for the mitochondria, the two other compounds being inactive even at higher doses. At lower doses, gestrinone and RU 38486 partially inhibit the action of progesterone and at higher doses they both show a complete antagonist effect by preventing the development of the mitochondria. 相似文献
92.
H. de Verneuil B. Grandchamp Chantal Foubert Dominique Weil Cong Van N'Guyen Marie-Sylvie Gross Shigeru Sassa Y. Nordmann 《Human genetics》1984,66(2-3):202-205
Summary A specific enzyme immunoassay of uroporphyrinogen decarboxylase was developed and applied to the detection of the human enzyme in man-rodent somatic cell hybrids. This method allowed to assign the gene for uroporphyrinogen decarboxylase to human chromosome 1. 相似文献
93.
Summary Hemoglobin J Mexico, an chain mutant, was studied in eight unrelated Algerian families. The quantities of the abnormal hemoglobin in 116 subjects are trimodally distributed: 55% in homozygotes, 31% and 38% in heterozygotes. Both hematological data and the / chain biosynthetic ratio are normal in heterozygotes with 31% Hb J and in homozygotes. In contrast, the MCV and MCH as well as the / biosynthetic ratio are slightly reduced in heterozygotes with 38% Hb J and in their relatives carrying Hb A. The elevated expression of J chains in heterozygotes with 38% Hb J may be due to an thalassemia gene trans to the >J locus. 相似文献
94.
Axel Kahn Hélène-Annie Buc Robert Girot Dominique Cottreau Claude Griscelli 《Human genetics》1978,40(3):293-304
Summary Two new deficient glucose-phosphate-isomerase (GPI) variants have been described in patients suffering from severe chronic hemolytic anemias. The patients' parents were consanguineous, such that the patients were true homozygotes for the mutated GPI genes. In both cases the main cause of the defect in enzyme activity was molecular instability of the mutated GPI molecules, their catalytic activity being nearly normal.GPI Paris was characterized by a slow electrophoretic migration and, above all, a drastically altered affinity for the substrates glucose-6-phosphate (decreased) and fructose-6-phosphate (increased). GPI Enfants malades exhibited a slightly reduced electrophoretic mobility, an abnormal curve of the activity in function of pH, and an abnormal ratio of maximal velocity in the backward direction (fructose-6-phosphate»glucose-6-phosphate) to that in the forward direction (glucose-6-phosphate»fructose-6-phosphate).No clear relation could be proved between the kinetic abnormalities of the mutant GPI variants on the one hand and the metabolic changes of the GPI-deficient red cells and the severity of hemolysis on the other.Finally we emphasized the possible role of the impairment of hexosemonophosphate pathway in the reduction of viability of the GPI-deficient red cells. 相似文献
95.
96.
Marie-Claire Meienhofer Dominique Cottreau Jean-Claude Dreyfus Axel Kahn 《FEBS letters》1980,110(2):219-222
97.
Monique Bedin Dominique Weil Thérèse Fournier Lise Cedard Jean Frezal 《Human genetics》1981,59(3):256-258
Summary Steroid sulfatase activities are significantly higher in placentas obtained after the birth of girls than after the birth of boys, and also in female fibroblasts compared to male strains. This constitutes biochemical evidence for the non-inactivation of the X-linked sulfatase locus. No hydrolytic activity is found in the fibroblasts of ichthyotic boys. Heterozygosity is demonstrated in the fibroblasts of the four mothers studied, as they have steroid sulfatase activity of less or equivalent to the normal male value. 相似文献
98.
Using a number of intrafamilial PLTs raised against identical HLA haplotypes it has been possible to construct a model in an informative family defining the HLA-D region as a genetic system. This system consists of at least two regions separated by a recombination between HLA-D and GLO. In relation to the site of recombination, a minimum of one centromeric and three telomeric components can be identified per haplotype.—Fourteen PLTs raised and defined within the family were subsequently tested in a Caucasian population (n=84) and in 13 unrelated, complete families.—It is concluded that the hypothetical model proposed for the HLA-D region as a genetic system of linked loci, coding at the cell surface for associated but distinct components (at least four per haplotype), allows for typing of the components of the HLA-D system of any given haplotype. Serological typing of HLA-D components should, in the near future, provide a more convenient way of establishing component phenotypes than the present use of primed lymphocyte typing reagents. Among the components isolated, some have a high association with the classic alleles defined either by homozygous typing cells or DR serology. Others form the basis of cross-reactivity but their presence does not interfere with standard typing. Others, however, seem by their mere presence to be responsible for false assignments.—The concept of HLA-D as a genetic system clarifies many of the inconsistencies observed with a one-locus system.Research scientists from INSERM.Research Fellow from the Danish Medical Research Council.Central Blood Bank — Marseille 相似文献
99.
Dominique Weil Nguyen Van-Cong Catherine Finaz R. Rebourcet Chantal Cochet J. de Grouchy J. Frézal 《Human genetics》1977,36(2):205-211
Summary 22 independent man-hamster (HGPRT–) hybrids using male human cells with balanced reciprocal translocation t(X;2)(p22;q32) were analysed for human genes localized on chromosome 2 (IDHS, MDHS), on chromosome X (PGK, GAL, G6PD) and for the different chromosomes in relation with the balanced reciprocal translocation (chr.2, chr.2q–, chr.Xp+).The following results were obtained:The chromosomes 2 and 2q– are absent in the 22 hybrids.In 9 hybrids, the absence of MDHS in spite of the presence of the chromosome Xp+ indicates that the gene for MDHS is not localized on this chromosome (or that the gene for MDHS is not on the segment 2q32-2qter translocated on X).In 14 hybrids, the three markers of X (PGK, GAL, G6PD) and IDHS are expressed in the presence of the chromosome Xp+. This result indicates that the genes for these markers are on Xp+ or that the genes PGK, GAL, G6PD are on X without the Xp22-Xter segment, translocated on the chr.2, and that the gene for IDHS is on the 2q32-2qter segment translocated on X.In 8 hybrids, in the absence of the intact chromosome Xp+, the higher percentage of the presence of G6PD (7 hybrids) and the lower percentage of the presence of IDHS (3 hybrids) are explained by the fact that these hybrids selected in HAT medium had to retain a segment of Xp+ bearing the human gene HGPRT. G6PD appeared very close to HGPRT and IDHS very distant from HGPRT.The study of the different correlations between the presence and the absence of these four markers on Xp+ in the different hybrids indicates the following order on the chromosome Xp+ from p to q: IDHs — PGK — GAL — G6PD.
Groupe INSERM: Directeur J. Frézal
Groupe CNRS, ER, 149: Directeur J. de Grouchy 相似文献
Groupe INSERM: Directeur J. Frézal
Groupe CNRS, ER, 149: Directeur J. de Grouchy 相似文献
100.
Françoise Phan-Dinh-Tuy Anne Weber Joëlle Henry Dominique Cottreau Axel Kahn 《Analytical biochemistry》1982,127(1):73-80
Electrophoresis on cellulose acetate strips was used to analyze protein kinases from normal rat liver. In addition to already well-characterized cAMP-dependent protein kinases type I and II and cAMP-independent casein kinases I and II, this method enabled the detection of several supplementary bands corresponding to kinases which were investigated according to their substrate specificity, activation by cAMP, and inhibition by the specific inhibitor of the catalytic subunit of cAMP-dependent protein kinases or by heparin. Using this rapid, sensitive, and resolutive electrophoretic method, different isozyme patterns could be obtained starting from minute amounts of different types of biological material. 相似文献