全文获取类型
收费全文 | 15211篇 |
免费 | 983篇 |
国内免费 | 2篇 |
出版年
2022年 | 76篇 |
2021年 | 147篇 |
2020年 | 74篇 |
2019年 | 108篇 |
2018年 | 146篇 |
2017年 | 118篇 |
2016年 | 229篇 |
2015年 | 353篇 |
2014年 | 389篇 |
2013年 | 944篇 |
2012年 | 755篇 |
2011年 | 690篇 |
2010年 | 401篇 |
2009年 | 437篇 |
2008年 | 700篇 |
2007年 | 696篇 |
2006年 | 689篇 |
2005年 | 684篇 |
2004年 | 733篇 |
2003年 | 701篇 |
2002年 | 661篇 |
2001年 | 556篇 |
2000年 | 536篇 |
1999年 | 457篇 |
1998年 | 199篇 |
1997年 | 189篇 |
1996年 | 161篇 |
1995年 | 132篇 |
1994年 | 139篇 |
1993年 | 147篇 |
1992年 | 347篇 |
1991年 | 323篇 |
1990年 | 325篇 |
1989年 | 290篇 |
1988年 | 252篇 |
1987年 | 248篇 |
1986年 | 222篇 |
1985年 | 193篇 |
1984年 | 151篇 |
1983年 | 139篇 |
1982年 | 123篇 |
1981年 | 114篇 |
1980年 | 95篇 |
1979年 | 119篇 |
1978年 | 95篇 |
1977年 | 84篇 |
1976年 | 87篇 |
1975年 | 72篇 |
1974年 | 89篇 |
1973年 | 99篇 |
排序方式: 共有10000条查询结果,搜索用时 15 毫秒
101.
102.
Point mutations in the upstream region of the α-galactosidase A gene exon 6 in an atypical variant of Fabry disease 总被引:5,自引:0,他引:5
Summary Single point mutations in the upstream region of exon 6 of the -galactosidase A gene were found in two Japanese cases of the cardiac form of Fabry disease; 301ArgGln (902GA) in a case that has already been published and 279GlnGlu (835CG) in a new case. They both expressed markedly low, but significant, amounts of residual activity in COS-1 cells. In contrast, two unrelated cases with classic Fabry disease were found to have different point mutations, which showed a complete loss of enzyme activity in a transient expression assay; 328GlyArg (982GA) in the downstream region of exon 6 in one case and two combined mutations, 66GluGln (196GC)/112ArgCys (334CT), in exon 2 in the other. We conclude, on the basis of the results recorded in this study and those in previous reports, that the pathogenesis of atypical Fabry disease is closely associated with point mutations in the upstream region of exon 6 of the -galactosidase A gene. 相似文献
103.
Complementation study of peroxisome-deficient disorders by immunofluorescence staining and characterization of fused cells 总被引:5,自引:0,他引:5
Shigehiro Yajima Yasuyuki Suzuki Nobuyuki Shimozawa Seiji Yamaguchi Tadao Orii Yukio Fujiki Takashi Osumi Takashi Hashimoto Hugo W. Moser 《Human genetics》1992,88(5):491-499
Summary Genetic heterogeneity in peroxisome-deficient disorders, including Zellweger's cerebrohepatorenal syndrome, neonatal adrenoleukodystrophy and infantile Refsum disease, was investigated. Fibroblasts from 17 patients were fused using polyethylene glycol, cultivated on cover slips, and the formation of peroxisomes in the fused cells was visualized by immunofluorescence staining, using anti-human catalase IgG. Two distinct staining patterns were observed: (1) peroxisomes appeared in the majority of multinucleated cells, and (2) practically no peroxisomes were identified. Single step 12-(1-pyrene) dodecanoic acid/ultraviolet (P12/UV)-selection confirmed that the former groups were resistant to this selection, most of the surviving cells contained abundant peroxisomes, and the latter cells died. In the complementary matching, [1-14C]lignoceric acid oxidation and the biosynthesis of peroxisomal proteins were also normalized. Five complementation groups were identified. Group A: Zellweger syndrome and infantile Refsum disease; Groups B, C and D: Zellweger syndrome; Group E: Zellweger syndrome, neonatal adrenoleukodystrophy and infantile Refsum disease. We compared these groupings with those of Roscher and identified eight complementation groups. There was no obvious relation between complementation groups and clinical phenotypes. These results indicate that the transport, intracellular processing and function of peroxisomal proteins were normalized in the complementary matching and that at least eight different genes are involved in the formation of normal peroxisomes and in the transport of peroxisomal enzymes. 相似文献
104.
Structure of the Bombyx mori fibroin light-chain-encoding gene: upstream sequence elements common to the light and heavy chain. 总被引:21,自引:0,他引:21
Yoshimi Kikuchi Kazuyuki Mori Satoshi Suzuki Kazunori Yamaguchi Shigeki Mizuno 《Gene》1992,110(2):151-158
Two overlapping genomic clones containing the fibroin light-chain (Fib-L)-encoding gene (Fib-L) were obtained from the cosmid library of the silkworm, Bombyx mori J-139, by hybridization with the Fib-L cDNA clone. Sequencing of the 14.6-kb region revealed that Fib-L was 13472 bp long containing seven exons, and that the gene contained a large first intron which occupied about 60% of the gene. Comparison of restriction patterns of the J-139 Fib-L with those of eight other B. mori breeds producing normal-level fibroin demonstrated that considerable restriction-fragment length polymorphisms were present in regions containing the first intron and the 3′-flanking sequence. However, sizes of the Fib-L mRNA and the Fib-L polypeptide were very similar among the nine breeds tested, suggesting that the exon sequences and the splice signals were all well conserved. 5′-Flanking regions of Fib-L and the fibroin heavy-chain (Fib-H)-encoding gene (Fib-H) compared in this study contained three 18-30-bp sequences of high similarity and many 8-10-bp common elements, six of which coincided with the binding sites of homeodomain proteins. Gel retardation assays with the nuclear extracts of the posterior and middle silk glands suggested that protein factors present in the posterior silk-gland nuclei could bind to a set of those common upstream elements. 相似文献
105.
A comparative study on immunosuppressive effects of cyclosporin A and FK 506 on peripheral blood lymphocytes in dogs 总被引:1,自引:0,他引:1
Kyoichi Takaori Yoshinori Nio Kazutomo Inoue Thein Tun Manaba Fukumoto Tohru Hashida Masato Yasuhara Ryohei Hori Takayoshi Tobe 《Biotherapy》1992,4(2):129-137
Immunosuppressive effects of cyclosporin A (CsA) and FK 506 (FK) on peripheral blood lymphocytes were studied in dogs in respect to mixed lymphocyte reaction, proliferative responses to recombinant interleukin-2 (rIL-2), phytohemagglutinin (PHA) and concanavalin-A (Con-A); phenotypes of OKIa1, CD3, CD8 and surface IgM; cytotoxic activity against xenogeneic tumor cells. CsA (2.0 or 5.0 mg/kg, intravenously) or FK (0.16 mg/kg, intramuscularly) was given to mongrel dogs every morning for serial 21 days. The blood concentrations of CsA, measured as trough levels by fluorescence polarization method, ranged from 37 to 350 ng/ml in dogs administered at 2.0 mg/kg and from 170 to 894 ng/ml in dogs administered at 5.0 mg/kg during treatment, respectively. In dogs treated with FK at a dose of 0.16 mg/kg, the drug concentrations in the plasma during treatment ranged from 0.16 to 1.8 ng/ml. Mixed lymphocyte reaction and proliferative responses to rIL-2, PHA and Con-A, which were declined by CsA, were not affected by FK. In contrast, the proportion of OKIa1+ cells was not affected by CsA, whereas FK decreased the proportion of OKIa1+ cells progressively during the course of treatment. Cytotoxic activity was suppressed by both CsA and FK. These results possibly indicate that CsA and FK exert their immunosuppressive effects via different mechanisms. 相似文献
106.
107.
108.
109.
Summary We have developed a bioreactor system for aerobic fermentation, using a new ceramic carrier APHROCELL which has a suitable shape for liquid and gas passage. In acetic acid fermentation byAcetobacter cells from ethanol, as a typical example of aerobic fermentation, a productivity of 17.25 g/l h was attained at continuous production of 23 g-acetic acid/l; at an acetic acid concentration around 53 g/l, the productivity was 6.4 g/l h. Thus a marketable vinegar can be obtained continuously by this bioreactor system. Because of the simplicity of the APHROCELL reactor, scale up should be relatively easy. 相似文献
110.
Summary Monoclonal antibody production rateper hybridoma cell was measured as much larger in mouse peritoneal cavity thanin vitro culture. To identify factors enhancing antibody productivity per cell, a hybridoma was culturedin vitro with ascites and peritoneal exudate cells(PEC). Both the ascites and the PEC improved the productivity per cellin vitro. Each chromatograph-fraction of ascites was assayed for the enhancing activity. 相似文献