全文获取类型
收费全文 | 92074篇 |
免费 | 8331篇 |
国内免费 | 43篇 |
出版年
2023年 | 253篇 |
2022年 | 731篇 |
2021年 | 1619篇 |
2020年 | 923篇 |
2019年 | 1186篇 |
2018年 | 1502篇 |
2017年 | 1296篇 |
2016年 | 2232篇 |
2015年 | 3776篇 |
2014年 | 4211篇 |
2013年 | 5022篇 |
2012年 | 6832篇 |
2011年 | 6742篇 |
2010年 | 4315篇 |
2009年 | 3948篇 |
2008年 | 5634篇 |
2007年 | 5652篇 |
2006年 | 5434篇 |
2005年 | 5239篇 |
2004年 | 5085篇 |
2003年 | 4877篇 |
2002年 | 4604篇 |
2001年 | 1046篇 |
2000年 | 830篇 |
1999年 | 1109篇 |
1998年 | 1315篇 |
1997年 | 892篇 |
1996年 | 820篇 |
1995年 | 730篇 |
1994年 | 679篇 |
1993年 | 739篇 |
1992年 | 701篇 |
1991年 | 653篇 |
1990年 | 598篇 |
1989年 | 563篇 |
1988年 | 549篇 |
1987年 | 465篇 |
1986年 | 415篇 |
1985年 | 515篇 |
1984年 | 635篇 |
1983年 | 492篇 |
1982年 | 578篇 |
1981年 | 562篇 |
1980年 | 490篇 |
1979年 | 403篇 |
1978年 | 410篇 |
1977年 | 342篇 |
1976年 | 333篇 |
1975年 | 274篇 |
1974年 | 321篇 |
排序方式: 共有10000条查询结果,搜索用时 15 毫秒
971.
972.
Jane Stratford Michael A. Wright Walter Reineke Heiner Mokross Jurgen Havel Christopher J. Knowles Gary K. Robinson 《Archives of microbiology》1996,165(3):213-218
Chlorobenzoates (CBA) arise as intermediates during the degradation of polychlorinated biphenyls (PCBs) and some chlorinated herbicides. Since PCBs were produced as complex mixtures, a range of mono-, di-, and possibly trichloro-substituted benzoates would be formed. Chlorobenzoate degradation has been proposed to be one of the rate-limiting steps in the overall PCB-degradation process. Three hybrid bacteria constructed to have the ability to completely mineralise 2-, 3-, or 4-monochlorobiphenyl respectively, have been studied to establish the range of mono- and diCBAs that can be utilised. The three strains were able to mineralise one or more of the following CBAs: 2-, 3-, and 4-monochlorobenzoate and 3,5-dichlorobenzoate. No utilisation of 2,3-, 2,5-, 2,6-, or 3,4-diCBA was observed, and only a low concentration (0.11 mM) of 2,4-diCBA was mineralised. When the strain with the widest substrate range (Burkholderia cepacia JHR22) was simultaneously supplied with two CBAs, one that it could utilise plus one that it was unable to utilise, inhibitory effects were observed. The utilisation of 2-CBA (2.5 mM) by this strain was inhibited by 2,3-CBA (200 M) and 3,4-CBA (50 M). Although 2,5-CBA and 2,6-CBA were not utilised as carbon sources by strain JHR22, they did not inhibit 2-CBA utilisation at the concentrations studied, whereas 2,4-CBA was co-metabolised with 2-CBA. The utilisation of 2-, 3-, and 4-chlorobiphenyl by strain JHR22 was also inhibited by the presence of 2,3- or 3,4-diCBA. We conclude that the effect of the formation of toxic intermediates is an important consideration when designing remediation strategies.Abbreviations
PCB
Polychlorinated biphenyl
-
CBA
Chlorobenzoate 相似文献
973.
A high frequency of distinct ATM gene mutations in ataxia-telangiectasia. 总被引:10,自引:1,他引:9
下载免费PDF全文
![点击此处可从《American journal of human genetics》网站下载免费的PDF全文](/ch/ext_images/free.gif)
J. Wright S. Teraoka S. Onengut A. Tolun R. A. Gatti H. D. Ochs P. Concannon 《American journal of human genetics》1996,59(4):839-846
The clinical features of the autosomal recessive disorder ataxia-telangiectasia (AT) include a progressive cerebellar ataxia, hypersensitivity to ionizing radiation, and an increased susceptibility to malignancies. Epidemiological studies have suggested that AT heterozygotes may also be at increased risk for malignancy, possibly as a consequence of radiation exposure. A gene mutated in AT patients (ATM) has recently been isolated, making mutation screening in both patients and the general population possible. Because of the relatively large size of the ATM gene, the design of screening programs will depend on the types and distribution of mutations in the general population. In this report, we describe 30 mutations identified in a panel of unrelated AT patients and controls. Twenty-five of the 30 were distinct, and most patients were compound heterozygotes. The most frequently detected mutation was found in three different families and had previously been reported in five others. This corresponds to a frequency of 8% of all reported ATM mutations. Twenty-two of the alterations observed would be predicted to lead to protein truncation at sites scattered throughout the molecule. Two fibroblast cell lines, which displayed normal responses to ionizing radiation, also proved to be heterozygous for truncation mutations of ATM. These observations suggest that the carrier frequency of ATM mutations may be sufficiently high to make population screening practical. However, such screening may need to be done prospectively, that is, by searching for new mutations rather than by screening for just those already identified in AT families. 相似文献
974.
A recombination outside the BB deletion refines the location of the X linked retinitis pigmentosa locus RP3. 总被引:1,自引:1,他引:0
下载免费PDF全文
![点击此处可从《American journal of human genetics》网站下载免费的PDF全文](/ch/ext_images/free.gif)
R. Fujita E. Bingham P. Forsythe C. McHenry V. Aita B. A. Navia K. Dry M. Segal M. Devoto G. Bruns A. F. Wright J. Ott P. A. Sieving A. Swaroop 《American journal of human genetics》1996,59(1):152-158
Genetic loci for X-linked retinitis pigmentosa (XLRP) have been mapped between Xp11.22 and Xp22.13 (RP2, RP3, RP6, and RP15). The RP3 gene, which is responsible for the predominant form of XLRP in most Caucasian populations, has been localized to Xp21.1 by linkage analysis and the map positions of chromosomal deletions associated with the disease. Previous linkage studies have suggested that RP3 is flanked by the markers DXS1110 (distal) and OTC (proximal). Patient BB was thought to have RP because of a lesion at the RP3 locus, in addition to chronic granulomatous disease, Duchenne muscular dystrophy (DMD), mild mental retardation, and the McLeod phenotype. This patient carried a deletion extending approximately 3 Mb from DMD in Xp21.3 to Xp21.1, with the proximal breakpoint located approximately 40 kb centromeric to DXS1110. The RP3 gene, therefore, is believed to reside between DXS1110 and the proximal breakpoint of the BB deletion. In order to refine the location of RP3 and to ascertain patients with RP3, we have been analyzing several XLRP families for linkage to Xp markers. Linkage analysis in an American family of 27 individuals demonstrates segregation of XLRP with markers in Xp21.1, consistent with the RP3 subtype. One affected mate shows a recombination event proximal to DXS1110. Additional markers within the DXS1110-OTC interval show that the crossover is between two novel polymorphic markers, DXS8349 and M6, both of which are present in BB DNA and lie centromeric to the proximal breakpoint. This recombination places the XLRP mutation in this family outside the BB deletion and redefines the location of RP3. 相似文献
975.
976.
Microbial Utilization of Estuarine Dissolved Organic Carbon: a Stable Isotope Tracer Approach Tested by Mass Balance 总被引:2,自引:0,他引:2
下载免费PDF全文
![点击此处可从《Applied microbiology》网站下载免费的PDF全文](/ch/ext_images/free.gif)
The natural stable isotope values of different plants have been used to trace the fate of organic carbon that enters estuarine ecosystems. Experiments were designed to determine the magnitude of (delta) (sup13)C changes of dissolved organic carbon (DOC) derived from tidal marsh vegetation that occurred during bacterial decomposition. Bacteria were grown on DOC leached from estuarine Spartina alterniflora and Typhus angustifolia plants. In four experiments, 25 to 80% of the initial carbon (2.6 to 9.1 mM organic C) was converted to bacterial biomass and CO(inf2). Mass balance calculations showed good recovery of total C and (sup13)C at the end of these experiments (100% (plusmn) 14% total C; (plusmn) 1(permil) (delta) (sup13)C). The (delta) (sup13)C values of DOC, bacterial biomass, and respired CO(inf2) changed only slightly in the four experiments by average values of -0.6, +1.4, and +0.5(permil), respectively. These changes are small relative to the range of (delta) (sup13)C values represented by different organic carbon sources to estuaries. Thus, microbial (delta) (sup13)C values determined in the field helped to identify the source of the carbon assimilated by bacteria. 相似文献
977.
MORBILLIVIRUS INFECTION IN BOTTLENOSE DOLPHINS: EVIDENCE FOR RECURRENT EPIZOOTICS IN THE WESTERN ATLANTIC AND GULF OF MEXICO 总被引:1,自引:0,他引:1
Pádraig J. Duignan Carol House Daniel K. Odell Randall S. Wells Larry J. Hansen Michael T. Walsh David J. St . Aubin Bert K. Rima Joseph R. Geraci 《Marine Mammal Science》1996,12(4):499-515
Morbillivirus infection is widespread among odontocetes of the western Atlantic and Gulf of Mexico. Serologic evidence of infection in bottlenose dolphins, Tursiops truncatus , was first detected during an epizootic along the mid-Atlantic coast in 1987. Here, we report recurrent epizootics in the coastal dolphin population since at least the early 1980s based on serological surveys and regional stranding frequencies. The first observed epizootic of this series occurred in the Indian and Banana Rivers in 1982 and was followed by others on the mid-Atlantic coast in 1987–1988 and in the Gulf of Mexico between 1992 and 1994. This temporal pattern of infection is likely facilitated by the population size and its fragmentation into relatively discrete coastal communities. Introduction of morbillivirus into a community with a sufficient number of naive hosts may precipitate an epizootic, depending on the potential for transmission within the group. Propagation of an epizootic along the coast is probably determined by frequency of contact between adjacent communities and seasonal migrations.
Morbillivirus antibodies were also detected in serum from offshore bottlenose dolphins. The sero-prevalence in the latter may be higher than in coastal dolphins because of their close association with enzootically infected pilot whales ( Globicephala spp.). Occasional contact between offshore and coastal dolphins may provide an epizootiologic link between pilot whales and coastal dolphin communities. 相似文献
Morbillivirus antibodies were also detected in serum from offshore bottlenose dolphins. The sero-prevalence in the latter may be higher than in coastal dolphins because of their close association with enzootically infected pilot whales ( Globicephala spp.). Occasional contact between offshore and coastal dolphins may provide an epizootiologic link between pilot whales and coastal dolphin communities. 相似文献
978.
Kjetil Taskn Rigmor Solberg Ying Zhao Vidar Hansson Tore Jahnsen Michael J. Siciliano 《Genomics》1996,36(3):535
We have determined the chromosomal localization of the gene for the catalytic subunit Cα of cAMP-dependent protein kinase (locus PRKACA) to human chromosome 19 using polymerase chain reaction (PCR) and Southern blot analysis of two different somatic cell hybrid mapping panels. In addition, PCR analysis of a chromosome 19 mapping panel revealed the presence of a human Cα-specific amplification product only in cell lines containing the region 19p13.1 to 19q12. Finally, two-color fluorescencein situhybridization to metaphase chromosomes using the human Cα cDNA and human chromosome 19 inter-Alu-PCR product as probes localized the human Cα gene to chromosome region 19p13.1. 相似文献
979.
Patricia Kramer Jennifer Yount Thomas Mitchell Dante LaMorticella Roque Carrero-Valenzuela Everett Lovrien Irene Maumenee Michael Litt 《Genomics》1996,35(3):539
Congenital cataracts are one of the most common major eye abnormalities and often lead to blindness in infants. At least a third of all cases are familial. Within this group, highly penetrant, autosomal dominant forms of congenital cataracts (ADCC) are most common. ADCC is a genetically heterogeneous group of disorders, in which at least eight different loci have been identified for nine clinically distinct forms. Among these, Armitageet al.(Nature Genet.9: 37–40, 1995) mapped a gene for cerulean blue cataracts to chromosome 17q24. Bodkeret al.(Am. J. Med. Genet.37: 54–59, 1990) described a large family with cerulean blue cataracts, in which the affected daughter of affected first cousins was presumed to be homozygous for the purported gene. We report linkage in this family to the region on chromosome 22q that includes two β crystallin genes (CRYBB2, CRYBB3) and one pseudogene (CRYBB2P1). The affected female in question is homozygous at all markers. 相似文献
980.
John B. Kerrison Vronique J. Arnould M. Michael Barmada Robert K. Koenekoop Barbara J. Schmeckpeper Irene H. Maumenee 《Genomics》1996,33(3):523
Congenital nystagmus is an idiopathic disorder characterized by bilateral ocular oscillations usually manifest during infancy. Vision is typically decreased due to slippage of images across the fovea. As such, visual acuity correlates with nystagmus intensity, which is the amplitude and frequency of eye movements at a given position of gaze. X-linked, autosomal dominant, and autosomal recessive pedigrees have been described, but no mapping studies have been published. We recently described a large pedigree with autosomal dominant congenital nystagmus. A genome-wide search resulted in six markers on 6p linked by two-point analysis at θ = 0 (D6S459, D6S452, D6S465, FTHP1, D6S257, D6S430). Haplotype analysis localizes the gene for autosomal dominant congenital motor nystagmus to an 18-cM region between D6S271 and D6S455. 相似文献