全文获取类型
收费全文 | 2899篇 |
免费 | 115篇 |
国内免费 | 276篇 |
专业分类
系统科学 | 824篇 |
丛书文集 | 19篇 |
教育与普及 | 70篇 |
理论与方法论 | 20篇 |
研究方法 | 29篇 |
综合类 | 2328篇 |
出版年
2022年 | 28篇 |
2021年 | 81篇 |
2020年 | 49篇 |
2019年 | 11篇 |
2018年 | 19篇 |
2016年 | 7篇 |
2015年 | 5篇 |
2014年 | 192篇 |
2013年 | 264篇 |
2012年 | 231篇 |
2011年 | 189篇 |
2010年 | 24篇 |
2009年 | 23篇 |
2008年 | 41篇 |
2007年 | 158篇 |
2006年 | 93篇 |
2005年 | 124篇 |
2004年 | 94篇 |
2003年 | 178篇 |
2002年 | 76篇 |
2001年 | 109篇 |
2000年 | 154篇 |
1999年 | 316篇 |
1998年 | 94篇 |
1997年 | 71篇 |
1996年 | 136篇 |
1995年 | 125篇 |
1994年 | 127篇 |
1993年 | 18篇 |
1992年 | 48篇 |
1991年 | 15篇 |
1990年 | 16篇 |
1989年 | 7篇 |
1988年 | 24篇 |
1987年 | 17篇 |
1986年 | 14篇 |
1985年 | 8篇 |
1984年 | 13篇 |
1983年 | 6篇 |
1982年 | 6篇 |
1981年 | 7篇 |
1980年 | 7篇 |
1979年 | 8篇 |
1978年 | 10篇 |
1977年 | 4篇 |
1976年 | 4篇 |
1963年 | 5篇 |
1962年 | 5篇 |
1958年 | 4篇 |
1957年 | 4篇 |
排序方式: 共有3290条查询结果,搜索用时 359 毫秒
71.
RK Koenekoop H Wang J Majewski X Wang I Lopez H Ren Y Chen Y Li GA Fishman M Genead J Schwartzentruber N Solanki EI Traboulsi J Cheng CV Logan M McKibbin BE Hayward DA Parry CA Johnson M Nageeb;Finding of Rare Disease Genes 《Nature genetics》2012,44(9):1035-1039
Leber congenital amaurosis (LCA) is a blinding retinal disease that presents within the first year after birth. Using exome sequencing, we identified mutations in the nicotinamide adenine dinucleotide (NAD) synthase gene NMNAT1 encoding nicotinamide mononucleotide adenylyltransferase 1 in eight families with LCA, including the family in which LCA was originally linked to the LCA9 locus. Notably, all individuals with NMNAT1 mutations also have macular colobomas, which are severe degenerative entities of the central retina (fovea) devoid of tissue and photoreceptors. Functional assays of the proteins encoded by the mutant alleles identified in our study showed that the mutations reduce the enzymatic activity of NMNAT1 in NAD biosynthesis and affect protein folding. Of note, recent characterization of the slow Wallerian degeneration (Wld(s)) mouse model, in which prolonged axonal survival after injury is observed, identified NMNAT1 as a neuroprotective protein when ectopically expressed. Our findings identify a new disease mechanism underlying LCA and provide the first link between endogenous NMNAT1 dysfunction and a human nervous system disorder. 相似文献
72.
Loos RJ Lindgren CM Li S Wheeler E Zhao JH Prokopenko I Inouye M Freathy RM Attwood AP Beckmann JS Berndt SI;Prostate Lung Colorectal Ovarian 《Nature genetics》2008,40(6):768-775
To identify common variants influencing body mass index (BMI), we analyzed genome-wide association data from 16,876 individuals of European descent. After previously reported variants in FTO, the strongest association signal (rs17782313, P = 2.9 x 10(-6)) mapped 188 kb downstream of MC4R (melanocortin-4 receptor), mutations of which are the leading cause of monogenic severe childhood-onset obesity. We confirmed the BMI association in 60,352 adults (per-allele effect = 0.05 Z-score units; P = 2.8 x 10(-15)) and 5,988 children aged 7-11 (0.13 Z-score units; P = 1.5 x 10(-8)). In case-control analyses (n = 10,583), the odds for severe childhood obesity reached 1.30 (P = 8.0 x 10(-11)). Furthermore, we observed overtransmission of the risk allele to obese offspring in 660 families (P (pedigree disequilibrium test average; PDT-avg) = 2.4 x 10(-4)). The SNP location and patterns of phenotypic associations are consistent with effects mediated through altered MC4R function. Our findings establish that common variants near MC4R influence fat mass, weight and obesity risk at the population level and reinforce the need for large-scale data integration to identify variants influencing continuous biomedical traits. 相似文献
73.
Scott RH Douglas J Baskcomb L Huxter N Barker K Hanks S Craft A Gerrard M Kohler JA Levitt GA Picton S Pizer B Ronghe MD Williams D;Factors Associated with Childhood Tumours 《Nature genetics》2008,40(11):1329-1334
Constitutional abnormalities at the imprinted 11p15 growth regulatory region cause syndromes characterized by disordered growth, some of which include a risk of Wilms tumor. We explored their possible contribution to nonsyndromic Wilms tumor and identified constitutional 11p15 abnormalities in genomic lymphocyte DNA from 13 of 437 individuals (3%) with sporadic Wilms tumor without features of growth disorders, including 12% of bilateral cases (P = 0.001) and in one familial Wilms tumor pedigree. No abnormality was detected in 220 controls (P = 0.006). Abnormalities identified included H19 DMR epimutations, uniparental disomy 11p15 and H19 DMR imprinting center mutations (one microinsertion and one microdeletion), thus identifying microinsertion as a new class of imprinting center mutation. Our data identify constitutional 11p15 defects as one of the most common known causes of Wilms tumor, provide mechanistic insights into imprinting disruption and reveal clinically important epigenotype-phenotype associations. The impact on clinical management dictates that constitutional 11p15 analysis should be considered in all individuals with Wilms tumor. 相似文献
74.
LI Hong 《系统科学与系统工程学报(英文版)》1999,(1)
1IntroductionEnvironment-economycoordinationdevelopmentisoneofthemostconcernsatpresenttime.However,whatiscoordinationdevelopment,itsspecificationandthekindofitsmodelstructureetc.arestillunknown.Infact,coordinationdevelopmentissimplyaslogan.Thispaperrepresentsthattheultimategoalofenvironmentalprotectionandeconomicdevelopmentistomeetthedemandofpeople.Thisisdecidedbythesubjectivevalueofthenecessitiesfortotalmankinddevelopment.Mankindproducesphysicalsubstanceandundertakesenvironmentalprotection,b… 相似文献
75.
Study on the REM Model for Group Decision Making 总被引:1,自引:0,他引:1
IntroductionAsocialchoicefunctionisamappingwhichassignsanonemptysubsetofthepotentialfeasiblesubsettoeachorderedpairconsistentofapotentialfeasiblesubsetofalternativesandascheduleorprofileofvoters'preferences.Consideranopinionaggregationprobleminwhichagroupofexpertsgivetheirscoresthatexpresstheirpreferencestoasetofalternativesanddesiretocombinetheirscoresintoasingleaggregationpreferenceoverthealternativeset.Theunderlyingideahereisthattheconsensusofnumerousexpertsresultsinasuperiorpreferenceonthe… 相似文献
76.
COHERENT, REGULAR AND SIMPLE SYSTEMS IN ZERO DECOMPOSITIONS OF PARTIAL DIFFERENTIAL SYSTEMS 总被引:1,自引:0,他引:1
LI Ziming 《系统科学与复杂性》1999,(Z1)
1.IntroductionWeareconcernedwithalgebraicdifferentialpolynomials(abbreviatedas'd-pcis)inafinitenumberofdifferentialindeterminatesoveradifferentialfieldofcharacteristic0.Givenafinitesetofsuchd-pcis,wewouldliketostudyd-Zero(),thedifferentialalgebraicsetdefinedbyPoverauniversaldifferentialfield.Wemayaskwhetherd-Zero()isempty,whetherad-pcivanishesond-Zero()(differentialradicalidealmembershipproblem),whatthedimensionofd-Zero()is,howtodecomposed-Zero()intoirreduciblealgebraicdifferentialsets,andso… 相似文献
77.
WANG Dongqian 《系统科学与复杂性》1999,(4)
1.IntroductionAnUInberofauthorshaveconsideredtheproblemoffittingmodelsofquasi-independencetocontingenCytabledatawhichareeithertriangUlarorhaveanexcludeddiagonal.hithispaperthevariousmethodsproposedforfittingthesemodelsarecompared.ItisthenshoWnthstparameterestimatesarethesamefordifferentmodelsinsomecases.Anew,verysample,easytoimplemeDtalgoritlunispreseDted.Finallysomepreviousiterstivemethodsarecomparedwiththenewmethod.2.Quasi-IndependenceModeisforContingencyamblesSupposethatatwo-waycolltin… 相似文献
78.
1.IntroductionThecolltrolproblemofnonlinearuncertainsystemshasbeenextensivelyresearchedformanyyears.Manymethodshavebeenproposedandsomeprogresshasbeenmade.[1]poiedsoutthatsomesecondordersystemshaveaninherelltpropertythatthereexistscertainregioninwhichalltrajectoriesofthesystemremaininginitaftercertaintimeTwillconvergetotheorigin.Basedonthisproperty,anewapproachhajsbeenputforwardtoconstructacolltinuousfeedbacklawsuchthatallsystem'strajectoriesmovetowardtheregionandeventuallyremaininit.In[2,3],a… 相似文献
79.
1.INTaODUCTIONThemultilayerfeedforwardneuralnetworks(MFNN)hasbeenwidelyappliedinmanyfieldssuchaspatternrecognition,nonlinearoptimization,systemidentificationandcontrol.Thesimpleback-propagation(BP)learningalgorithmisusuallyadoptedtoupdateitsweights.TheBPalgorithm,however,suffersdrawbacksofslowconvergenceandunpredictablesolutionsduringlearning.Althoughmailyapproachessuchasvariablestepsize,conjugategradient,pseudo-Newtonmethods[4]andothermethods[3,10],havebeendevelopedtoovercometheproble… 相似文献