全文获取类型
收费全文 | 327篇 |
免费 | 1篇 |
国内免费 | 1篇 |
专业分类
系统科学 | 9篇 |
教育与普及 | 1篇 |
理论与方法论 | 2篇 |
现状及发展 | 68篇 |
研究方法 | 91篇 |
综合类 | 146篇 |
自然研究 | 12篇 |
出版年
2022年 | 2篇 |
2021年 | 1篇 |
2019年 | 1篇 |
2018年 | 4篇 |
2017年 | 7篇 |
2016年 | 11篇 |
2015年 | 2篇 |
2014年 | 8篇 |
2013年 | 5篇 |
2012年 | 34篇 |
2011年 | 45篇 |
2010年 | 15篇 |
2009年 | 3篇 |
2008年 | 23篇 |
2007年 | 28篇 |
2006年 | 24篇 |
2005年 | 22篇 |
2004年 | 19篇 |
2003年 | 20篇 |
2002年 | 22篇 |
2001年 | 2篇 |
2000年 | 2篇 |
1999年 | 2篇 |
1998年 | 2篇 |
1996年 | 1篇 |
1992年 | 2篇 |
1985年 | 2篇 |
1982年 | 1篇 |
1981年 | 1篇 |
1980年 | 3篇 |
1978年 | 1篇 |
1976年 | 1篇 |
1975年 | 2篇 |
1973年 | 1篇 |
1972年 | 1篇 |
1969年 | 1篇 |
1968年 | 1篇 |
1967年 | 3篇 |
1965年 | 2篇 |
1964年 | 1篇 |
1963年 | 1篇 |
排序方式: 共有329条查询结果,搜索用时 15 毫秒
321.
Dodé C Levilliers J Dupont JM De Paepe A Le Dû N Soussi-Yanicostas N Coimbra RS Delmaghani S Compain-Nouaille S Baverel F Pêcheux C Le Tessier D Cruaud C Delpech M Speleman F Vermeulen S Amalfitano A Bachelot Y Bouchard P Cabrol S Carel JC Delemarre-van de Waal H Goulet-Salmon B Kottler ML Richard O Sanchez-Franco F Saura R Young J Petit C Hardelin JP 《Nature genetics》2003,33(4):463-465
We took advantage of overlapping interstitial deletions at chromosome 8p11-p12 in two individuals with contiguous gene syndromes and defined an interval of roughly 540 kb associated with a dominant form of Kallmann syndrome, KAL2. We establish here that loss-of-function mutations in FGFR1 underlie KAL2 whereas a gain-of-function mutation in FGFR1 has been shown to cause a form of craniosynostosis. Moreover, we suggest that the KAL1 gene product, the extracellular matrix protein anosmin-1, is involved in FGF signaling and propose that the gender difference in anosmin-1 dosage (because KAL1 partially escapes X inactivation) explains the higher prevalence of the disease in males. 相似文献
322.
323.
Susan Leigh Star 《Systemic Practice and Action Research》1992,5(4):395-410
The paper summarizes recent work on organizations, artificial intelligence systems, human-computer interaction, etc., which emphasizes thesituated, distributed, andfluid nature of social systems. This contrasts with the traditional way of writing and thinking about social systems which sees them as disembodied, ideal, formal notions of thought. The implications of this new view of systems for social actors, information, knowledge, and technology are discussed. The literature reviewed offers a new way of talking about systems and their practices. 相似文献
324.
Anne Dain G. A. Kerkut R. C. Smith K. A. Munday T. H. Wilmshurst 《Cellular and molecular life sciences : CMLS》1964,20(2):76-78
Résumé L'addition de mélanine au blanc d'uf frais diminue la concentration des radicaux libres due à la lumière de 366 mµ. Cela permet de supposer que la mélanine agit comme un capteur d'électrons. 相似文献
325.
326.
Rivalan P Delmas V Angulo E Bull LS Hall RJ Courchamp F Rosser AM Leader-Williams N 《Nature》2007,447(7144):529-530
327.
328.
Psoriasis is one of the most common human skin diseases and is considered to have key genetic underpinnings. It is characterized by excessive growth and aberrant differentiation of keratinocytes, but is fully reversible with appropriate therapy. The trigger of the keratinocyte response is thought to be activation of the cellular immune system, with T cells, dendritic cells and various immune-related cytokines and chemokines implicated in pathogenesis. The newest therapies for psoriasis target its immune components and may predict potential treatments for other inflammatory human diseases. 相似文献
329.
APP locus duplication causes autosomal dominant early-onset Alzheimer disease with cerebral amyloid angiopathy 总被引:24,自引:0,他引:24
Rovelet-Lecrux A Hannequin D Raux G Le Meur N Laquerrière A Vital A Dumanchin C Feuillette S Brice A Vercelletto M Dubas F Frebourg T Campion D 《Nature genetics》2006,38(1):24-26
We report duplication of the APP locus on chromosome 21 in five families with autosomal dominant early-onset Alzheimer disease (ADEOAD) and cerebral amyloid angiopathy (CAA). Among these families, the duplicated segments had a minimal size ranging from 0.58 to 6.37 Mb. Brains from individuals with APP duplication showed abundant parenchymal and vascular deposits of amyloid-beta peptides. Duplication of the APP locus, resulting in accumulation of amyloid-beta peptides, causes ADEOAD with CAA. 相似文献