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排序方式: 共有80条查询结果,搜索用时 15 毫秒
11.
Letter: T cell-dependent mediator in the immune response 总被引:11,自引:0,他引:11
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Ross AJ May-Simera H Eichers ER Kai M Hill J Jagger DJ Leitch CC Chapple JP Munro PM Fisher S Tan PL Phillips HM Leroux MR Henderson DJ Murdoch JN Copp AJ Eliot MM Lupski JR Kemp DT Dollfus H Tada M Katsanis N Forge A Beales PL 《Nature genetics》2005,37(10):1135-1140
The evolutionarily conserved planar cell polarity (PCP) pathway (or noncanonical Wnt pathway) drives several important cellular processes, including epithelial cell polarization, cell migration and mitotic spindle orientation. In vertebrates, PCP genes have a vital role in polarized convergent extension movements during gastrulation and neurulation. Here we show that mice with mutations in genes involved in Bardet-Biedl syndrome (BBS), a disorder associated with ciliary dysfunction, share phenotypes with PCP mutants including open eyelids, neural tube defects and disrupted cochlear stereociliary bundles. Furthermore, we identify genetic interactions between BBS genes and a PCP gene in both mouse (Ltap, also called Vangl2) and zebrafish (vangl2). In zebrafish, the augmented phenotype results from enhanced defective convergent extension movements. We also show that Vangl2 localizes to the basal body and axoneme of ciliated cells, a pattern reminiscent of that of the BBS proteins. These data suggest that cilia are intrinsically involved in PCP processes. 相似文献
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Eukaryotic cells have systems of internal organelles to synthesize lipids and membrane proteins, to release secreted proteins, to take up nutrients and to degrade membrane-bound and internalized molecules. Proteins and lipids move from organelle to organelle using transport vesicles. The accuracy of this traffic depends upon organelles being correctly recognized. In general, organelles are identified by the activated GTPases and specific lipid species that they display. These short-lived determinants provide organelles with an identity that is both unique and flexible. Recent studies have helped to establish how cells maintain and restrict these determinants and explain how this system is exploited by invading pathogens. 相似文献
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多孔泡沫材料的声吸收特性 总被引:5,自引:4,他引:5
为了结合泡沫金属兼有的高吸声和高热传导两种特性以进一步提高其吸声性能,回顾了泡沫金属材料的应用和声学建模;通过对泡沫金属和用于制造泡沫金属的高分子基体材料的实验,比较了作者提出的声波通过泡沫金属传播的3种黏滞模型的预测结果,表明所有模型在泡沫金属典型胞元尺寸所对应的低雷诺数范围内是有效的(假定声波为线性,幅值低于160 dB).第一种模型考虑了声波沿平行于刚性圆柱束轴线方向传播时所受到的空气曳力,第二种模型考虑了声波沿垂直于刚性圆柱束轴线方向的传播,第三种模型考虑了声波通过球形节点的传播.结合这3种模型,提出了一种泡沫金属声学性能的综合模型,可以用来预测泡沫金属的声吸收特性.此外,还介绍了一种用于泡沫金属材料基本声传播特性实验的后处理技术. 相似文献
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Walther TC Askjaer P Gentzel M Habermann A Griffiths G Wilm M Mattaj IW Hetzer M 《Nature》2003,424(6949):689-694
In metazoa, the nuclear envelope breaks down and reforms during each cell cycle. Nuclear pore complexes (NPCs), which serve as channels for transport between the nucleus and cytoplasm, assemble into the reforming nuclear envelope in a sequential process involving association of a subset of NPC proteins, nucleoporins, with chromatin followed by the formation of a closed nuclear envelope fenestrated by NPCs. How chromatin recruitment of nucleoporins and NPC assembly are regulated is unknown. Here we demonstrate that RanGTP production is required to dissociate nucleoporins Nup107, Nup153 and Nup358 from Importin beta, to target them to chromatin and to induce association between separate NPC subcomplexes. Additionally, either an excess of RanGTP or removal of Importin beta induces formation of NPC-containing membrane structures--annulate lamellae--both in vitro in the absence of chromatin and in vivo. Annulate lamellae formation is strongly and specifically inhibited by an excess of Importin beta. The data demonstrate that RanGTP triggers distinct steps of NPC assembly, and suggest a mechanism for the spatial restriction of NPC assembly to the surface of chromatin. 相似文献
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OPCML at 11q25 is epigenetically inactivated and has tumor-suppressor function in epithelial ovarian cancer 总被引:12,自引:0,他引:12
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Mutations in INVS encoding inversin cause nephronophthisis type 2, linking renal cystic disease to the function of primary cilia and left-right axis determination 总被引:20,自引:0,他引:20
Otto EA Schermer B Obara T O'Toole JF Hiller KS Mueller AM Ruf RG Hoefele J Beekmann F Landau D Foreman JW Goodship JA Strachan T Kispert A Wolf MT Gagnadoux MF Nivet H Antignac C Walz G Drummond IA Benzing T Hildebrandt F 《Nature genetics》2003,34(4):413-420
Nephronophthisis (NPHP), an autosomal recessive cystic kidney disease, leads to chronic renal failure in children. The genes mutated in NPHP1 and NPHP4 have been identified, and a gene locus associated with infantile nephronophthisis (NPHP2) was mapped. The kidney phenotype of NPHP2 combines clinical features of NPHP and polycystic kidney disease (PKD). Here, we identify inversin (INVS) as the gene mutated in NPHP2 with and without situs inversus. We show molecular interaction of inversin with nephrocystin, the product of the gene mutated in NPHP1 and interaction of nephrocystin with beta-tubulin, a main component of primary cilia. We show that nephrocystin, inversin and beta-tubulin colocalize to primary cilia of renal tubular cells. Furthermore, we produce a PKD-like renal cystic phenotype and randomization of heart looping by knockdown of invs expression in zebrafish. The interaction and colocalization in cilia of inversin, nephrocystin and beta-tubulin connect pathogenetic aspects of NPHP to PKD, to primary cilia function and to left-right axis determination. 相似文献