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排序方式: 共有4911条查询结果,搜索用时 15 毫秒
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Brücken Erik Gdda Akiko Ott Jennifer Naaranoja Tiin Martikainen Laur Karadzhinova Ferrer Aneliy Kalliokoski Matti Golovlova Mari Kirschenmann Stefanie Litichevskyi Vladyslav Petrine An Luukka Panj Hrknen Jaakko 《湘潭大学自然科学学报》2018,(4):115-120
该文描述了碲化镉(CdTe)探测器,硅漂移探测器和外加一层转换闪烁体硅探测器(SiS)的设计与制作方法这些硅及CdTe芯片制作工艺及其连接方法是在芬兰的Micronova中心完成的与硅工艺不同的是,CdTe工艺必须在低于150 ℃的温度下完成我们因此专门为CdTe探测器开发了一种低温原子层淀积的氧化铝(Al2O3)薄膜工艺,大小为(10×10×1) mm3的CdTe晶圆由一种无接触的光刻工艺完成.探测器性能是由电流 电压(I V),电容 电压(C V)、瞬间电流方法和精确微质子数方法来检测的实验结果与具有材料和缺陷参数的TCAD模拟结果相符 相似文献
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蠕变是决定纤维状生物质压缩的一个重要特性,不同的蠕变特性影响压缩过程的能耗.为分析出最佳原料匹配参数,达到降低压缩能耗的目的,采用Burger模型,分析了在不同含水率和切段处理条件下梯牧草的压缩蠕变特性.非线性回归分析显示,Burger模型适用于梯牧草草捆的压缩蠕变过程;试验结果表明含水率对蠕变特性产生较大影响,切段在... 相似文献
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Harakalova M van Harssel JJ Terhal PA van Lieshout S Duran K Renkens I Amor DJ Wilson LC Kirk EP Turner CL Shears D Garcia-Minaur S Lees MM Ross A Venselaar H Vriend G Takanari H Rook MB van der Heyden MA Asselbergs FW Breur HM Swinkels ME Scurr IJ Smithson SF Knoers NV van der Smagt JJ Nijman IJ Kloosterman WP van Haelst MM van Haaften G Cuppen E 《Nature genetics》2012,44(7):793-796
Cantú syndrome is characterized by congenital hypertrichosis, distinctive facial features, osteochondrodysplasia and cardiac defects. By using family-based exome sequencing, we identified a de novo mutation in ABCC9. Subsequently, we discovered novel dominant missense mutations in ABCC9 in 14 of the 16 individuals with Cantú syndrome examined. The ABCC9 protein is part of an ATP-dependent potassium (K(ATP)) channel that couples the metabolic state of a cell with its electrical activity. All mutations altered amino acids in or close to the transmembrane domains of ABCC9. Using electrophysiological measurements, we show that mutations in ABCC9 reduce the ATP-mediated potassium channel inhibition, resulting in channel opening. Moreover, similarities between the phenotype of individuals with Cantú syndrome and side effects from the K(ATP) channel agonist minoxidil indicate that the mutations in ABCC9 result in channel opening. Given the availability of ABCC9 antagonists, our findings may have direct implications for the treatment of individuals with Cantú syndrome. 相似文献
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Genome-wide association study of flowering time and grain yield traits in a worldwide collection of rice germplasm 总被引:9,自引:0,他引:9
Huang X Zhao Y Wei X Li C Wang A Zhao Q Li W Guo Y Deng L Zhu C Fan D Lu Y Weng Q Liu K Zhou T Jing Y Si L Dong G Huang T Lu T Feng Q Qian Q Li J Han B 《Nature genetics》2012,44(1):32-39
A high-density haplotype map recently enabled a genome-wide association study (GWAS) in a population of indica subspecies of Chinese rice landraces. Here we extend this methodology to a larger and more diverse sample of 950 worldwide rice varieties, including the Oryza sativa indica and Oryza sativa japonica subspecies, to perform an additional GWAS. We identified a total of 32 new loci associated with flowering time and with ten grain-related traits, indicating that the larger sample increased the power to detect trait-associated variants using GWAS. To characterize various alleles and complex genetic variation, we developed an analytical framework for haplotype-based de novo assembly of the low-coverage sequencing data in rice. We identified candidate genes for 18 associated loci through detailed annotation. This study shows that the integrated approach of sequence-based GWAS and functional genome annotation has the potential to match complex traits to their causal polymorphisms in rice. 相似文献
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Loss-of-function mutations in TGFB2 cause a syndromic presentation of thoracic aortic aneurysm 总被引:2,自引:0,他引:2
ME Lindsay D Schepers NA Bolar JJ Doyle E Gallo J Fert-Bober MJ Kempers EK Fishman Y Chen L Myers D Bjeda G Oswald AF Elias HP Levy BM Anderlid MH Yang EM Bongers J Timmermans AC Braverman N Canham GR Mortier HG Brunner PH Byers J Van Eyk L Van Laer HC Dietz BL Loeys 《Nature genetics》2012,44(8):922-927
Loeys-Dietz syndrome (LDS) associates with a tissue signature for high transforming growth factor (TGF)-β signaling but is often caused by heterozygous mutations in genes encoding positive effectors of TGF-β signaling, including either subunit of the TGF-β receptor or SMAD3, thereby engendering controversy regarding the mechanism of disease. Here, we report heterozygous mutations or deletions in the gene encoding the TGF-β2 ligand for a phenotype within the LDS spectrum and show upregulation of TGF-β signaling in aortic tissue from affected individuals. Furthermore, haploinsufficient Tgfb2(+/-) mice have aortic root aneurysm and biochemical evidence of increased canonical and noncanonical TGF-β signaling. Mice that harbor both a mutant Marfan syndrome (MFS) allele (Fbn1(C1039G/+)) and Tgfb2 haploinsufficiency show increased TGF-β signaling and phenotypic worsening in association with normalization of TGF-β2 expression and high expression of TGF-β1. Taken together, these data support the hypothesis that compensatory autocrine and/or paracrine events contribute to the pathogenesis of TGF-β-mediated vasculopathies. 相似文献
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Zollino M Orteschi D Murdolo M Lattante S Battaglia D Stefanini C Mercuri E Chiurazzi P Neri G Marangi G 《Nature genetics》2012,44(6):636-638
The chromosome 17q21.31 deletion syndrome is a genomic disorder characterized by highly distinctive facial features, moderate-to-severe intellectual disability, hypotonia and friendly behavior. Here, we show that de novo loss-of-function mutations in KANSL1 (also called KIAA1267) cause a full del(17q21.31) phenotype in two unrelated individuals that lack deletion at 17q21.31. These findings indicate that 17q21.31 deletion syndrome is a monogenic disorder caused by haploinsufficiency of KANSL1. 相似文献
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Lifestyle transitions in plant pathogenic Colletotrichum fungi deciphered by genome and transcriptome analyses 总被引:8,自引:0,他引:8
RJ O'Connell MR Thon S Hacquard SG Amyotte J Kleemann MF Torres U Damm EA Buiate L Epstein N Alkan J Altmüller L Alvarado-Balderrama CA Bauser C Becker BW Birren Z Chen J Choi JA Crouch JP Duvick MA Farman P Gan D Heiman B Henrissat RJ Howard M Kabbage C Koch B Kracher Y Kubo AD Law MH Lebrun YH Lee I Miyara N Moore U Neumann K Nordström DG Panaccione R Panstruga M Place RH Proctor D Prusky G Rech R Reinhardt JA Rollins S Rounsley CL Schardl DC Schwartz N Shenoy K Shirasu UR Sikhakolli K Stüber 《Nature genetics》2012,44(9):1060-1065
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