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Study on In vitro Bacteriostasis of the Different Extract from Ilex Kudingcha C.J.Tseng 被引量:1
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作者 吕平 黄强 《Agricultural Science & Technology》 CAS 2009年第6期125-127,共3页
[ Objective ] The aim was to study bacteriostatic activity of the different extract from Ilex Kudingcha C. J. Tseng. [ Method ] By using test tube 2 -fold dilution method and Kirby-Baueer Disc Diffusionto,we conducted... [ Objective ] The aim was to study bacteriostatic activity of the different extract from Ilex Kudingcha C. J. Tseng. [ Method ] By using test tube 2 -fold dilution method and Kirby-Baueer Disc Diffusionto,we conducted inhibitory test on S. aureus and E, coil and determined the minimal inhibitory concentration (MIC) and minimum bactericidal concentration (MBC)and the diameter of inhibition zone. [ Result ] The extract 1 had fairly strong in vitro bacteriostasis activities and than the extract2 and extract3. The MIC and MBC was 3.91 mg/ml and 31.25 mg/ml on S. aureus and E. coil [ Conclusion] The result showed that the different extract from Ilex Kudingcha C. J. Tseng had varying amount of different bacteriostatic activities,the Ilex Kudingcha C. J. Tseng was developed new and safe bacteriostatic reagent to provide reference. 展开更多
关键词 Ilex Kudingcha c. J. Tseng: Extract In vitro bacteriostasis
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An interpretation of J. C. Catford's A linguistic theory of translation 被引量:2
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作者 陈顺意 《Sino-US English Teaching》 2009年第7期36-41,共6页
J. C. Catford is a world-renowned British linguist and translation theorist, and also an outstanding representative of the linguistic school of translation. His famous work A linguistic theory of translation was first... J. C. Catford is a world-renowned British linguist and translation theorist, and also an outstanding representative of the linguistic school of translation. His famous work A linguistic theory of translation was first published by Oxford University Press in 1965, and republished in 1967. It was among the list of the series of language and linguistic studies of Oxford University. This essay attempts to interpret this great book in five aspects, namely, definition of translation, types of translation, translation equivalence, transference, and limits of translatability, and tries to probe into the positive impact of its theories on the development of translation theory and its limitations. 展开更多
关键词 J. c. catford A linguistic theory of translation TRANSLATION LINGUISTIcS
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采用熔解曲线法进行RHD c.1227G>A基因检测研究
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作者 马玲 刘太香 薛敏 《南京医科大学学报(自然科学版)》 CAS CSCD 北大核心 2021年第10期1542-1545,共4页
目的:建立用于RHD c.1227G>A基因检测的熔解曲线分析方法。方法:根据RHD c.1227G>A基因位点设计并合成序列特异性引物,在荧光定量PCR反应后对产物进行熔解曲线分析,根据熔解曲线峰值的差异进行基因检测结果判断,并与常规序列特异... 目的:建立用于RHD c.1227G>A基因检测的熔解曲线分析方法。方法:根据RHD c.1227G>A基因位点设计并合成序列特异性引物,在荧光定量PCR反应后对产物进行熔解曲线分析,根据熔解曲线峰值的差异进行基因检测结果判断,并与常规序列特异性引物PCR(PCR-sequence specific primer,PCR-SSP)RHD c.1227G>A基因分型方法进行比较;对与血清学结果不一致的标本进行测序分析。结果:在87例血清学初筛为RhD阴性表型的标本中,采用熔解曲线分析法,检测出16例含有RHD c.1227G>A,占比18.4%;与常规PCR-SSP分型结果一致。对1例与血清学吸收放散结果不一致的标本进行RHD exon 9测序分析,证实均为1227A纯合子。熔解曲线分析法的灵敏度、特异度、阳性预测值、阴性预测值和总符合率均为100%,约登指数为1.0。结论:成功建立用于红细胞RHD c.1227G>A基因分型的熔解曲线分析方法,可以高效、快速地进行RHD c.1227G>A基因检测研究。 展开更多
关键词 熔解曲线 RHD基因 RHD c.1227G>A
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Interspecific Transition Among Caragana microphylla, C. davazamcii and C. korshinskii Along Geographic Gradient. Ⅱ. Characteristics of Photosynthesis and Water Metabolism 被引量:13
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作者 马成仓 高玉葆 +1 位作者 郭宏宇 王金龙 《Acta Botanica Sinica》 CSCD 2003年第10期1228-1237,共10页
The characteristics of photosynthesis and water metabolism of Caragana microphylla Lam.,C. davazamcii Sancz. and C. korshinskii Kom. populations in different sites (117.6o-105.7o E, 44.6o-38.8o N)were studied. (1) Fro... The characteristics of photosynthesis and water metabolism of Caragana microphylla Lam.,C. davazamcii Sancz. and C. korshinskii Kom. populations in different sites (117.6o-105.7o E, 44.6o-38.8o N)were studied. (1) From the east to the west, the responses of the three species to photosyntheticallyavailable radiation (PAR) in net photosynthesis rate increased, the relative humidity of the air whichcorresponded to the occurrence of maximum photosynthesis rate decreased, and the corresponding airtemperature increased. Along the same gradient, the before-noon superiority of the photosynthesis be-came evident, and the photosynthesis rate and the light use efficiency (LUE ) increased, while the transp-iration rate decreased, thus the water use efficiency (WUE ) increased notably, and the leaf water contentdecreased gradually. From the east to the west, the plants took a water-saving strategy step by step withhigher photosynthesis rate and lower transpiration rate. These physiological changes in the plants wereadaptable to the conditions of light, temperature and humidity in the habitat of the plants, and might be thebiological foundation for the geographical transition among C. microphylla , C. davazamcii and C. korshinskii.(2) The adaptation of photosynthetic system of C. microphylla , C. davazamcii and C. korshinskii to PAR, airhumidity and temperature exhibited the interspecific continuity, which was consistent with theenvironmental gradient. In different species and different sites, the diurnal changes of net photosynthesisrate, the daily cumulative value of net photosynthesis, the diurnal changes of transpiration rate, the dailycumulative value of transpiration, the water use efficiency and the diurnal changes of leaf water contentvaried with longitudinal descent (from the east to the west). The characteristics of photosynthesis andwater metabolism indicated that the geographical transition among C. microphylla , C. davazamcii and C.korshinskii was in gradual change, and these three species formed a geographical cline. 展开更多
关键词 caragana microphylla c. davazamcii c. korshinskii geographical transition PHOTOSYNTHESIS water metabolism
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Interspecific Transition Among Caragana microphylla, C. davazamcii and C. korshinskii Along Geographic Gradient. Ⅰ. Ecological and RAPD Evidence 被引量:12
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作者 马成仓 高玉葆 +2 位作者 刘惠芬 王金龙 郭宏宇 《Acta Botanica Sinica》 CSCD 2003年第10期1218-1227,共10页
The former plant population survey has shown that three genetically-related species, Caraganamicrophylla Lam., C. davazamcii Sancz. and C. korshinskii Kom., form a geographical replacement series inNei Mongol Plateau.... The former plant population survey has shown that three genetically-related species, Caraganamicrophylla Lam., C. davazamcii Sancz. and C. korshinskii Kom., form a geographical replacement series inNei Mongol Plateau. The present study on population distribution, taxonomy, morphology, development andgenetic structure demonstrated that the geographical distribution of these three species was successiveand in gradual change, thus forming a geographical cline which extended from the east to the west of NeiMongol Plateau. With an analysis of climate change over time, it was considered that the formation of thisgeographical cline was a result of plant adaptation to its natural environment. 展开更多
关键词 caragana microphylla c. davazamcii c. korshinskii interspecific transition alonggeographical gradient Morphology TAXONOMY RAPD
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Charles C.Fries的语言学思想对系统功能语言学的启示
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作者 陈文革 《鸡西大学学报(综合版)》 2012年第5期126-128,共3页
Charles C.Fries与布龙菲尔德、萨皮尔一起被认为是结构主义语言学的代表人物之一。Fries虽处在结构主义语言学鼎盛时期,但他的研究与同时期语言学家大不一样。对Fries的语言观和语言研究方法进行审视发现,Charles C.Fries的语言学理论... Charles C.Fries与布龙菲尔德、萨皮尔一起被认为是结构主义语言学的代表人物之一。Fries虽处在结构主义语言学鼎盛时期,但他的研究与同时期语言学家大不一样。对Fries的语言观和语言研究方法进行审视发现,Charles C.Fries的语言学理论与系统功能语言学有许多相似之处,比如他强调口语语料和对比性特征即纵聚合在语言分析中的第一性,他也关心语言的语境和语域。应重新评价Charles C.Fries和其他结构语言学家的语言学贡献。 展开更多
关键词 charles c.Fries 系统功能语言学 语境
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Ginsenoside-Rg_6, a Novel Triterpenoid Saponin from the Stem-Leaves of Panax ginseng C. A. Mey. 被引量:12
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作者 Xiu Wei YANG, Long Yun LI, Jian Ming TIAN, Zhi Wei ZHANG, Jin Mei YE, Wei Fang Gu (National Research Laboratory of Natural & Biomimetic Drugs, Peking University, Beijing 100083) (Academy of Chinese Traditional Medicine and Materia Medica of Jilin Provinc 《Chinese Chemical Letters》 SCIE CAS CSCD 2000年第10期909-912,共4页
A novel dammarane-type triterpene oligoglycoside, named ginsenoside-Rg6 3, was isolated from the stem-leaves of Panax ginseng C. A. Mey., together with two known ones, 20(S)-ginsenoside-Rg2 1 and 20(R)-ginsenoside-Rg2... A novel dammarane-type triterpene oligoglycoside, named ginsenoside-Rg6 3, was isolated from the stem-leaves of Panax ginseng C. A. Mey., together with two known ones, 20(S)-ginsenoside-Rg2 1 and 20(R)-ginsenoside-Rg2 2. On the basis of chemical and physicochemical evidence , the structure of ginsenoside-Rg6 have been elucidated as 6-O-(-L-rhamnosyl-(1?2)-(-D-glucopyranosyl-dammarane-(E)-20(22), 24-diene-3(, 6(, 12(-triol. 展开更多
关键词 Panax ginseng c. A. Mey. stem-leaves TRITERPENOID ginsenoside-Rg_6.
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法学教育视野下的Willem C.Vis模拟国际商事仲裁辩论赛 被引量:4
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作者 何其生 范晓亮 《时代法学》 2012年第4期112-120,共9页
随着全球经济一体化的深入发展,国际民商事纠纷也迅速增多,国际商事仲裁作为一种争议解决方式越来越受重视,这就需要既精通国际法律知识,又具备较强英语能力的专业人才。而作为全球知名的国际法专业赛事,Willem C.Vis模拟国际商事仲裁... 随着全球经济一体化的深入发展,国际民商事纠纷也迅速增多,国际商事仲裁作为一种争议解决方式越来越受重视,这就需要既精通国际法律知识,又具备较强英语能力的专业人才。而作为全球知名的国际法专业赛事,Willem C.Vis模拟国际商事仲裁辩论赛目的在于通过模拟国际商事仲裁的程序,推广国际商事仲裁和《联合国国际货物销售合同公约》的适用。该赛事有利于学生们通过模拟实践来加深对国际商事仲裁、国际商法等知识的理解,以及对于庭辩技巧、英文法律文书写作等综合技能的掌握,有利于培养具有国际竞争力的法律人才,具有良好的法学教育功能。 展开更多
关键词 模拟国际商事仲裁比赛 Willem c.Vis 法学教育 国际法
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Charles C.Fries语言理论中功能元素和认知元素解读
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作者 邱艳芳 《邢台学院学报》 2021年第2期138-142,共5页
Charles C.Fries是20世纪美国结构主义语言学派代表人物之一,但与同时期其他结构语言学家不同,他的语言思想在不同程度上折射出了系统功能语言学及认知语言学的部分理论元素。对Fries的语言使用观、意义观和语境观进行解读,分析该语言... Charles C.Fries是20世纪美国结构主义语言学派代表人物之一,但与同时期其他结构语言学家不同,他的语言思想在不同程度上折射出了系统功能语言学及认知语言学的部分理论元素。对Fries的语言使用观、意义观和语境观进行解读,分析该语言理论中所体现的功能元素和认知元素,有助于对其理论作出更为合理的认识和评价。 展开更多
关键词 charles c.Fries 基于使用的语言观 意义观 语境观 认知元素
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论Charles C.Fries的语料库语言学思想及方法 被引量:1
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作者 樊斌 韩存新 《集美大学学报(哲学社会科学版)》 2014年第2期117-120,共4页
Charles C.Fries是被语料库语言学界遗忘的一位重量级人物。他既是美国结构主义的代表人物,同时也是一位具有超前意识的语料库语言学思想家和杰出实践者。他的一些语料库语言学思想及方法至今对语料库语言学仍具有指导意义。对其主要的... Charles C.Fries是被语料库语言学界遗忘的一位重量级人物。他既是美国结构主义的代表人物,同时也是一位具有超前意识的语料库语言学思想家和杰出实践者。他的一些语料库语言学思想及方法至今对语料库语言学仍具有指导意义。对其主要的语料库语言学思想及方法:真实客观的数据观、将语言对比特征作为研究的焦点以及实证的研究范式,进行了评述,并在最后指出其思想和方法上的一些缺陷。 展开更多
关键词 chades c FRIES 语料库语言学 思想 方法
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Charles C.Fries与语篇分析 被引量:1
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作者 陈文革 《重庆理工大学学报(社会科学)》 CAS 2012年第9期73-77,84,共6页
Charles C.Fries的贡献不仅在语言教育上,他的语言学理论在当时也独树一帜。讨论并分析了Fries的相关语篇分析思想。研究发现,Fries的语境观与Malinowski和同时期J.R.Firth的语境观大同小异。Fries的序列信号显示了他对话语衔接和连贯... Charles C.Fries的贡献不仅在语言教育上,他的语言学理论在当时也独树一帜。讨论并分析了Fries的相关语篇分析思想。研究发现,Fries的语境观与Malinowski和同时期J.R.Firth的语境观大同小异。Fries的序列信号显示了他对话语衔接和连贯的看法,他的这些语篇分析思想对后来的超句子研究有着一定积极影响。有必要重新审视和评价Charles C.Fries对结构语言学的贡献。 展开更多
关键词 charles c.Fries 语篇分析 语境
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A new triterpene saponin from Panax japonicus C.A.Meyer var major(Burk.) C.Y.Wu et K.M.Feng 被引量:4
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作者 Hong Zhao Lin Shi +3 位作者 Jia Qing Cao Wei Li Xia Wen Yu Qing Zhao 《Chinese Chemical Letters》 SCIE CAS CSCD 2010年第10期1216-1218,共3页
One new Iriterpene saponin was isolated from Panaxjaponicus C. A. Meyer var major (Burk.) C. Y. Wu et K. M. Feng, and established as oleanolic acid 3-O-[β-D-glucopyranosyl-(1 →2)-β-D-glucuronopyranosyl-6'-O-n-... One new Iriterpene saponin was isolated from Panaxjaponicus C. A. Meyer var major (Burk.) C. Y. Wu et K. M. Feng, and established as oleanolic acid 3-O-[β-D-glucopyranosyl-(1 →2)-β-D-glucuronopyranosyl-6'-O-n-butyl ester] which showed mod- erate antitumor activities against the A2780 cells and OVCAR-3 cells. Its structure was established by means of spectral data, particularly NMR, including HSQC and HMBC techniques. 展开更多
关键词 Oleanolic acid 3-O-[β-D-glucopyranosyl-(1 2)-β-D-glucuronopyranosyl-6'-O-n-butyl ester] Antitumor Panax japonicus c. A.Meyer vat major (Burk) c Y wu et K. M Feng Triterpene saponin
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Computational analysis of genetic loci required for amphid structure and functions and their possibly corresponding microRNAs in C. elegans 被引量:1
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作者 胡亚欧 孙阳 +1 位作者 叶波平 王大勇 《Neuroscience Bulletin》 SCIE CAS CSCD 2007年第1期9-20,共12页
Objective To examine the important roles of microRNAs (miRNAs) in regulating amphid structure and function, we performed a computational analysis for the genetic loci required for the sensory perception and their po... Objective To examine the important roles of microRNAs (miRNAs) in regulating amphid structure and function, we performed a computational analysis for the genetic loci required for the sensory perception and their possibly corresponding miRNAs in C. elegans. Methods Total 55 genetic loci required for the amphid structure and function were selected. Sequence alignment was combined with E value evaluation to investigate and identify the possible corresponding miRNAs. Results Total 30 genes among the 55 genetic loci selected have their possible corresponding regulatory miRNA(s), and identified genes participate in the regulation of almost all aspects of amphid structure and function. In addition, our data suggest that both the amphid structure and the amphid functions might be regulated by a series of network signaling pathways. Moreover, the distribution of miRNAs along the 3' untranslated region (UTR) of these 30 genes exhibits different patterns. Conclusion We present the possible miRNA-mediated signaling pathways involved in the regulation of chemosensation and thermosensation by controlling the corresponding sensory neuron and interneuron functions. Our work will be useful for better understanding of the miRNA-mediated control of the chemotaxis and thermotaxis in C. elegans. 展开更多
关键词 microRNA cilia structure sensory neuron INTERNEURON computational analysis c. elegans
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Charles C.Fries的意义理论及对外语教学的启示 被引量:1
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作者 韩存新 《浙江外国语学院学报》 2013年第5期38-43,共6页
意义理论是Charles C.Fries语言学理论的重要组成部分。Fries认为,语言的意义可以按照其执行的社会功能来分类。任何言语信号的"意义"包含的不仅是引起发生特定语音的实际情况,而且是这些特定语音(通过语言)在另一个人身上产... 意义理论是Charles C.Fries语言学理论的重要组成部分。Fries认为,语言的意义可以按照其执行的社会功能来分类。任何言语信号的"意义"包含的不仅是引起发生特定语音的实际情况,而且是这些特定语音(通过语言)在另一个人身上产生的实际反应。语言的全部意义体现在词汇意义、语法结构意义和社会文化意义这三个层次上。Fries的意义理论对外语教学的启示是:重视对目的语和源语社会文化的学习;同时重视对"语境中的词语",特别是词语搭配的教学。 展开更多
关键词 charles c FRIES 意义理论 外语教学 启示
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FADS2基因 c.1571 A>G 突变对中国荷斯坦牛泌乳性状的影响
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作者 王梦琦 倪炜 +6 位作者 唐程 郭佳禾 张强 梁艳 张慧敏 李明勋 毛永江 《安徽农业科学》 CAS 2019年第10期92-93,115,共3页
[目的]探索FADS2基因c.1571A>G多态性对中国荷斯坦牛泌乳性状的影响。[方法]对866头中国荷斯坦牛FADS2基因c.1571A>G位点进行多态性分析,采用最小二乘法分析其对泌乳性状的影响。[结果]FADS2c.1571A>G对日产奶量、乳蛋白率、... [目的]探索FADS2基因c.1571A>G多态性对中国荷斯坦牛泌乳性状的影响。[方法]对866头中国荷斯坦牛FADS2基因c.1571A>G位点进行多态性分析,采用最小二乘法分析其对泌乳性状的影响。[结果]FADS2c.1571A>G对日产奶量、乳蛋白率、乳脂率、总固形物以及尿素氮含量等泌乳性状有显著影响(P<0.05)。GG基因型个体具有较高的日产奶量,而AA型个体的乳脂率、乳蛋白率及总固形物含量显著高于其他基因型个体。FADS2c.1571A>G突变对体细胞评分(SCS)无显著影响(P>0.05)。[结论]FADS2可作为优化中国荷斯坦牛泌乳性能的潜在分子标记。 展开更多
关键词 FADS2 c.1571A>G 体细胞评分 泌乳性状
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磁化率在 C.G.S单位制和 SI单位制之间的单位转换及公式 被引量:6
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作者 王春凤 田英 《哈尔滨师范大学自然科学学报》 CAS 2000年第1期61-64,共4页
本文讨论了在络合物磁化率测定实验中 ,有关单位换算出现的问题 ,给出了磁化率单位在 C.G.S单位制和 SI单位制间的转换关系 ,以及
关键词 磁化率 c.G.S单位制 SI单位制 单位转换
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Study on Segregation of Fruit Characters and Primary Selection of Excellent Single Plants from the Hybrid Population of Corylus heterophylla×C. heterophylla × C. avellana
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作者 王蕾 周兰 +2 位作者 曹后男 张楠 朴永振 《Agricultural Science & Technology》 CAS 2017年第7期1179-1182,共4页
In order to obtain hazelnut lines with the characteristics of strong cold re- sistance, large fruit and high quality capable of overwintering safely in Yanbian ar- eas, with 173 lines of Corylus heterophylla × C.... In order to obtain hazelnut lines with the characteristics of strong cold re- sistance, large fruit and high quality capable of overwintering safely in Yanbian ar- eas, with 173 lines of Corylus heterophylla × C. heterophylla ×C. avellana hazelnut as materials, fruit-related characters were measured and analyzed. Eight excellent individuals were preliminarily selected for further identification of cold resistance and high yield. They selected single plants were 57-1, 57-3 and 55-24 of combination 6- 34-1×S1-5, 56-3, 56-5 and 55-19 of combination 6-34-1×S3-1, 21-12 of combination 6-37-2×S3-1 and 21-12 of combination 6-23-1×S3-1. 展开更多
关键词 c. heterophylla×c heterophylla×c .avellana Nut characters Good sin-gle plants
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外语教师发展的有效策略——评介Jack C.Richards所著Professional Development for Language Teachers:Strategies for Teacher Learning
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作者 陈桂松 薛文 《柳州职业技术学院学报》 2018年第3期48-53,共6页
J.C.Richards教授在英语语言教学和教师培训领域的实践经历与理论研究,对其本人关于"语言教师发展"思想体系的构建起到了积极的促进作用。他于2005年出版的Professional Development for Language Teachers:Strategies for Te... J.C.Richards教授在英语语言教学和教师培训领域的实践经历与理论研究,对其本人关于"语言教师发展"思想体系的构建起到了积极的促进作用。他于2005年出版的Professional Development for Language Teachers:Strategies for Teacher Learning(《语言教师专业发展:教师学习策略》)一书论述了"教师教育"的本质,分析了关于"教师发展"的六个重要观点,尤其是对语言教师发展策略进行系统性阐述,以章节形式对十一种发展策略进行全面细致的介绍,并附有具体事例、教师反思、参考书目等。该书是作者对英语语言教师发展领域的理论研究及实践经验的总结,其内容及框架在注重理论知识的科学性和系统性的同时,也突出了实践操作的必要性和可行性,可以作为我国外语教师开展职业发展、提升职业素质的有益参考。 展开更多
关键词 J.c.Ric.ards 外语教师发展 书评
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Determination of Saponins in Leaf of Panax Ginseng C. A. Mey. by High Performance Liquid Chromatography
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作者 LIU Gui-ying ZHOU Hong-yu +6 位作者 LU Juan ZHU Na GUI Ming-yu JIN Yong-ri ZHANG Yi-hua WANG Xin LI Xu-wen 《Chemical Research in Chinese Universities》 SCIE CAS CSCD 2009年第3期297-301,共5页
A high performance liquid chromatography(HPLC) with UV detection was established for simultaneous determination of saponins in the leaf of Panax ginseng C. A. Mey. Nine ginsenosides(Rbl, Rb2, Rb3, Rc, Rd, F1, F2, F... A high performance liquid chromatography(HPLC) with UV detection was established for simultaneous determination of saponins in the leaf of Panax ginseng C. A. Mey. Nine ginsenosides(Rbl, Rb2, Rb3, Rc, Rd, F1, F2, F3, F5) and notoginsenoside Fe(NFe) were studied. Among the saponins, the ginsenosides F1, F2, F3, F5 and NFe were determined by HPLC-UV method for the first time. The determination of the ginsenosides via the HPLC-UV method was performed on a reversed-phase C18 column with gradient elution in 40 min. The linearity, precision, accuracy, and detection limit for determining the saponins were studied and the samples from different areas in China were analyzed. The HPLC-ESI-MS was used to identify the saponins. The results indicate that the HPLC-UV provided a good accuracy, reproducibility and sensitivity for the determination of the ten saponins. 展开更多
关键词 Panax ginseng c. A. Mey. SAPONINS HPLc Quantification DETERMINATION
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Enhanced but hypofunctional osteoclastogenesis in an autosomal dominant osteopetrosis type II case carrying a c.1856C>T mutation in CLCN7 被引量:1
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作者 Xiang Chen Kun Zhang +2 位作者 Janet Hock Chunyu Wang Xijie Yu 《Bone Research》 SCIE CAS CSCD 2016年第4期232-240,共9页
Type II autosomal dominant osteopetrosis(ADO2), which is the most common form of osteopetrosis, is caused by heterozygous mutations in the chloride channel 7(CLCN7) gene. The osteopetrosis of ADO2 has been attributed ... Type II autosomal dominant osteopetrosis(ADO2), which is the most common form of osteopetrosis, is caused by heterozygous mutations in the chloride channel 7(CLCN7) gene. The osteopetrosis of ADO2 has been attributed to hypofunctional osteoclasts. The mechanism underlying the abnormality in osteoclast function remains largely unknown. This study was designed to investigate gene mutations and osteoclast function in a case that was clinically diagnosed as ADO2. Genomic DNA was extracted from blood samples of this patient, and the 25 exons of CLCN7 were amplified. Peripheral blood from the ADO2 subject and a healthy age- and sex-matched control was used to evaluate osteoclastogenesis, osteoclast morphology, and bone resorption. Analysis of DNA from the patient showed a germline heterozygous missense mutation,c.1856C>T(p.P619L), in exon 20 of CLCN7. A similar homozygous mutation at this site was previously reported in a patient with autosomal recessive osteopetrosis. When cultured, the peripheral blood mononuclear cells(PBMCs) from the ADO2 patient spontaneously differentiated into mature osteoclasts in vitro. The ADO2 patient’s PBMCs formed enhanced, but heterogeneous, osteoclasts in both the presence and absence of macrophage-colony stimulating factor, and nuclear factor-?B ligand. Bone resorption was reduced in the ADO2 patient’s osteoclasts, which exhibited aberrant morphology and abnormal distribution of integrin avβ3. Gene analysis found increased c-fos expression and reduced Rho A and integrin beta 3expression in ADO2 cells. In conclusion, our data suggest that enhanced, heterogeneous osteoclast induction may be an intrinsic characteristic of ADO2. 展开更多
关键词 ADO T mutation in cLcN7 Enhanced but hypofunctional osteoclastogenesis in an autosomal dominant osteopetrosis type II case carrying a c.1856c cASE type II
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