目的探讨由RAB27A基因缺陷导致的格里塞利综合征2型(Griscelli syndrome type 2,GS2)的临床及免疫学特征。方法收集1家系2例GS2患儿组成的临床资料、生化检查及病理活检结果,采集头发显微镜检查,抽取外周静脉血进行免疫系统基因外显子...目的探讨由RAB27A基因缺陷导致的格里塞利综合征2型(Griscelli syndrome type 2,GS2)的临床及免疫学特征。方法收集1家系2例GS2患儿组成的临床资料、生化检查及病理活检结果,采集头发显微镜检查,抽取外周静脉血进行免疫系统基因外显子阵列测序,桑格测序验证患儿及父母RAB27A基因突变位点,采用蛋白印迹法检测外周血单个核细胞RAB27A蛋白表达水平,采用流式细胞术进行CTL细胞及NK细胞毒性功能检测,健康对照为正常同龄儿童。结果2例患者为亲兄妹,生后均表现毛发色素减退合,均以反复发热、反复呼吸道感染合并噬血细胞性淋巴组织细胞增生症(HLH)为主要临床表现,妹妹伴全身弥漫靶形损害样皮疹。2例患者头发内均有不规则黑色素团块堆积,妹妹皮损组织病理示表皮基底层黑色素细胞不规则分布,RAB27A基因5号外显子发生c.377 delC纯合移码突变,其父母为近亲结婚,均为携带者,2例患儿RAB27A蛋白表达均明显降低,NK细胞及CTL的细胞毒功能均受损。2例患儿均未接受化疗及造血干细胞移植(HSCT),先后因HLH死亡。结论GS2的确诊依赖临床表现及基因检测,免疫功能检测亦有助于诊断,同种异体造血干细胞移植是目前根治GS2的唯一方法。展开更多
The term transnational originated in the historical field when, in the late 1990s, lan Tyrrell wrote a seminal essay entitled ＂What is Transnational History？＂ and changed the course of the academic discipline, clai...The term transnational originated in the historical field when, in the late 1990s, lan Tyrrell wrote a seminal essay entitled ＂What is Transnational History？＂ and changed the course of the academic discipline, claiming that studying the history of a nation from inside its borders was outmoded because the study of history concerns the movements of peoples, ideas, technologies, and institutions across national boundaries. The study of cross-national influences and the focus on the relationship between nation and factors beyond the nation spilled over into many other fields, especially into cinematic studies. Today transnational refers to the impossibility of assigning a fixed national identity to much cinema, to the dissolution of any stable connection between film＇s place of production and the nationality of its makers and performers. Because there is a lot of critical debate about what constitutes national and transnational cinema, the study of international remakes is a promising method to map the field with some accuracy. This essay will analyze the journey from Hammett＇s novel to Kurosawa＇s film and then to Leone＇s western, and will demonstrate how the process of adaptation functions and what happens to a ＂text＂ when it becomes tmnsnational and polysemic. Because Leone is the creator of the Italian western, the one who initiated the cycle that was copied many times over for a decade, we must look at A Fistful of Dollars as a prototype, a movie that when dissected can shed light on the national-transnational dichotomy of the spaghetti western. However, before studying the prototype, we must look at the complex history of the origin of the first spaghetti western, taking into account that A Fistful of Dollars was ＂transcoded＂ by Leone from Kurosawa＇s Yojimbo （1961） that derived his script from Dashiell Hammett＇s RedHarvest （1929）.展开更多
In this paper,the poly(acrylamide)hydrogel used to immobilize saccharomyces cerevisiae for asymmetric synthesis of R(-)-mandelic acid was prepared with free radical ploymerization in deionized water at room temperatur...In this paper,the poly(acrylamide)hydrogel used to immobilize saccharomyces cerevisiae for asymmetric synthesis of R(-)-mandelic acid was prepared with free radical ploymerization in deionized water at room temperature under nitrogen atmosphere.The influence of the composition of hydrogel,loading amount of cells and culture conditions on the asymmetric synthesis was investigated.Results show that PAAm hydrogel is a feasible carrier for immobilization of cells which is a potential alternative method to prepare enantiomerically pure R(-)-mandelic acid.展开更多
The present study was conducted to investigate the heavy metal contents （As, Ba, Cd, Co, Cr, Cu, Ga, Mo, Mn, Rb, Pb, Cu, Ni, V, U and Zn） in seawater samples, which were collected at two c （5 and 100 m）, from 9 di...The present study was conducted to investigate the heavy metal contents （As, Ba, Cd, Co, Cr, Cu, Ga, Mo, Mn, Rb, Pb, Cu, Ni, V, U and Zn） in seawater samples, which were collected at two c （5 and 100 m）, from 9 different sites of Azerbaijan sector of the Caspian Sea. The Agitent 7700x Series ICP-MS （inductively coupled plasma mass spectrometry） with HMI （high matrix introduction） system applied to analysis seawater. It was shown, the average concentrations of elements as Cr, Co, Cu, Mn, Pb and Zn in surface water slightly higher than their concentrations in depth water at 100 m. Practically it is not deference of the Mo, Rb and U average concentrations in surface and 100 m depth column water. There was little variation observed for nickel and vanadium. In contrast the concentrations of As, Ga and Ba in samples taken from 100 m, were significantly higher than the concentration in surface samples. Dependence concentration of elements from water columns depth can be explained in accordance with the geochemical system of classification of dissolved forms of elements in the sea water.展开更多
We propose a novel flow measurement method for gas–liquid two-phase slug flow by using the blind source separation technique. The flow measurement model is established based on the fluctuation characteristics of diff...We propose a novel flow measurement method for gas–liquid two-phase slug flow by using the blind source separation technique. The flow measurement model is established based on the fluctuation characteristics of differential pressure(DP) signals measured from a Venturi meter. It is demonstrated that DP signals of two-phase flow are a linear mixture of DP signals of single phase fluids. The measurement model is a combination of throttle relationship and blind source separation model. In addition, we estimate the mixture matrix using the independent component analysis(ICA) technique. The mixture matrix could be described using the variances of two DP signals acquired from two Venturi meters. The validity of the proposed model was tested in the gas–liquid twophase flow loop facility. Experimental results showed that for most slug flow the relative error is within 10%.We also find that the mixture matrix is beneficial to investigate the flow mechanism of gas–liquid two-phase flow.展开更多
Through carefully reading and studying the emotional crisis of Holden in The catcher in the rye, the main character＇s anxiety, isolation, bewilderment in the crisis of existence and his efforts to identify himself in...Through carefully reading and studying the emotional crisis of Holden in The catcher in the rye, the main character＇s anxiety, isolation, bewilderment in the crisis of existence and his efforts to identify himself in the dilemma are interpreted. His experience exemplifies the plight experienced by the postwar generation and provides a good text from which one can study the American society and culture in 1950s.展开更多
文摘目的探讨由RAB27A基因缺陷导致的格里塞利综合征2型(Griscelli syndrome type 2,GS2)的临床及免疫学特征。方法收集1家系2例GS2患儿组成的临床资料、生化检查及病理活检结果,采集头发显微镜检查,抽取外周静脉血进行免疫系统基因外显子阵列测序,桑格测序验证患儿及父母RAB27A基因突变位点,采用蛋白印迹法检测外周血单个核细胞RAB27A蛋白表达水平,采用流式细胞术进行CTL细胞及NK细胞毒性功能检测,健康对照为正常同龄儿童。结果2例患者为亲兄妹,生后均表现毛发色素减退合,均以反复发热、反复呼吸道感染合并噬血细胞性淋巴组织细胞增生症(HLH)为主要临床表现,妹妹伴全身弥漫靶形损害样皮疹。2例患者头发内均有不规则黑色素团块堆积,妹妹皮损组织病理示表皮基底层黑色素细胞不规则分布,RAB27A基因5号外显子发生c.377 delC纯合移码突变,其父母为近亲结婚,均为携带者,2例患儿RAB27A蛋白表达均明显降低,NK细胞及CTL的细胞毒功能均受损。2例患儿均未接受化疗及造血干细胞移植(HSCT),先后因HLH死亡。结论GS2的确诊依赖临床表现及基因检测,免疫功能检测亦有助于诊断,同种异体造血干细胞移植是目前根治GS2的唯一方法。
文摘The term transnational originated in the historical field when, in the late 1990s, lan Tyrrell wrote a seminal essay entitled ＂What is Transnational History？＂ and changed the course of the academic discipline, claiming that studying the history of a nation from inside its borders was outmoded because the study of history concerns the movements of peoples, ideas, technologies, and institutions across national boundaries. The study of cross-national influences and the focus on the relationship between nation and factors beyond the nation spilled over into many other fields, especially into cinematic studies. Today transnational refers to the impossibility of assigning a fixed national identity to much cinema, to the dissolution of any stable connection between film＇s place of production and the nationality of its makers and performers. Because there is a lot of critical debate about what constitutes national and transnational cinema, the study of international remakes is a promising method to map the field with some accuracy. This essay will analyze the journey from Hammett＇s novel to Kurosawa＇s film and then to Leone＇s western, and will demonstrate how the process of adaptation functions and what happens to a ＂text＂ when it becomes tmnsnational and polysemic. Because Leone is the creator of the Italian western, the one who initiated the cycle that was copied many times over for a decade, we must look at A Fistful of Dollars as a prototype, a movie that when dissected can shed light on the national-transnational dichotomy of the spaghetti western. However, before studying the prototype, we must look at the complex history of the origin of the first spaghetti western, taking into account that A Fistful of Dollars was ＂transcoded＂ by Leone from Kurosawa＇s Yojimbo （1961） that derived his script from Dashiell Hammett＇s RedHarvest （1929）.
文摘In this paper,the poly(acrylamide)hydrogel used to immobilize saccharomyces cerevisiae for asymmetric synthesis of R(-)-mandelic acid was prepared with free radical ploymerization in deionized water at room temperature under nitrogen atmosphere.The influence of the composition of hydrogel,loading amount of cells and culture conditions on the asymmetric synthesis was investigated.Results show that PAAm hydrogel is a feasible carrier for immobilization of cells which is a potential alternative method to prepare enantiomerically pure R(-)-mandelic acid.
文摘The present study was conducted to investigate the heavy metal contents （As, Ba, Cd, Co, Cr, Cu, Ga, Mo, Mn, Rb, Pb, Cu, Ni, V, U and Zn） in seawater samples, which were collected at two c （5 and 100 m）, from 9 different sites of Azerbaijan sector of the Caspian Sea. The Agitent 7700x Series ICP-MS （inductively coupled plasma mass spectrometry） with HMI （high matrix introduction） system applied to analysis seawater. It was shown, the average concentrations of elements as Cr, Co, Cu, Mn, Pb and Zn in surface water slightly higher than their concentrations in depth water at 100 m. Practically it is not deference of the Mo, Rb and U average concentrations in surface and 100 m depth column water. There was little variation observed for nickel and vanadium. In contrast the concentrations of As, Ga and Ba in samples taken from 100 m, were significantly higher than the concentration in surface samples. Dependence concentration of elements from water columns depth can be explained in accordance with the geochemical system of classification of dissolved forms of elements in the sea water.
基金Supported by the National Natural Science Foundation of China(51304231)the Natural Science Foundation of Shandong Province(ZR2010EQ015)
文摘We propose a novel flow measurement method for gas–liquid two-phase slug flow by using the blind source separation technique. The flow measurement model is established based on the fluctuation characteristics of differential pressure(DP) signals measured from a Venturi meter. It is demonstrated that DP signals of two-phase flow are a linear mixture of DP signals of single phase fluids. The measurement model is a combination of throttle relationship and blind source separation model. In addition, we estimate the mixture matrix using the independent component analysis(ICA) technique. The mixture matrix could be described using the variances of two DP signals acquired from two Venturi meters. The validity of the proposed model was tested in the gas–liquid twophase flow loop facility. Experimental results showed that for most slug flow the relative error is within 10%.We also find that the mixture matrix is beneficial to investigate the flow mechanism of gas–liquid two-phase flow.
文摘Through carefully reading and studying the emotional crisis of Holden in The catcher in the rye, the main character＇s anxiety, isolation, bewilderment in the crisis of existence and his efforts to identify himself in the dilemma are interpreted. His experience exemplifies the plight experienced by the postwar generation and provides a good text from which one can study the American society and culture in 1950s.
