目的采用基因芯片技术筛查精神发育迟滞伴小阴茎男童的基因组微失衡,探讨Y染色体无精子症因子C区(AZFc)部分缺失对其的致病性。方法提取1例精神发育迟滞伴小阴茎男童(先证者)及其父亲外周血DNA,利用Cyto One Array染色体芯片行全基因组...目的采用基因芯片技术筛查精神发育迟滞伴小阴茎男童的基因组微失衡,探讨Y染色体无精子症因子C区(AZFc)部分缺失对其的致病性。方法提取1例精神发育迟滞伴小阴茎男童(先证者)及其父亲外周血DNA,利用Cyto One Array染色体芯片行全基因组微失衡初筛,之后采用定制4×180K SNP+CNV精神发育相关芯片行全基因组微失衡筛查。选择特异性STS标签s Y1191,s Y1291作为引物,利用PCR特异性扩增11例正常男童,9/20例4×180K SNP+CNV芯片检测异常的精神发育迟滞未伴小阴茎男童,先证者及其父亲的Y染色体AZFc区域。进一步对170例精神发育迟滞未伴小阴茎男童行PCR扩增,分析AZFc区域缺失频率。结果 1Cyto One Array染色体芯片检测发现先证者存在AZFc区域全缺失(chr Y:24646690-28103711),定制4×180K SNP+CNV精神发育相关芯片排除其他精神发育相关基因组微失衡,发现AZFc区域部分缺失(chr Y:24873066-25203027;chr Y:25850255-26245019),结果不同于Cyto One Array染色体芯片。220例精神发育迟滞伴小阴茎男童行4×180K CNV+SNP芯片检测,5例存在Y染色体AZFc区域部分缺失,但缺失起止点不同;4例为已知的致精神发育异常的基因组微失衡。3PCR验证表明先证者及其父亲为AZFc区域b2/b3缺失,此片段缺失同时存在于1/11例正常男童、4/9例精神发育迟滞未伴小阴茎男童。4精神发育迟滞未伴小阴茎男童AZFc区域b2/b3缺失频率为15.1%(27/179)。结论 AZFc区域b2/b3缺失可能不是精神发育迟滞伴小阴茎男童的致病病因,此AZFc区域部分缺失在中国人群中为多态性位点。展开更多
This paper investigates the regional distribution and pollution of energyintensive industries in China. Through the analysis of provincial panel data collected during 1998-2008, this work estimates the drivers of poll...This paper investigates the regional distribution and pollution of energyintensive industries in China. Through the analysis of provincial panel data collected during 1998-2008, this work estimates the drivers of pollution in 30 of China＇s provincial-level divisions. The paper concludes that while China＇s energy-intensive industries are heavily distributed in eastern and central China, the speed of development toward central and western China has, in recent years, risen continuously. Industries located in eastern China do, however, remain the primary polluters in the country. Notably, regional agglomeration of energy-intensive industries plays a positive role in energy conservation and pollution control in China. This paper also finds that patterns of pollution in China follow the environmental Kuznets curve （EKC） with strong inter-provincial discrepancies.展开更多
Industrial cluster has become an important part of regional economy,which status of global industry value chain is critical in the future, Innovation-based Cluster is the industrial cluster theory research at home and...Industrial cluster has become an important part of regional economy,which status of global industry value chain is critical in the future, Innovation-based Cluster is the industrial cluster theory research at home and abroad made to deepen the difference between the lower part of the Industrial cluster. Promoting innovative industrial cluster formation not only is one of the important means and carrier to cultivate strategic emerging industries, but also an important booster construction of national innovation system. According to the connotation,evolution process and characteristics of the Innovation-based Cluster, This paper from the perspective of three to analysis Innovation-based Clusters＇ innovative influence factors to find reasonable explanation to the creative industry cluster formation and more in-depth research.展开更多
Interior is the main space of human activities, which is an important part of human life and design activities, and is the link between spiritual civilization and material civilization. Interior design originates from...Interior is the main space of human activities, which is an important part of human life and design activities, and is the link between spiritual civilization and material civilization. Interior design originates from life, and needs to solve the problem of the material world and spiritual world. People improve the living environment and life quality through interior designs. The style of design depends on the geographical features and various cultures, which refi ects the artistic features and personality. The development history of modern architecture and interior design shows that the style of interior design is an important part of the whole architectural style, which reffects the design features of the whole building. Therefore, interior design and outdoor design should be in harmony. The orientation and characteristics of interior design determine the necessity of the style reforms. The future development trend of interior development may have the following characteristics humanism, green design, nationalization, intelligence and regionalization.展开更多
An MRPC prototype for the future CBM-TOF inner area has been developed.The counter assembled with low resistive glass consists of 10×0.22 mm gas gaps and 2×8 readout pads.The size of each pad is 20 mm×2...An MRPC prototype for the future CBM-TOF inner area has been developed.The counter assembled with low resistive glass consists of 10×0.22 mm gas gaps and 2×8 readout pads.The size of each pad is 20 mm×20 mm with an interval of 2 mm between pads.The signals of the counter are collected by 4 eight-pin connectors.Preliminary tests performed with cosmic-rays showed an efficiency over 98%and a time resolution around 60 ps.The measured crosstalk,dark current and dark rate were at a very low levels.Results under uniform irradiation performed at JINR with high energy deuteron showed a time resolution of40–50 ps and over 98%efficiency at a low flux rate,meanwhile at a flux up to 70 kHz/cm2,93%efficiency and 77 ps time resolution still remained.These excellent performances call fully meet the demand of CBM-TOF inner wall.展开更多
基金This paper was made possible by grants from the Modern Business Research Center of Zhejiang Gongshang University, Zhejiang Provincial Natural Science Foundation.
文摘This paper investigates the regional distribution and pollution of energyintensive industries in China. Through the analysis of provincial panel data collected during 1998-2008, this work estimates the drivers of pollution in 30 of China＇s provincial-level divisions. The paper concludes that while China＇s energy-intensive industries are heavily distributed in eastern and central China, the speed of development toward central and western China has, in recent years, risen continuously. Industries located in eastern China do, however, remain the primary polluters in the country. Notably, regional agglomeration of energy-intensive industries plays a positive role in energy conservation and pollution control in China. This paper also finds that patterns of pollution in China follow the environmental Kuznets curve （EKC） with strong inter-provincial discrepancies.
文摘Industrial cluster has become an important part of regional economy,which status of global industry value chain is critical in the future, Innovation-based Cluster is the industrial cluster theory research at home and abroad made to deepen the difference between the lower part of the Industrial cluster. Promoting innovative industrial cluster formation not only is one of the important means and carrier to cultivate strategic emerging industries, but also an important booster construction of national innovation system. According to the connotation,evolution process and characteristics of the Innovation-based Cluster, This paper from the perspective of three to analysis Innovation-based Clusters＇ innovative influence factors to find reasonable explanation to the creative industry cluster formation and more in-depth research.
文摘Interior is the main space of human activities, which is an important part of human life and design activities, and is the link between spiritual civilization and material civilization. Interior design originates from life, and needs to solve the problem of the material world and spiritual world. People improve the living environment and life quality through interior designs. The style of design depends on the geographical features and various cultures, which refi ects the artistic features and personality. The development history of modern architecture and interior design shows that the style of interior design is an important part of the whole architectural style, which reffects the design features of the whole building. Therefore, interior design and outdoor design should be in harmony. The orientation and characteristics of interior design determine the necessity of the style reforms. The future development trend of interior development may have the following characteristics humanism, green design, nationalization, intelligence and regionalization.
基金supported by the National Natural Science Foundation of China (Grant Nos. 11020101059, 10775082, and 11275108)
文摘An MRPC prototype for the future CBM-TOF inner area has been developed.The counter assembled with low resistive glass consists of 10×0.22 mm gas gaps and 2×8 readout pads.The size of each pad is 20 mm×20 mm with an interval of 2 mm between pads.The signals of the counter are collected by 4 eight-pin connectors.Preliminary tests performed with cosmic-rays showed an efficiency over 98%and a time resolution around 60 ps.The measured crosstalk,dark current and dark rate were at a very low levels.Results under uniform irradiation performed at JINR with high energy deuteron showed a time resolution of40–50 ps and over 98%efficiency at a low flux rate,meanwhile at a flux up to 70 kHz/cm2,93%efficiency and 77 ps time resolution still remained.These excellent performances call fully meet the demand of CBM-TOF inner wall.
