Objective:Autosomal recessive bestrophinopathy(ARB),a retinal degenerative disease,is characterized by central visual loss,yellowish multifocal diffuse subretinal deposits,and a dramatic decrease in the light peak on ...Objective:Autosomal recessive bestrophinopathy(ARB),a retinal degenerative disease,is characterized by central visual loss,yellowish multifocal diffuse subretinal deposits,and a dramatic decrease in the light peak on electrooculogram.The potential pathogenic mechanism involves mutations in the BEST1 gene,which encodes Ca2+-activated Cl−channels in the retinal pigment epithelium(RPE),resulting in degeneration of RPE and photoreceptor.In this study,the complete clinical characteristics of two Chinese ARB families were summarized.Methods:Pacific Biosciences(PacBio)single-molecule real-time(SMRT)sequencing was performed on the probands to screen for disease-causing gene mutations,and Sanger sequencing was applied to validate variants in the patients and their family members.Results:Two novel mutations,c.202T>C(chr11:61722628,p.Y68H)and c.867+97G>A,in the BEST1 gene were identified in the two Chinese ARB families.The novel missense mutation BEST1 c.202T>C(p.Y68H)resulted in the substitution of tyrosine with histidine in the N-terminal region of transmembrane domain 2 of bestrophin-1.Another novel variant,BEST1 c.867+97G>A(chr11:61725867),located in intron 7,might be considered a regulatory variant that changes allele-specific binding affinity based on motifs of important transcriptional regulators.Conclusion:Our findings represent the first use of third-generation sequencing(TGS)to identify novel BEST1 mutations in patients with ARB,indicating that TGS can be a more accurate and efficient tool for identifying mutations in specific genes.The novel variants identified further broaden the mutation spectrum of BEST1 in the Chinese population.展开更多
It makes more sense to look at which is better for their own people.During a debate comparing Chinese and Western systems of governance,held in Cambridge,Massachusetts,by the American nonprofit educational organizatio...It makes more sense to look at which is better for their own people.During a debate comparing Chinese and Western systems of governance,held in Cambridge,Massachusetts,by the American nonprofit educational organization Intercollegiate Studies Institute on April 5,French entrepreneur and Internet influencer Arnaud Bertrand made a case for the suitability of China’s system for promoting the flourishing of its people.Edited excerpts of his presentation follow.展开更多
Digital transformation in the AEC industry(Architecture,Engineering and Construction)is a key driver to enhance technical innovation in the branch and adds dynamic to all work processes and methods.A more differentiat...Digital transformation in the AEC industry(Architecture,Engineering and Construction)is a key driver to enhance technical innovation in the branch and adds dynamic to all work processes and methods.A more differentiated understanding of the responsible use of innovative technologies aims not only towards increased technical,environmental,educational,societal and gender equality sustainability and more efficient building life cycles but also to recognize the unintended effects such as artificial intelligence(AI).The study is part of a larger primary research on Corporate Digital Responsibility(CDR)in Construction 4.0.This identifies,analyzes and systematically evaluates key factors of a sustainable digital transformation,especially in the traditionally small-scale Construction Industry,in which there can be no standardized procedure.The study uses interdisciplinary literature and data research and expert interviews.The qualitative method enables a critical-reflexive analysis of the key factors of meaningful and sustainable implementation of innovative technologies in Construction.Application examples show possible approaches,some of which are implemented as prototypes and provide guidance for small to medium-sized companies.The study outlines the necessary steps for companies to define their own potential fields of application and find suitable methods.Another aim of the study is to take stock of the acceptance of new technologies by comparing different perspectives from experts.The study results show new perspectives on the transformation of the Construction Industry.They show that Digital Transformation in Construction 4.0 has great potential for an economical,efficient construction life cycle,but requires the responsible,sensible use of innovative technologies.展开更多
Part II on Best Practices in Construction 4.0 follows up on the previously published study Part I.This study examines corporate strategies from different angles,defines potential fields of application and works out ex...Part II on Best Practices in Construction 4.0 follows up on the previously published study Part I.This study examines corporate strategies from different angles,defines potential fields of application and works out existing empirical values and trends in the digitization process of the building sector.It highlights the unintended consequences of technological development and offers concrete practical approaches for responsible use.Using the qualitative research method,the study concludes that digital methods,such as Building Information Modelling(BIM)and Digital Twins,and Artificial Intelligence(AI)can add value,significantly reduce resources and increase sustainability.The study is part of a larger primary research on Corporate Digital Responsibility(CDR)in Construction 4.0;it identifies,analyzes and systematically evaluates the pillars of a sustainable digital transformation,especially in the Construction Industry.The holistic,interdisciplinary view of this study aims to provide orientation for small to medium-sized companies(SMEs)developing their individual digital strategy.An outline of the necessary prerequisites but also design options,as they result from the evaluation of expert interviews and literature research,supports companies in the design of Construction 4.0 that is in line with the needs of people,society and the environment and shaping more economically efficient building life cycles.It highlights that digital transformation has also reached the traditionally small-scale AEC industry(small-scale architecture,engineering and construction industry)and catalyzes the variety of innovations.展开更多
The principle of the best interests of the child,as a criterion for substantive review,is conceptually ambiguous and uncertain in its application.To mitigate this dilemma in the application of the principle of the bes...The principle of the best interests of the child,as a criterion for substantive review,is conceptually ambiguous and uncertain in its application.To mitigate this dilemma in the application of the principle of the best interests of the child,the European Court of Human Rights(ECHR)has made a procedural transition in the interpretation and application of this principle,shifting from conducting specific proportionality analysis or interests balancing in cases related to children to examining whether States Parties have applied the principle of the best interests of the child in their judicial procedures.Moreover,ECHR has developed three procedural review schemes:holistic reviews,key factors-based reviews and factor list-based reviews.Compared with substantive reviews,procedural reviews adhere to the ECHR doctrine of margin of appreciation,restrict the free discretion of the court,give play to the effect of procedural autonomy,and pursue the value of subjective procedural justice,which has its own unique theoretical value and practical significance,and provides a feasible reference for China to interpret and apply the principle of the best interests of the child.展开更多
近年来,社会化推荐作为推荐算法之一被广泛应用于各大平台.由于引入了用户的社交信息,社会化推荐可以较好地缓解数据稀疏问题.然而,大部分社会化推荐难以高效地从原始信息中提取用户的有效信息,导致引入社会信息的同时也会引入大量噪声...近年来,社会化推荐作为推荐算法之一被广泛应用于各大平台.由于引入了用户的社交信息,社会化推荐可以较好地缓解数据稀疏问题.然而,大部分社会化推荐难以高效地从原始信息中提取用户的有效信息,导致引入社会信息的同时也会引入大量噪声.为了解决上述问题,本文提出了SRBHL(Social Recommendation Based on Hypergraph embedding and Limited attention)模型,通过超图嵌入模块提取用户的历史行为信息和社交信息,以缓解原始目标用户数据稀疏问题,并结合有限注意力模块来过滤原始信息的噪声,最后将得到的有效好友信息用于推荐.在Yelp-Urbana、Yelp-Phoenix和Epinions3个真实数据集上的实验结果表明SRBHL模型相比其他的推荐算法表现更出色.此外,本文还对SRBHL模型进行了鲁棒性分析,并给出了模型最优参数的取值范围.展开更多
文摘Objective:Autosomal recessive bestrophinopathy(ARB),a retinal degenerative disease,is characterized by central visual loss,yellowish multifocal diffuse subretinal deposits,and a dramatic decrease in the light peak on electrooculogram.The potential pathogenic mechanism involves mutations in the BEST1 gene,which encodes Ca2+-activated Cl−channels in the retinal pigment epithelium(RPE),resulting in degeneration of RPE and photoreceptor.In this study,the complete clinical characteristics of two Chinese ARB families were summarized.Methods:Pacific Biosciences(PacBio)single-molecule real-time(SMRT)sequencing was performed on the probands to screen for disease-causing gene mutations,and Sanger sequencing was applied to validate variants in the patients and their family members.Results:Two novel mutations,c.202T>C(chr11:61722628,p.Y68H)and c.867+97G>A,in the BEST1 gene were identified in the two Chinese ARB families.The novel missense mutation BEST1 c.202T>C(p.Y68H)resulted in the substitution of tyrosine with histidine in the N-terminal region of transmembrane domain 2 of bestrophin-1.Another novel variant,BEST1 c.867+97G>A(chr11:61725867),located in intron 7,might be considered a regulatory variant that changes allele-specific binding affinity based on motifs of important transcriptional regulators.Conclusion:Our findings represent the first use of third-generation sequencing(TGS)to identify novel BEST1 mutations in patients with ARB,indicating that TGS can be a more accurate and efficient tool for identifying mutations in specific genes.The novel variants identified further broaden the mutation spectrum of BEST1 in the Chinese population.
文摘It makes more sense to look at which is better for their own people.During a debate comparing Chinese and Western systems of governance,held in Cambridge,Massachusetts,by the American nonprofit educational organization Intercollegiate Studies Institute on April 5,French entrepreneur and Internet influencer Arnaud Bertrand made a case for the suitability of China’s system for promoting the flourishing of its people.Edited excerpts of his presentation follow.
文摘Digital transformation in the AEC industry(Architecture,Engineering and Construction)is a key driver to enhance technical innovation in the branch and adds dynamic to all work processes and methods.A more differentiated understanding of the responsible use of innovative technologies aims not only towards increased technical,environmental,educational,societal and gender equality sustainability and more efficient building life cycles but also to recognize the unintended effects such as artificial intelligence(AI).The study is part of a larger primary research on Corporate Digital Responsibility(CDR)in Construction 4.0.This identifies,analyzes and systematically evaluates key factors of a sustainable digital transformation,especially in the traditionally small-scale Construction Industry,in which there can be no standardized procedure.The study uses interdisciplinary literature and data research and expert interviews.The qualitative method enables a critical-reflexive analysis of the key factors of meaningful and sustainable implementation of innovative technologies in Construction.Application examples show possible approaches,some of which are implemented as prototypes and provide guidance for small to medium-sized companies.The study outlines the necessary steps for companies to define their own potential fields of application and find suitable methods.Another aim of the study is to take stock of the acceptance of new technologies by comparing different perspectives from experts.The study results show new perspectives on the transformation of the Construction Industry.They show that Digital Transformation in Construction 4.0 has great potential for an economical,efficient construction life cycle,but requires the responsible,sensible use of innovative technologies.
文摘Part II on Best Practices in Construction 4.0 follows up on the previously published study Part I.This study examines corporate strategies from different angles,defines potential fields of application and works out existing empirical values and trends in the digitization process of the building sector.It highlights the unintended consequences of technological development and offers concrete practical approaches for responsible use.Using the qualitative research method,the study concludes that digital methods,such as Building Information Modelling(BIM)and Digital Twins,and Artificial Intelligence(AI)can add value,significantly reduce resources and increase sustainability.The study is part of a larger primary research on Corporate Digital Responsibility(CDR)in Construction 4.0;it identifies,analyzes and systematically evaluates the pillars of a sustainable digital transformation,especially in the Construction Industry.The holistic,interdisciplinary view of this study aims to provide orientation for small to medium-sized companies(SMEs)developing their individual digital strategy.An outline of the necessary prerequisites but also design options,as they result from the evaluation of expert interviews and literature research,supports companies in the design of Construction 4.0 that is in line with the needs of people,society and the environment and shaping more economically efficient building life cycles.It highlights that digital transformation has also reached the traditionally small-scale AEC industry(small-scale architecture,engineering and construction industry)and catalyzes the variety of innovations.
文摘The principle of the best interests of the child,as a criterion for substantive review,is conceptually ambiguous and uncertain in its application.To mitigate this dilemma in the application of the principle of the best interests of the child,the European Court of Human Rights(ECHR)has made a procedural transition in the interpretation and application of this principle,shifting from conducting specific proportionality analysis or interests balancing in cases related to children to examining whether States Parties have applied the principle of the best interests of the child in their judicial procedures.Moreover,ECHR has developed three procedural review schemes:holistic reviews,key factors-based reviews and factor list-based reviews.Compared with substantive reviews,procedural reviews adhere to the ECHR doctrine of margin of appreciation,restrict the free discretion of the court,give play to the effect of procedural autonomy,and pursue the value of subjective procedural justice,which has its own unique theoretical value and practical significance,and provides a feasible reference for China to interpret and apply the principle of the best interests of the child.
文摘近年来,社会化推荐作为推荐算法之一被广泛应用于各大平台.由于引入了用户的社交信息,社会化推荐可以较好地缓解数据稀疏问题.然而,大部分社会化推荐难以高效地从原始信息中提取用户的有效信息,导致引入社会信息的同时也会引入大量噪声.为了解决上述问题,本文提出了SRBHL(Social Recommendation Based on Hypergraph embedding and Limited attention)模型,通过超图嵌入模块提取用户的历史行为信息和社交信息,以缓解原始目标用户数据稀疏问题,并结合有限注意力模块来过滤原始信息的噪声,最后将得到的有效好友信息用于推荐.在Yelp-Urbana、Yelp-Phoenix和Epinions3个真实数据集上的实验结果表明SRBHL模型相比其他的推荐算法表现更出色.此外,本文还对SRBHL模型进行了鲁棒性分析,并给出了模型最优参数的取值范围.
