Amyotrophic lateral sclerosis(ALS)is a neuromuscular condition resulting from the progressive degeneration of motor neurons in the cortex,brainstem,and spinal cord.While the typical clinical phenotype of ALS involves ...Amyotrophic lateral sclerosis(ALS)is a neuromuscular condition resulting from the progressive degeneration of motor neurons in the cortex,brainstem,and spinal cord.While the typical clinical phenotype of ALS involves both upper and lower motor neurons,human and animal studies over the years have highlighted the potential spread to other motor and non-motor regions,expanding the phenotype of ALS.Although superoxide dismutase 1(SOD1)mutations represent a minority of ALS cases,the SOD1 gene remains a milestone in ALS research as it represents the first genetic target for personalized therapies.Despite numerous single case reports or case series exhibiting extramotor symptoms in patients with ALS mutations in SOD1(SOD1-ALS),no studies have comprehensively explored the full spectrum of extramotor neurological manifestations in this subpopulation.In this narrative review,we analyze and discuss the available literature on extrapyramidal and non-motor features during SOD1-ALS.The multifaceted expression of SOD1 could deepen our understanding of the pathogenic mechanisms,pointing towards a multidisciplinary approach for affected patients in light of new therapeutic strategies for SOD1-ALS.展开更多
Under the analysis framework of output distance function,this paper makes overall measurement of rural Agriculture Logistics Efficiency( ALE) by constructing non-parametric production frontier function model using rel...Under the analysis framework of output distance function,this paper makes overall measurement of rural Agriculture Logistics Efficiency( ALE) by constructing non-parametric production frontier function model using related panel date of 28 provinces over the period1994-2014 in China. The accession to the WTO is the most important milestone in the process of China's globalization. It employs the bi-directional fixed effects model and the difference in differences( DID) method to study the impacts of globalization( joining WTO) on ALE and its mechanism of action in the context of China. The results show that the WTO makes ALE of China generally increased by about 34. 2%,promoting ALE with a long-term dynamic gradual process; long-term impact of globalization on ALE is about 83. 3%,which is higher than shortterm effect; the promotion of ALE is obtained mainly by the improvement of rural logistics infrastructure,rural informatization level,regional division of agriculture and the quality of rural labor force brought by globalization; agriculture logistics professional level has not yet acquired obvious enhancement during the process of globalization in China so far.展开更多
在物流4.0以及电子商务时代背景下,仓储配送中心在供应链中扮演着至关重要的角色,其中,订单拣选系统作为仓储作业的核心,备受学术界和工业界的关注。首先,介绍移动机器人(Autonomous Mobile Robots,AMR)订单拣选系统的类型,重点关注AMR-...在物流4.0以及电子商务时代背景下,仓储配送中心在供应链中扮演着至关重要的角色,其中,订单拣选系统作为仓储作业的核心,备受学术界和工业界的关注。首先,介绍移动机器人(Autonomous Mobile Robots,AMR)订单拣选系统的类型,重点关注AMR-assisted系统、AMR-pick系统和移动机器人履行系统的优化内容和优化目标;其次,针对上述三类系统,从布局设计、存储分配、订单分批、任务分配、路径规划以及联合优化等方面综述其研究进展;最后,总结当前研究中存在的不足,并提出若干进一步研究内容。展开更多
文摘Amyotrophic lateral sclerosis(ALS)is a neuromuscular condition resulting from the progressive degeneration of motor neurons in the cortex,brainstem,and spinal cord.While the typical clinical phenotype of ALS involves both upper and lower motor neurons,human and animal studies over the years have highlighted the potential spread to other motor and non-motor regions,expanding the phenotype of ALS.Although superoxide dismutase 1(SOD1)mutations represent a minority of ALS cases,the SOD1 gene remains a milestone in ALS research as it represents the first genetic target for personalized therapies.Despite numerous single case reports or case series exhibiting extramotor symptoms in patients with ALS mutations in SOD1(SOD1-ALS),no studies have comprehensively explored the full spectrum of extramotor neurological manifestations in this subpopulation.In this narrative review,we analyze and discuss the available literature on extrapyramidal and non-motor features during SOD1-ALS.The multifaceted expression of SOD1 could deepen our understanding of the pathogenic mechanisms,pointing towards a multidisciplinary approach for affected patients in light of new therapeutic strategies for SOD1-ALS.
基金the Social Science Foundation of Hunan Province in China(2014JD29)the National Social Science Foundation of China(12BJY123)the National Social Science Foundation of China(15AJL006)
文摘Under the analysis framework of output distance function,this paper makes overall measurement of rural Agriculture Logistics Efficiency( ALE) by constructing non-parametric production frontier function model using related panel date of 28 provinces over the period1994-2014 in China. The accession to the WTO is the most important milestone in the process of China's globalization. It employs the bi-directional fixed effects model and the difference in differences( DID) method to study the impacts of globalization( joining WTO) on ALE and its mechanism of action in the context of China. The results show that the WTO makes ALE of China generally increased by about 34. 2%,promoting ALE with a long-term dynamic gradual process; long-term impact of globalization on ALE is about 83. 3%,which is higher than shortterm effect; the promotion of ALE is obtained mainly by the improvement of rural logistics infrastructure,rural informatization level,regional division of agriculture and the quality of rural labor force brought by globalization; agriculture logistics professional level has not yet acquired obvious enhancement during the process of globalization in China so far.
文摘在物流4.0以及电子商务时代背景下,仓储配送中心在供应链中扮演着至关重要的角色,其中,订单拣选系统作为仓储作业的核心,备受学术界和工业界的关注。首先,介绍移动机器人(Autonomous Mobile Robots,AMR)订单拣选系统的类型,重点关注AMR-assisted系统、AMR-pick系统和移动机器人履行系统的优化内容和优化目标;其次,针对上述三类系统,从布局设计、存储分配、订单分批、任务分配、路径规划以及联合优化等方面综述其研究进展;最后,总结当前研究中存在的不足,并提出若干进一步研究内容。