Targeted editing of intronic-splicing silencer enhancement of SMN2 Exon 7 inclusion by CRISPR/Case 9

Spinal muscular atrophy (SMA) is an autosomal recessive hereditary neuromuscular disease. Exon 7 and 8 of survival of motor neuron 1 (SMN1) gene or only exon 7 homology deletion leads to the failure to produce a full-length SMN gene. The copy number of SMN2 gene with high homology of SMN1 affects the degree of disease and was the target gene for targeting therapy, in which splicing silencer in intron 7 was the key to suppress the inclusion of exon 7. In this study, we projected to use CRISPR/Case 9 for the targeted editing of intronic-splicing silencer (ISS) sequence to promote the inclusion of SMN2 exon 7 and increase the production of SMN2 full-length (FL) gene expression. It happens that there was a protospacer adjacent motif (PAM) at one end of the ISS sequence according to the design of sgRNA. The recombinant vector of sgRNA HSMN2 CRISPR/Case 9 was constructed and transfected into HEK293 cells. Sequencing results showed that the ISS sequence could be edited accurately and targeting in the predicted direction, in which deleting small fragments, inserting small amounts and mutation. Quantitative analysis of RT-PCR products by restriction enzyme of DdeI digestion showed that the FL of SMN2 increased by 8% (P < 0.05). In the primary cultured chondrocytes of SMA mice, in which sgRNA HSMN2 CRISPR/Case9 recombinant vector transfection could increase the SMN2 FL gene by 23% (P < 0.05) and significantly improve SMN protein levels (P < 0.05). CRISPR/Case 9 is an effective tool for gene editing and therapy of hereditary diseases, but it is rarely reported in the treatment of SMA diseases. This study shows that CRISPR/Case 9 was first used for the precision target of ISS sequence editing, which can effectively promote the production of SMN2 FL gene expressions, in which there was an important clinical reference value.     

Exploration of GH94 Sequence Space for Enzyme Discovery Reveals a Novel Glucosylgalactose Phosphorylase Specificity

The substantial increase in DNA sequencing efforts has led to a rapid expansion of available sequences in glycoside hydrolase families. The ever-increasing sequence space presents considerable opportunities for the search for enzymes with novel functionalities. In this work, the sequence-function space of glycoside hydrolase family 94 (GH94) was explored in detail, using a combined approach of phylogenetic analysis and sequence similarity networks. The identification and experimental screening of unknown clusters led to the discovery of an enzyme from the soil bacterium Paenibacillus polymyxa that acts as a 4-O-β-d -glucosyl-d -galactose phosphorylase (GGalP), a specificity that has not been reported to date. Detailed characterization of GGalP revealed that its kinetic parameters were consistent with those of other known phosphorylases. Furthermore, the enzyme could be used for production of the rare disaccharides 4-O-β-d -glucosyl-d -galactose and 4-O-β-d -glucosyl-l -arabinose. Our current work highlights the power of rational sequence space exploration in the search for novel enzyme specificities, as well as the potential of phosphorylases for rare disaccharide synthesis.     

基于PMU的电力系统监控体系结构优化设计

文中提出了一种利用有限数量的相量测量单元(PMU)和相量数据集中器(PDC)设计最优监控结构的方法。通过在大量的设定值场景下,使电力系统可观测性曲线的期望值最大化,同时使通信基础设施成本最小化,最终确定PMU和PDC的最佳位置。提出了一种非线性动态扩展卡尔曼滤波(EFK)状态观测器。这种状态观测器可以将暂态行为转换为由代数微分方程描述的广域电力系统,而无需非线性反演技术。最后以IEEE-5电力系统为例,说明了该方法的有效性。     

立柱加固型框架大楼的爆破拆除

采用定向控制爆破技术拆除立柱加固型7层框架大楼。为了彻底炸毁加固立柱,确保大楼顺利定向倾倒,通过多次爆破试验确定了炸药单耗、布孔参数和装药结构。为了控制大楼塌落时的触地震动,采取了秒差分区爆破、空中解体、铺垫缓冲层和开挖减震沟等技术措施。爆破过程中进行了震动监测。此外还介绍了起爆顺序、安全防护措施及爆破结果。     

Image sequence-based risk behavior detection of power operation inspection personnel

A novel image sequence-based risk behavior detection method to achieve high-precision risk behavior detection for power maintenance personnel is proposed in this paper. In this method, the original image sequence data is first separated from the foreground and background. Then, the free anchor frame detection method is used in the foreground image to detect the personnel and correct their direction. Finally, human posture nodes are extracted from each frame of the image sequence, which are then used to identify the abnormal behavior of the human. Simulation experiment results demonstrate that the proposed algorithm has significant advantages in terms of the accuracy of human posture node detection and risk behavior identification.     

火电机组一次调频性能实时监测系统

为了实时监测和评价火电机组一次调频性能，开发了火电机组一次调频性能实测监测系统。该系统具有一次调频参数异动感知、一次调频性能量化预测及自适应校正等关键功能。某机组的实际应用表明，该系统能够实现火电机组一次调频关键参数的实时监测和调频性能的准确预测。     

Photomemory and Pulse Monitoring Featured Solution-Processed Near-Infrared Graphene/Organic Phototransistor with Detectivity of 2.4 × 1013 Jones

Emerging graphene/organic phototransistors are eye-catching technologies owing to their unique merits including easy/low-cost fabrication, temperature independent, and achieving various functions. However, their development in the near-infrared (NIR) region is experiencing a bottleneck of inferior sensitivity due to low exciton dissociation efficiency and inefficient charge extraction rate. Here, a novel-design solution-processed graphene/organic NIR phototransistor is reported, that is, creatively introducing electron extraction layer of ZnO on graphene channel and employing organic ternary bulk heterojunction as photosensitive layer, successfully breaking that bottleneck. The phototransistor exhibits a high responsivity of 6.1 × 10⁶ A W⁻¹, a superior detectivity of 2.4 × 10¹³ Jones, and a remarkable minimum detection power of 1.75 nW cm⁻² under 850 nm radiation. Considering its excellent NIR detection performance, a noncontact transmission-type pulse monitoring is carried out with no external circuit support, from which human pulse signal and heart rate can be displayed in real time. The phototransistor, interestingly, can be switched into a photomemory function with a retention time of 1000 s in the atmosphere through a gate voltage of −20 V. The design takes the characteristics of graphene/organic phototransistors to a higher level, beyond the limit of sensitivity, and opens up a novel approach for developing multifunction devices.     

Estimating long-term time-resolved indoor PM2.5 of outdoor and indoor origin using easily obtainable inputs

To evaluate the separate impacts on human health and establish effective control strategies, it is crucial to estimate the contribution of outdoor infiltration and indoor emission to indoor PM_2.5 in buildings. This study used an algorithm to automatically estimate the long-term time-resolved indoor PM_2.5 of outdoor and indoor origin in real apartments with natural ventilation. The inputs for the algorithm were only the time-resolved indoor/outdoor PM_2.5 concentrations and occupants’ window actions, which were easily obtained from the low-cost sensors. This study first applied the algorithm in an apartment in Tianjin, China. The indoor/outdoor contribution to the gross indoor exposure and time-resolved infiltration factor were automatically estimated using the algorithm. The influence of outdoor PM_2.5 data source and algorithm parameters on the estimated results was analyzed. The algorithm was then applied in four other apartments located in Chongqing, Shenyang, Xi'an, and Urumqi to further demonstrate its feasibility. The results provided indirect evidence, such as the plausible explanations for seasonal and spatial variation, to partially support the success of the algorithm used in real apartments. Through the analysis, this study also identified several further development directions to facilitate the practical applications of the algorithm, such as robust long-term outdoor PM_2.5 monitoring using low-cost light-scattering sensors.     

浅议生态环境监测机构的风险控制

现阶段,为了探索创新性的发展途径,生态环境监测机构应始终增强风险控制意识。应加强风险管理,立足于风险控制,以求不断健康发展。     

环境监测的现状、发展及优化对策浅析

社会经济的快速发展对生态环境造成了较大破坏,并产生了一系列连锁反应,不利于我国可持续发展目标的实现。为此,必须要切实做好环境监测工作。本文从环境监测的概念出发,就现阶段的环境监测现状进行了分析,并在此基础上阐述了环境监测工作未来发展及优化对策,以供参考。