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.     

建筑用气密膜相关强度性能试验研究

气密膜是被动房和超低能耗建筑气密性处理的常用材料,由于试验设备及方法等原因,国内对建筑用气密膜性能的试验研究较少,从施工应用角度出发,针对国内外无纺布基气密膜的相关强度性能开展试验研究,为气密膜的工程应用提供参考.     

伊拉克米桑油田水平井钻井关键技术

针对伊拉克米桑油田盐膏层巨厚、钻井液密度窗口窄、存在高压盐水层,储层段漏塌同层导致复杂时效高等问题,通过对比分析国内外盐膏层和窄压力窗口地层钻井技术,总结了米桑油田钻井面临的主要技术难点,从井身结构优化、钻井提速和复杂预防等方面入手,研究形成了适合米桑油田的钻井关键技术,并进行了现场试验应用,取得了显著的效果.伊拉克米桑油田钻井关键技术对该地区钻井技术方案设计和钻井提速提效具有较强的借鉴和指导作用.     

CuO-SiO2气凝胶@TiO2纳米纤维无牺牲剂光催化还原CO2

采用溶胶-凝胶法制备CuO-SiO2复合气凝胶,通过在气凝胶孔道内填充TiCl4,然后将其气相水解,得到了在CuO-SiO2气凝胶表面生长了高结晶度的TiO2纳米纤维(CuO-SiO2@TiO2),纤维直径～16 nm.通过XPS、UPS、UV-Vis DRS、荧光光谱(PL)等表征了材料的结构及光电性能.结果表明,制备的CuO-SiO2@TiO2对可见光有明显吸收,且荧光强度较商用TiO2(P25)大幅降低,光生电子-空穴对更加稳定.再在纳米纤维上负载CuO,所得CuO-SiO2@TiO2/CuO在可见光区的荧光强度进一步增强.以300 W氙灯为光源,分别以CuO-SiO2@TiO2及CuO-SiO2@TiO2/CuO为催化剂,无牺牲剂条件下光催化还原CO2,4 h后甲醇产率分别为1304.0及1589.0μmol/g-cat,转换频率(TOF)分别为0.038及0.046 h–1.循环实验表明,纳米纤维具有较好的光催化稳定性,经过4次光催化循环实验后,CuO-SiO2@TiO2/CuO的保留率～94％,甲醇产率可达1472.0μmol/g-cat,TOF为0.042 h–1.     

水厂生产废水处理工艺节地高效化设计探索

随着对环保及自用水率的要求日益严格,水厂生产废水的减排已成为发展趋势.但既有水厂用地紧张和生产废水的处理工艺占地多存在矛盾,制约了水厂新增生产废水处理工艺的可行性.文中引入斜管沉淀提升沉淀效率、取消浓缩池、使用叠螺脱水机代替离心脱水机的优化设计方案,相较原设计的传统分离式生产废水处理工艺,生产废水停留时间减少75％,占地面积减少约48％.实践证明,该优化设计方案能够有效解决水厂生产废水处理工艺设计过程中遇到的用地不足问题,并有效地降低建设投资.     

基于有限元的低成本塔桅设计及承载能力分析

为研究铁塔公司发布的最新《低成本铁塔标准图集V1.0》的共享改造能力,文章选取了单斜撑三管塔0.65 kN/m2基本风压下的系列塔图,从各塔段的主管应力比和塔顶位移角两个角度,考虑新增天线设备、馈线、爬梯荷载,以及天线设备之间的相互遮挡,利用有限元软件SAP2000对每种塔型进行了常规设计分析和改造增载后的研究分析.由分析结果可知,每种塔型均有可共享改造能力,且随着塔身高度的增加,共享改造的能力逐渐降低.     

Additive Manufacturing of Stable Energy Storage Devices Using a Multinozzle Printing System

The development of the Internet of things has prompted an exponential increase in the demand for flexible, wearable devices, thereby posing new challenges to their integration and conformalization. Additive manufacturing facilitates the fabrication of complex parts via a single integrated process. Herein, the development of a multinozzle, multimaterial printing device is reported. This device accommodates the various characteristics of printing materials, ensures high-capacity printing, and can accommodate a wide range of material viscosities from 0 to 1000 Cp. Complete capacitors, inclusive of the current collector, electrode, and electrolyte, can be printed without repeated clamping to complete the preheating, printing, and sintering processes. This method addresses the poor stability issue associated with printed electrode materials. Furthermore, after the intercalation of LiFePO₄ with Na ions, X-ray photoelectron spectroscopy and X-ray diffraction results reveal that the Na ions permeate the interlayer structure of LiFePO₄, enhancing the ion migration channels by increasing the ion transmission rate. A current rate of 2.5 mAh ensures >2000 charge/discharge cycles, while retaining a charge/discharge efficiency of 96% and a discharge capacity of 91.3 mAh g⁻¹. This manufacturing process can provide conformal power modules for a diverse range of portable devices with various shapes, improving space utilization.     

大型球铁水表壳加工工装设计与加工

针对表壳具有较多复杂曲面及三端加工特点及需求,通过对球铁水表壳的外形及加工特点进行分析,提出合理的设计加工工序方案,选用特种加工设备及专业的工装夹具.通过针对表壳外形及加工工艺特点设计专业的工装夹具达到一次装夹多工序同时加工,有效解决了传统加工工艺的多工序多道加工,提高加工效率并确保加工质量.     

Self-Adaptive Control System for Additive Manufacturing Using Double Electrode Micro Plasma Arc Welding

Wire arc additive manufacturing(WAAM)has been investigated to deposit large-scale metal parts due to its high deposition efficiency and low material cost.However,in the process of automatically manufacturing the high-quality metal parts by WAAM,several problems about the heat build-up,the deposit-path optimization,and the stability of the process parameters need to be well addressed.To overcome these issues,a new WAAM method based on the double electrode micro plasma arc welding(DE-MPAW)was designed.The circuit principles of different metal-transfer models in the DE-MPAW deposition process were analyzed theoretically.The effects between the parameters,wire feed rate and torch stand-off distance,in the process of WAAM were investigated experimentally.In addition,a real-time DE-MPAW control system was developed to optimize and stabilize the deposition process by self-adaptively changing the wire feed rate and torch stand-off distance.Finally,a series of tests were performed to evaluate the con-trol system's performance.The results show that the capability against interferences in the process of WAAM has been enhanced by this self-adaptive adjustment system.Further,the deposition paths about the metal part's layer heights in WAAM are simplified.Finally,the appearance of the WAAM-deposited metal layers is also improved with the use of the control system.     

ZIF衍生氧还原反应催化剂的研究进展

从沸石咪唑酯骨架材料(ZIF)衍生的含过渡金属催化剂出发,介绍ZIF衍生催化剂前驱体的制备和热解温度、气氛对催化剂的影响,总结ZIF衍生的铂和非贵金属催化剂材料用于氧还原反应(ORR)的研究进展.提出有关ZIF衍生ORR催化剂发展面临的实际应用挑战和活性位点探究问题.