Antisense Oligonucleotide-Based Rescue of Aberrant Splicing Defects Caused by 15 Pathogenic Variants in ABCA4

The discovery of novel intronic variants in the ABCA4 locus has contributed significantly to solving the missing heritability in Stargardt disease (STGD1). The increasing number of variants affecting pre-mRNA splicing makes ABCA4 a suitable candidate for antisense oligonucleotide (AON)-based splicing modulation therapies. In this study, AON-based splicing modulation was assessed for 15 recently described intronic variants (three near-exon and 12 deep-intronic variants). In total, 26 AONs were designed and tested in vitro using a midigene-based splice system. Overall, partial or complete splicing correction was observed for two variants causing exon elongation and all variants causing pseudoexon inclusion. Together, our results confirm the high potential of AONs for the development of future RNA therapies to correct splicing defects causing STGD1.     

压力容器环缝焊接自动跟踪系统的研究

作者研制了一种新型摆动式电弧传感器及其焊缝自动跟踪系统。采用计算机控制，成功地应用于压力容器的环缝脉冲ＭＡＧ焊工艺。实践证明采用这种系统跟踪精度高并获得了优质焊缝。     

在线监测结晶器调宽换向阀阀芯卡阻方法的实现

对某炼钢厂铸机结晶器在线调宽液压系统的工作原理进行了分析,并重点分析了调宽过程中换向阀阀芯卡阻的问题,针对该问题对其液压系统进行了改进,改进后可以在线监测换向阀阀芯是否卡阻。该改进的实施在减少结晶器在线调宽时发生漏钢故障方面起到了重要作用。     

绝缘栅双极晶体管脉冲斩波器的设计与应用

高频脉冲电流加工及光整加工技术能提高零件的加工精度和表面质量。脉冲斩波器的设计是应用该技术的关键所在。介绍了脉冲电源的构成原理及设计思路，并采用绝缘栅双极晶体管(1GBT)设计制造出高频、大电流脉冲斩波器。用所设计的脉冲电源进行了脉冲电化学光整加工实验，得到了满意的结果。     

脉冲进气旋转喷吹技术的水力模拟

在铝合金熔体净化旋转喷吹技术的基础上 ,采用水力模拟实验研究了脉冲进气方式对气泡大小的影响。实验结果表明 :脉冲进气方式有利于产生射流 ,使气泡变小 ,并增加了气泡与熔体接触的比表面积 ,从而改善了除氢的动力学条件 ,有利于提高除氢效果。     

高密度脉冲电流对A356铝合金凝固组织的影响

研究了高密度脉冲电流对A356铝合金凝固组织的影响，实验中对液相线以下溶体的凝固过程在不同阶段进行电脉冲处理。对比实验结果表明：经过电脉冲处理后，A356的合金凝固组织得到明显细化；在同样的电脉冲条件下，凝固组织细化效果与电脉冲放电开始时间有关，凝固过程中放电开始时间越早，凝固组织细化效果越明显；反之，凝固组织细化效果较差；同时，脉冲充电电压越高，凝固组织细化程度越大。定量计算了脉冲电流产生的电磁力并分析了脉冲电流影响凝固组织的作用机制。     

扩展接触器零部件通用性的研究

本文介绍了一种只用十几个分立元件的电子模块，与大型接触器配合使用，可使接触器节省铜线材料60％～70％，大大减少电磁系统的体积。预置电子模块的参数，可实现交流接触器在起动过程、吸持过程的最佳电流控制，为扩展接触器零部件通用性研制出节能、节材、无噪声等性能优良的小型而容易实施的交流接触器产品提供了依据。     

燃气加热托辊型网带炉生产线的研制与应用

介绍了燃气加热托辊型网带炉生产线的组成、燃气脉冲控制和空气/燃气比例连续调节技术和计算机多媒体集散控制系统.生产应用表明炉温均匀性良好,处理产品质量稳定,能耗成本降低,取得了明显的经济效益.     

一种检测电火花加工间隙状态的柔性方法

介绍了一种在数控电火花加工脉冲电源中实现间隙状态检测的柔性方法，具有阈值设置柔性、状态识别准确的特点，巧妙地用一个稳压二极管的钳位作用提高了间隙状态识别的精度，非常适合需经常改变加工参数的加工实验使用。     

4-methoxy-2,6-bis(3,5-dimethylpyrazoyl)-1,3,5-triazine modified carbon paste electrode for trace Cu(Ⅱ) determination by differential pulse volt-ammetry

A differential pulse voltammetric method was developed for the sensitive and selective determination of Cu(Ⅱ) at 4-methoxy-2,6-bis(3,5-dimethylpyrazoyl)-l,3,5-triazine modified carbon paste electrode in 0.05 mol/L KHC8H4O4 solution (pH = 4.02). The oxidation peak of Cu(Ⅱ) was observed at 0.065 V(vs Ag/AgCl) by scanning the potential in positive direc-tion. The analysis procedure consisted of an open circuit accumulation step in stirred sample solution. It was followed by medium exchange to a clean solution and subsequently an anodic potential scan was affected to obtain the voltammetric peak. The current was proportional to the concentration of the Cu(Ⅱ) ion in a range of 1× 10-7-1×10-4 mol/L for 6 min accumulation; the most of metal ions did not interfere with the determination. The developed method was applied to Cu(Ⅱ) determination in coal-ash sample, the results agreed with that of atomic adsorption spectroscopy(AAS).