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.     

S120在薄带铸轧主轧机控制系统上的应用

薄带铸轧生产线设备紧凑,生产工艺更加低耗高效,是钢铁工业流行发展的趋势.SINAMICS S120控制系统应用在薄带铸轧主轧机传动系统上,可以根据需求灵活增添功能配置.通过介绍该生产线工艺流程,突出S120传动控制系统的优势.此外,针对S120控制系统设计和应用,尤其是负荷平衡控制、SINAMICS Link通讯、安全转矩关断等特殊应用的实现进行了详细的阐述.     

调控Al2O3晶型控制MgAl2O4:Mn4+荧光粉中Mn价态研究

Mn⁴⁺激活红光荧光粉是白光半导体发光二极管(wLEDs)领域当前研究热点之一。Mn⁴⁺离子²E→⁴A₂跃迁在铝酸盐中的最短发光波长是在MgAl₂O₄中实现的651 nm发光, 由于其结构中含有形成四面体或八面体配位的两种阳离子格位(Mg²⁺/Al³⁺), 易造成所掺杂锰元素存在多种价态(+2/+4/+3等)。本研究通过改变起始原料中Al₂O₃的晶型(γ/α比例)及退火处理来调控锰离子在MgAl₂O₄晶格中的占据格位, 对其主要存在价态实现调控。采用荧光光谱和紫外-可见-近红外漫反射光谱技术来表征所合成荧光粉中Mn离子的价态及其演变。研究发现, 高α/(α+γ)比铝源促进Mn²⁺形成, 而低α/(α+γ)比铝源促进Mn⁴⁺形成。通过使用高活性纳米γ-Al₂O₃为铝源, 有效抑制了锰离子在MgAl₂O₄中Mg²⁺格位的占据及Mn²⁺离子的形成, 经空气中1550 ℃保温5 h的一次高温热处理即可制备出在可见光区只有Mn⁴⁺红光发光的高纯高亮度MgAl₂O₄:Mn⁴⁺荧光粉。氧化铝晶型影响锰离子掺杂格位和掺杂价态的本质规律是: 氧化铝活性决定实际固溶掺杂反应步骤, 进而影响锰离子掺杂格位和价态。本研究提出的反应步骤调控作为反应气氛、电荷补偿剂、反应温度三种调控方法外的一种新方法, 为Mn⁴⁺激活铝酸盐荧光粉中锰离子掺杂价态调控提供了新思路。     

基于GA-BP神经网络的电网暂稳极限切除时间预测

本文介绍了一种利用PSASP软件结合GA-BP神经网络的极限切除时间预测方法.通过PSASP软件搭建三机九节点系统,模拟系统发生三相短路故障,得到实验数据,将实验数据分为训练集和测试集,构建基于GA-BP的极限切除时间预测模型.结果表明,基于GA-BP模型的极限切除时间预测值与实际值基本一致,且GA-BP模型的预测精度高于BP模型.     

基于用户体验的压路机操纵手柄交互品质研究

目的以压路机操纵手柄为对象,探讨交互品质研究方法,改进用户体验。方法对交互品质进行解析,由用户体验与交互品质的关联性,提出基于用户体验的交互品质研究方法;面向压路机操纵手柄典型任务进行用户体验研究,梳理压路机手柄的操作流程,并对交互行为进行聚类分析,结合语义分析构建用户体验词库,运用半结构式访谈与问卷调查,以定量与定性方法相结合对驾驶员的操纵体验进行调研;以专家研讨的形式进行驾驶员的操纵体验向交互特征的映射,构建交互特征集,进而对操纵手柄的交互品质进行分析与阐释。结果得出了压路机操纵手柄的交互特征与交互品质。结论通过用户体验可以映射交互特征,得到了交互品质,对于从交互品质的角度指导交互设计提供了借鉴。